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1. The vacuum and cryogenics system of the SOXS spectrograph

Author

Scuderi, S., Bellassai, G., Di Benedetto, R., Martinetti, E., Micciché, A., Nicotra, G., Occhipinti, G., Sciré, C., Aliverti, M., Genoni, M., Vitali, F., Campana, S., Claudi, R., Schipani, P., Baruffolo, A., Ben-Ami, S., Capasso, G., Cosentino, R., D'Alessio, F., D'Avanzo, P., Hershko, O., Kuncarayakti, H., Landoni, M., Munari, M., Pignata, G., Radhakrishnan, K., Rubin, A., Young, D., Achren, J., Araiza-Duran, J. A., Arcavi, I., Battaini, F., Brucalassi, A., Bruch, R., Cappellaro, E., Colapietro, M., Della Valle, M., D'Orsi, S., Gal-Yam, A., Diaz, M. Hernandez, Kotilainen, J., Causi, G. Li, Marty, L., Mattila, S., Rappaport, M., Ricci, D., Riva, M., Salasnich, B., Smartt, S., Sanchez, R. Zanmar, Stritzinger, M., and Ventura, H. Perez

Subjects
Astrophysics - Instrumentation and Methods for Astrophysics, Physics - Instrumentation and Detectors
Abstract

SOXS (Son Of X-Shooter) is a single object spectrograph built by an international consortium for the ESO NTT telescope. SOXS is based on the heritage of the X-Shooter at the ESO-VLT with two arms (UV-VIS and NIR) working in parallel, with a Resolution-Slit product of about 4500, capable of simultaneously observing over the entire band the complete spectral range from the U- to the H-band. SOXS will carry out rapid and long-term Target of Opportunity requests on a variety of astronomical objects. The SOXS vacuum and cryogenic control system has been designed to evacuate, cool down and maintain the UV-VIS detector and the entire NIR spectrograph to their operating temperatures. The design chosen allows the two arms to be operated independently. This paper describes the final design of the cryo-vacuum control system, its functionalities and the tests performed in the integration laboratories., Comment: 12 pages, 6 figures, SPIE proceedings of the conference Advances in Optical and Mechanical Technologies for Telescopes and Instrumentation IV

Published
2022

3. Formation of complex organic molecules in molecular clouds: acetaldehyde, vinyl alcohol, ketene, and ethanol via the 'energetic' processing of C$_2$H$_2$ ice

Author

Chuang, K. -J., Fedoseev, G., Scirè, C., Baratta, G. A., Jäger, C., Henning, Th., Linnartz, H., and Palumbo, M. E.

Subjects
Astrophysics - Astrophysics of Galaxies, Astrophysics - Solar and Stellar Astrophysics, Physics - Chemical Physics
Abstract

The simultaneous detection of organic molecules of the form C$_2$H$_{\text{n}}$O, such as ketene (CH$_2$CO), acetaldehyde (CH$_3$CHO), and ethanol (CH$_3$CH$_2$OH), toward early star-forming regions offers hints of shared chemical history. Several reaction routes have been proposed and experimentally verified under various interstellar conditions to explain the formation pathways involved. Most noticeably, the non-energetic processing of C$_2$H$_2$ ice with OH-radicals and H-atoms was shown to provide formation routes to ketene, acetaldehyde, ethanol, and vinyl alcohol (CH$_2$CHOH) along the H$_2$O formation sequence on grain surfaces. In this work, the non-energetic formation scheme is extended with laboratory measurements focusing on the energetic counterpart, induced by cosmic rays penetrating the H$_2$O-rich ice mantle. The focus here is on the H$^+$ radiolysis of interstellar C$_2$H$_2$:H$_2$O ice analogs at 17 K. Ultra-high vacuum experiments were performed to investigate the 200 keV H$^+$ radiolysis chemistry of predeposited C$_2$H$_2$:H$_2$O ices, both as mixed and layered geometries. Fourier-transform infrared spectroscopy was used to monitor in situ newly formed species as a function of the accumulated energy dose (or H$^+$ fluence). The infrared (IR) spectral assignments are further confirmed in isotope labeling experiments using H$_2$$^{18}$O. The energetic processing of C$_2$H$_2$:H$_2$O ice not only results in the formation of (semi-) saturated hydrocarbons (C$_2$H$_4$ and C$_2$H$_6$) and polyynes as well as cumulenes (C$_4$H$_2$ and C$_4$H$_4$), but it also efficiently forms O-bearing COMs, including vinyl alcohol, ketene, acetaldehyde, and ethanol, for which the reaction cross-section and product composition are derived. A clear composition transition of the product, from H-poor to H-rich species, is observed as a function of the accumulated energy dose., Comment: 14 pages, 7 figures, 2 tables

Published
2021
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5. Broadband spectroscopy of astrophysical ice analogs. I. Direct measurement of complex refractive index of CO ice using terahertz time-domain spectroscopy

Author

Giuliano, B. M., Gavdush, A. A., Müller, B., Zaytsev, K. I., Grassi, T., Ivlev, A. V., Palumbo, M. E., Baratta, G. A., Scirè, C., Komandin, G. A., Yurchenko, S. O., and Caselli, P.

Subjects
Astrophysics - Instrumentation and Methods for Astrophysics
Abstract

Context: Reliable, directly measured optical properties of astrophysical ice analogs in the infrared (IR) and terahertz (THz) range are missing. These parameters are of great importance to model the dust continuum radiative transfer in dense and cold regions, here thick ice mantles are present, and are necessary for the interpretation of future observations planned in the far-IR region. Aims: Coherent THz radiation allows direct measurement of the complex dielectric function (refractive index) of astrophysically relevant ice species in the THz range. Methods: The time-domain waveforms and the frequency-domain spectra of reference samples of CO ice, deposited at a temperature of 28.5 K and annealed to 33 K at different thicknesses, have been recorded. A new algorithm is developed to reconstruct the real and imaginary parts of the refractive index from the time-domain THz data. Results: The complex refractive index in the wavelength range of 1 mm - 150 ${\mu}$m (0.3 - 2.0 THz) has been determined for the studied ice samples, and compared with available data found in the literature. Conclusions: The developed algorithm of reconstructing the real and imaginary parts of the refractive index from the time-domain THz data enables, for the first time, the determination of optical properties of astrophysical ice analogs without using the Kramers-Kronig relations. The obtained data provide a benchmark to interpret the observational data from current ground based facilities as well as future space telescope missions, and have been used to estimate the opacities of the dust grains in presence of CO ice mantles.

Published
2019
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6. Characterization of the silicon+6LiF thermal neutron detection technique

Author

Pappalardo, A., Barbagallo, M., Cosentino, L., Marchetta, C., Musumarra, A., Scirè, C., Scirè, S., Vecchio, G., and Finocchiaro, P.

Subjects
Physics - Instrumentation and Detectors
Abstract

The worldwide need to replace 3He for the neutron detection has triggered R&D on new technologies and methods. A promising one is based on commercial solid state silicon detectors coupled with thin neutron converter layers containing 6Li. After proving the feasibility of this technique, we characterized the behavior of such a detector with different converter layer thicknesses. In this paper we also disentangle other contributions to the overall spectrum shape observed with this kind of detector, proving that its detection efficiency can be made reasonably high and that the gamma/neutron discrimination capability is comparable to the one of 3He tubes., Comment: Submitted to Nuclear Instruments and Methods in Physics Research, section A

Published
2015
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7. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products

Author

Talarico, R, Ramirez, G, Barreira, S, Cardamone, C, Triggianese, P, Aguilera, S, Andersen, J, Avcin, T, Benistan, K, Bertsias, G, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Callens, S, Carreira, P, Cervera, R, Cutolo, M, Damian, L, Della-Torre, E, Faria, R, Fonseca, J, Galetti, I, Hachulla, E, Iaccarino, L, Jacobsen, S, Khmelinskii, N, Limper, M, Marinello, D, Meyer, A, Moroncini, G, Nagy, G, Olesinska, M, Pamfil, C, Pileckyte, M, Pistello, M, Rednic, S, Richez, C, Romao, V, Schneider, M, Sciascia, S, Scire, C, Simonini, G, Smith, V, Sulli, A, Tani, C, Tas, S, Tincani, A, Vonk, M, Tektonidou, M, Mosca, M, Talarico R., Ramirez G. A., Barreira S. C., Cardamone C., Triggianese P., Aguilera S., Andersen J., Avcin T., Benistan K., Bertsias G., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Callens S., Carreira P. E., Cervera R., Cutolo M., Damian L., Della-Torre E., Faria R., Fonseca J. E., Galetti I., Hachulla E., Iaccarino L., Jacobsen S., Khmelinskii N., Limper M., Marinello D., Meyer A., Moroncini G., Nagy G., Olesinska M., Pamfil C., Pileckyte M., Pistello M., Rednic S., Richez C., Romao V. C., Schneider M., Sciascia S., Scire C. A., Simonini G., Smith V., Sulli A., Tani C., Tas S. W., Tincani A., Vonk M. C., Tektonidou M., Mosca M., Talarico, R, Ramirez, G, Barreira, S, Cardamone, C, Triggianese, P, Aguilera, S, Andersen, J, Avcin, T, Benistan, K, Bertsias, G, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Callens, S, Carreira, P, Cervera, R, Cutolo, M, Damian, L, Della-Torre, E, Faria, R, Fonseca, J, Galetti, I, Hachulla, E, Iaccarino, L, Jacobsen, S, Khmelinskii, N, Limper, M, Marinello, D, Meyer, A, Moroncini, G, Nagy, G, Olesinska, M, Pamfil, C, Pileckyte, M, Pistello, M, Rednic, S, Richez, C, Romao, V, Schneider, M, Sciascia, S, Scire, C, Simonini, G, Smith, V, Sulli, A, Tani, C, Tas, S, Tincani, A, Vonk, M, Tektonidou, M, Mosca, M, Talarico R., Ramirez G. A., Barreira S. C., Cardamone C., Triggianese P., Aguilera S., Andersen J., Avcin T., Benistan K., Bertsias G., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Callens S., Carreira P. E., Cervera R., Cutolo M., Damian L., Della-Torre E., Faria R., Fonseca J. E., Galetti I., Hachulla E., Iaccarino L., Jacobsen S., Khmelinskii N., Limper M., Marinello D., Meyer A., Moroncini G., Nagy G., Olesinska M., Pamfil C., Pileckyte M., Pistello M., Rednic S., Richez C., Romao V. C., Schneider M., Sciascia S., Scire C. A., Simonini G., Smith V., Sulli A., Tani C., Tas S. W., Tincani A., Vonk M. C., Tektonidou M., and Mosca M.

Abstract

Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP

Published
2023

8. Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC)

Author

Quartuccio, L, Bortoluzzi, A, Scire, C, Marangoni, A, Del Frate, G, Treppo, E, Castelnovo, L, Saccardo, F, Zani, R, Candela, M, Fraticelli, P, Mazzaro, C, Renoldi, P, Scaini, P, Filippini, D, Visentini, M, Scarpato, S, Giuggioli, D, Mascia, M, Sebastiani, M, Zignego, A, Lauletta, G, Fiorilli, M, Casato, M, Ferri, C, Pietrogrande, M, Pioltelli, P, De Vita, S, Monti, G, Galli, M, Quartuccio L., Bortoluzzi A., Scire C. A., Marangoni A., Del Frate G., Treppo E., Castelnovo L., Saccardo F., Zani R., Candela M., Fraticelli P., Mazzaro C., Renoldi P., Scaini P., Filippini D. A., Visentini M., Scarpato S., Giuggioli D., Mascia M. T., Sebastiani M., Zignego A. L., Lauletta G., Fiorilli M., Casato M., Ferri C., Pietrogrande M., Pioltelli P. E., De Vita S., Monti G., Galli M., Quartuccio, L, Bortoluzzi, A, Scire, C, Marangoni, A, Del Frate, G, Treppo, E, Castelnovo, L, Saccardo, F, Zani, R, Candela, M, Fraticelli, P, Mazzaro, C, Renoldi, P, Scaini, P, Filippini, D, Visentini, M, Scarpato, S, Giuggioli, D, Mascia, M, Sebastiani, M, Zignego, A, Lauletta, G, Fiorilli, M, Casato, M, Ferri, C, Pietrogrande, M, Pioltelli, P, De Vita, S, Monti, G, Galli, M, Quartuccio L., Bortoluzzi A., Scire C. A., Marangoni A., Del Frate G., Treppo E., Castelnovo L., Saccardo F., Zani R., Candela M., Fraticelli P., Mazzaro C., Renoldi P., Scaini P., Filippini D. A., Visentini M., Scarpato S., Giuggioli D., Mascia M. T., Sebastiani M., Zignego A. L., Lauletta G., Fiorilli M., Casato M., Ferri C., Pietrogrande M., Pioltelli P. E., De Vita S., Monti G., and Galli M.

Abstract

Cryoglobulinemic vasculitis (CV) or mixed cryoglobulinemic syndrome (MCS) is a systemic small-vessel vasculitis characterized by the proliferation of B-cell clones producing pathogenic immune complexes, called cryoglobulins. It is often secondary to hepatitis C virus (HCV), autoimmune diseases, and hematological malignancies. CV usually has a mild benign clinical course, but severe organ damage and life-threatening manifestations can occur. Recently, evidence in favor of rituximab (RTX), an anti-CD 20 monoclonal antibody, is emerging in CV: nevertheless, questions upon the safety of this therapeutic approach, especially in HCV patients, are still being issued and universally accepted recommendations that can help physicians in MCS treatment are lacking. A Consensus Committee provided a prioritized list of research questions to perform a systematic literature review (SLR). A search was made in Medline, Embase, and Cochrane library, updated to August 2021. Of 1227 article abstracts evaluated, 27 studies were included in the SLR, of which one SLR, 4 RCTs, and 22 observational studies. Seventeen recommendations for the management of mixed cryoglobulinemia with rituximab from the Italian Study Group of Cryoglobulinemia (GISC) were developed to give a valuable tool to the physician approaching RTX treatment in CV.

Published
2023

9. Predictors of disease activity in gout: a 12-month analysis of the ATTACk (Achieving improvement in the management of crystal-induced arthritis) multicentre cohort study

Author

Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Bortoluzzi, A, Ceccarelli, F, Lucchetti, R, Furini, F, Del Ross, T, Zanetti, A, Carrara, G, Scire, C, Doria, A, Ramonda, R, Lorenzin M., Ughi N., Ariani A., Raffeiner B., Frallonardo P., Hoxha A., Ortolan A., Favero M., Parisi S., Bortoluzzi A., Ceccarelli F., Lucchetti R., Furini F., Del Ross T., Zanetti A., Carrara G., Scire C. A., Doria A., Ramonda R., Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Bortoluzzi, A, Ceccarelli, F, Lucchetti, R, Furini, F, Del Ross, T, Zanetti, A, Carrara, G, Scire, C, Doria, A, Ramonda, R, Lorenzin M., Ughi N., Ariani A., Raffeiner B., Frallonardo P., Hoxha A., Ortolan A., Favero M., Parisi S., Bortoluzzi A., Ceccarelli F., Lucchetti R., Furini F., Del Ross T., Zanetti A., Carrara G., Scire C. A., Doria A., and Ramonda R.

Abstract

Objective Gout treatment is largely suboptimal in clinical practice. We aimed to assess the predictors of disease-activity at 12 months in a real-life setting. Methods Consecutive patients referred to Rheumatology Units for suspected acute crystal-induced arthritis were enrolled in a multicentre-cohort study. Only patients with clinical diagnosis of gout were eligible. Disease-activity was evaluated by the Patient Acceptable Symptom State (PASS) on a visual analogue scale (VAS, 0=unsatisfactory, 100=satisfactory) at 0 (T0) and 12 months (T12), and the composite score called Gout Activity Score (GAS) calculated on the number of arthritic attacks (flare count), serum uric acid (sUA), cumulative number of tophi, VAS (T12), PtGA (T12). Multivariate linear regression model was performed to assess predictors of gout disease-activity at T12 with PASS and GAS as outcomes. Results: 201 patients had gout (diagnosis on synovial fluid in 45%, tophi in 26%, mean sUA 7.4±1.9 mg/L, 85% with urate-lowering therapy (ULT) in progress/initiated at T0); mean age 63±13 years, 88% men, median (interquartile range) disease duration 2.9 years (0.7-9.4). Follow-up visits were performed in 113 (56%) patients at T12. Mean PASS observed at T0 and at T12 were 38±27 and 74±23, respectively, whereas GAS at T12 was 10±8. A significant association was observed between the presence of tophi and PASS at T12 (-15.3, 95% CI -25.5, -5.2; p=0.003) and GAS at T12 (+4.0, 95% CI 0.6,7.4; p=0.02), adjusted for age, sex, disease duration, sUA <6 mg/dL, tender joint count, PASS at T0, ULT). CONCLUSIONS:The baseline presence of tophi may predict high disease-activity at T12, thus worsening GAS and patients’ pain perception.

Published
2023

10. Similarities and differences between younger and older disease onset patients with newly diagnosed systemic lupus erythematosus

Author

Prevete, I, Iuliano, A, Cauli, A, Piga, M, Iannone, F, Coladonato, L, Bortoluzzi, A, Silvagni, E, Tani, C, Elefante, E, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Frediani, B, Garcia, E, Scire, C, Zanetti, A, Rozza, D, Carrara, G, Sebastiani, G, Prevete I., Iuliano A., Cauli A., Piga M., Iannone F., Coladonato L., Bortoluzzi A., Silvagni E., Tani C., Elefante E., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Frediani B., Garcia E. G., Scire C. A., Zanetti A., Rozza D., Carrara G., Sebastiani G. D., Prevete, I, Iuliano, A, Cauli, A, Piga, M, Iannone, F, Coladonato, L, Bortoluzzi, A, Silvagni, E, Tani, C, Elefante, E, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Frediani, B, Garcia, E, Scire, C, Zanetti, A, Rozza, D, Carrara, G, Sebastiani, G, Prevete I., Iuliano A., Cauli A., Piga M., Iannone F., Coladonato L., Bortoluzzi A., Silvagni E., Tani C., Elefante E., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Frediani B., Garcia E. G., Scire C. A., Zanetti A., Rozza D., Carrara G., and Sebastiani G. D.

Abstract

Objective Several studies show that age at onset has an impact on the clinical-serological presentation, comorbidities and disease course of patients with systemic lupus erythematosus (SLE). We evaluated whether, in patients with recent onset SLE, the age at onset correlates with clinical-serological manifestations and with comorbidities. Methods We analysed 171 patients with a SLE diagnosis obtained within 12 months of diagnosis enrolled in the Early Lupus project. Based on the age of onset of the first disease symptom, they were stratified into 2 groups: early onset (18–45 years) and late onset (>45 years). The analysis was replicated by stratifying patients based on age at diagnosis (fulfillment of ACR classification criteria). Each comparison was made at baseline and at 36 months of follow-up. Results Baseline: patients with late onset displayed comorbidities (hypertension, dyslipidaemia and osteoporosis) more frequently than early onset group. 11.4% of late onset patients had a malignancy in medical history, not recorded in the early onset cohort. The two groups differed neither in organ involvement (domain BILAG) nor in disease activity (ECLAM). Patients with early onset showed a disease with signs of higher serologic activity (higher frequency of anti-dsDNA positivity and lower mean C3 and C4 levels) and had malar rash more frequently than the late onset group (36.2% vs. 18.2%, p=0.042). Similar results were obtained by stratifying patients by age of diagnosis (18-45 years and >45 years), except for the higher frequency of discoid rash in the group with age at diagnosis >45 years (18% vs. 6.6%, p=0.045). 36 months: the 2 groups of patients independently of the stratification applied did not differ in the accumulation of damage, but showed a different pattern of 8 organ involvement. Musculoskeletal involvement was more frequent both in the late onset group (18.6% vs. 7.3%, p=0.043) and in the group with age at diagnosis >45 years (20.4% vs. 5.9%, p

Published
2023

11. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author

De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., and Rossella T.

Abstract

Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. Methods Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets. Results Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). Conclusion The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.

Published
2023

12. Reliability assessment of ultrasound muscle echogenicity in patients with rheumatic diseases: Results of a multicenter international web-based study

Author

Di Matteo, A, Moscioni, E, Lommano, M, Cipolletta, E, Smerilli, G, Farah, S, Airoldi, C, Aydin, S, Becciolini, A, Bonfiglioli, K, Carotti, M, Carrara, G, Cazenave, T, Corradini, D, Cosatti, M, de Agustin, J, Destro Castaniti, G, Di Carlo, M, Di Donato, E, Di Geso, L, Elliott, A, Fodor, D, Francioso, F, Gabba, A, Hernandez-Diaz, C, Horvath, R, Hurnakova, J, Jesus, D, Marin, J, Martire, M, Mashadi Mirza, R, Massarotti, M, Musca, A, Nair, J, Okano, T, Papalopoulos, I, Rosa, J, Rosemffet, M, Rovisco, J, Rozza, D, Salaffi, F, Scioscia, C, Scire, C, Tamas, M, Tanimura, S, Ventura-Rios, L, Villota-Eraso, C, Villota, O, Voulgari, P, Vreju, F, Vukatana, G, Hereter, J, Zanetti, A, Grassi, W, Filippucci, E, Di Matteo A., Moscioni E., Lommano M. G., Cipolletta E., Smerilli G., Farah S., Airoldi C., Aydin S. Z., Becciolini A., Bonfiglioli K., Carotti M., Carrara G., Cazenave T., Corradini D., Cosatti M. A., de Agustin J. J., Destro Castaniti G. M., Di Carlo M., Di Donato E., Di Geso L., Elliott A., Fodor D., Francioso F., Gabba A., Hernandez-Diaz C., Horvath R., Hurnakova J., Jesus D., Marin J., Martire M. V., Mashadi Mirza R., Massarotti M., Musca A. A., Nair J., Okano T., Papalopoulos I., Rosa J., Rosemffet M., Rovisco J., Rozza D., Salaffi F., Scioscia C., Scire C. A., Tamas M. -M., Tanimura S., Ventura-Rios L., Villota-Eraso C., Villota O., Voulgari P. V., Vreju F. A., Vukatana G., Hereter J. Z., Zanetti A., Grassi W., Filippucci E., Di Matteo, A, Moscioni, E, Lommano, M, Cipolletta, E, Smerilli, G, Farah, S, Airoldi, C, Aydin, S, Becciolini, A, Bonfiglioli, K, Carotti, M, Carrara, G, Cazenave, T, Corradini, D, Cosatti, M, de Agustin, J, Destro Castaniti, G, Di Carlo, M, Di Donato, E, Di Geso, L, Elliott, A, Fodor, D, Francioso, F, Gabba, A, Hernandez-Diaz, C, Horvath, R, Hurnakova, J, Jesus, D, Marin, J, Martire, M, Mashadi Mirza, R, Massarotti, M, Musca, A, Nair, J, Okano, T, Papalopoulos, I, Rosa, J, Rosemffet, M, Rovisco, J, Rozza, D, Salaffi, F, Scioscia, C, Scire, C, Tamas, M, Tanimura, S, Ventura-Rios, L, Villota-Eraso, C, Villota, O, Voulgari, P, Vreju, F, Vukatana, G, Hereter, J, Zanetti, A, Grassi, W, Filippucci, E, Di Matteo A., Moscioni E., Lommano M. G., Cipolletta E., Smerilli G., Farah S., Airoldi C., Aydin S. Z., Becciolini A., Bonfiglioli K., Carotti M., Carrara G., Cazenave T., Corradini D., Cosatti M. A., de Agustin J. J., Destro Castaniti G. M., Di Carlo M., Di Donato E., Di Geso L., Elliott A., Fodor D., Francioso F., Gabba A., Hernandez-Diaz C., Horvath R., Hurnakova J., Jesus D., Marin J., Martire M. V., Mashadi Mirza R., Massarotti M., Musca A. A., Nair J., Okano T., Papalopoulos I., Rosa J., Rosemffet M., Rovisco J., Rozza D., Salaffi F., Scioscia C., Scire C. A., Tamas M. -M., Tanimura S., Ventura-Rios L., Villota-Eraso C., Villota O., Voulgari P. V., Vreju F. A., Vukatana G., Hereter J. Z., Zanetti A., Grassi W., and Filippucci E.

Abstract

Objectives: To investigate the inter/intra-reliability of ultrasound (US) muscle echogenicity in patients with rheumatic diseases. Methods: Forty-two rheumatologists and 2 radiologists from 13 countries were asked to assess US muscle echogenicity of quadriceps muscle in 80 static images and 20 clips from 64 patients with different rheumatic diseases and 8 healthy subjects. Two visual scales were evaluated, a visual semi-quantitative scale (0–3) and a continuous quantitative measurement (“VAS echogenicity,” 0–100). The same assessment was repeated to calculate intra-observer reliability. US muscle echogenicity was also calculated by an independent research assistant using a software for the analysis of scientific images (ImageJ). Inter and intra reliabilities were assessed by means of prevalence-adjusted bias-adjusted Kappa (PABAK), intraclass correlation coefficient (ICC) and correlations through Kendall’s Tau and Pearson’s Rho coefficients. Results: The semi-quantitative scale showed a moderate inter-reliability [PABAK = 0.58 (0.57–0.59)] and a substantial intra-reliability [PABAK = 0.71 (0.68–0.73)]. The lowest inter and intra-reliability results were obtained for the intermediate grades (i.e., grade 1 and 2) of the semi-quantitative scale. “VAS echogenicity” showed a high reliability both in the inter-observer [ICC = 0.80 (0.75–0.85)] and intra-observer [ICC = 0.88 (0.88–0.89)] evaluations. A substantial association was found between the participants assessment of the semi-quantitative scale and “VAS echogenicity” [ICC = 0.52 (0.50–0.54)]. The correlation between these two visual scales and ImageJ analysis was high (tau = 0.76 and rho = 0.89, respectively). Conclusion: The results of this large, multicenter study highlighted the overall good inter and intra-reliability of the US assessment of muscle echogenicity in patients with different rheumatic diseases.

Published
2023

13. Treatment with COLchicine in hospitalized patients affected by COVID-19: The COLVID-19 trial

Author

Perricone, C, Scarsi, M, Brucato, A, Pisano, P, Pigatto, E, Becattini, C, Cingolani, A, Tiso, F, Prota, R, Tomasoni, L, Cutolo, M, Tardella, M, Rozza, D, Zerbino, C, Andreoni, M, Poletti, V, Bartoloni, E, Gerli, R, Cafaro, G, Mendeni, M, Colombo, E, Medico, M, Cabras, P, Schiesaro, M, Franco, L, Fantoni, M, Friso, L, Gallo, V, Franceschini, F, Paolino, S, Salaffi, F, Scire, C, Zanetti, A, Diana, C, Passaro, A, Foti, R, Serino, F, Cassol, M, Bucaneve, G, Rocchi, R, Perricone C., Scarsi M., Brucato A., Pisano P., Pigatto E., Becattini C., Cingolani A., Tiso F., Prota R., Tomasoni L. R., Cutolo M., Tardella M., Rozza D., Zerbino C., Andreoni M., Poletti V., Bartoloni E., Gerli R., Cafaro G., Mendeni M., Colombo E., Medico M. D., Cabras P., Schiesaro M. G., Franco L., Fantoni M., Friso L., Gallo V., Franceschini F., Paolino S., Salaffi F., Scire C. A., Zanetti A., Diana C., Passaro A., Foti R., Serino F. S., Cassol M., Bucaneve G., Rocchi R. E., Perricone, C, Scarsi, M, Brucato, A, Pisano, P, Pigatto, E, Becattini, C, Cingolani, A, Tiso, F, Prota, R, Tomasoni, L, Cutolo, M, Tardella, M, Rozza, D, Zerbino, C, Andreoni, M, Poletti, V, Bartoloni, E, Gerli, R, Cafaro, G, Mendeni, M, Colombo, E, Medico, M, Cabras, P, Schiesaro, M, Franco, L, Fantoni, M, Friso, L, Gallo, V, Franceschini, F, Paolino, S, Salaffi, F, Scire, C, Zanetti, A, Diana, C, Passaro, A, Foti, R, Serino, F, Cassol, M, Bucaneve, G, Rocchi, R, Perricone C., Scarsi M., Brucato A., Pisano P., Pigatto E., Becattini C., Cingolani A., Tiso F., Prota R., Tomasoni L. R., Cutolo M., Tardella M., Rozza D., Zerbino C., Andreoni M., Poletti V., Bartoloni E., Gerli R., Cafaro G., Mendeni M., Colombo E., Medico M. D., Cabras P., Schiesaro M. G., Franco L., Fantoni M., Friso L., Gallo V., Franceschini F., Paolino S., Salaffi F., Scire C. A., Zanetti A., Diana C., Passaro A., Foti R., Serino F. S., Cassol M., Bucaneve G., and Rocchi R. E.

Abstract

Objective: To evaluate whether the addition of colchicine to standard of care (SOC) results in better outcomes in hospitalized patients with COVID-19. Design: This interventional, multicenter, randomized, phase 2 study, evaluated colchicine 1.5 mg/day added to SOC in hospitalized COVID-19 patients (COLVID-19 trial) and 227 patients were recruited. The primary outcome was the rate of critical disease in 30 days defined as need of mechanical ventilation, intensive care unit (ICU), or death. Results: 152 non-anti-SARS-CoV-2-vaccinated patients (colchicine vs controls: 77vs75, mean age 69.1±13.1 vs 67.9±15 years, 39% vs 33.3% females, respectively) were analyzed. There was no difference in co-primary end-points between patients treated with colchicine compared to controls (mechanical ventilation 5.2% vs 4%, ICU 1.3% vs 5.3%, death 9.1% vs 6.7%, overall 11 (14.3%) vs 10 (13.3%) patients, P=ns, respectively). Mean time to discharge was similar (colchicine vs controls 14.1±10.4 vs 14.7±8.1 days). Older age (>60 years, P=0.025), P/F<275 mmHg (P=0.005), AST>40 U/L (P<0.001), pre-existent heart (P=0.02), lung (P=0.003), upper-gastrointestinal (P=0.014), lower-gastrointestinal diseases (P=0.009) and cancer (P=0.008) were predictive of achieving the primary outcome. Diarrhoea (9.1% vs 0%, p=0.0031) and increased levels of AST at 6 days (76.9±91.8 vs 33.5±20.7 U/l, P=0.016) were more frequent in the colchicine group. Conclusion: Colchicine did not reduce the rate and the time to the critical stage. Colchicine was relatively safe although adverse hepatic effects require caution. We confirm that older (>60 years) patients with comorbidities are characterized by worse outcome.

Published
2023

14. The provisional OMERACT ultrasonography score for giant cell arteritis

Author

Dejaco, C, Ponte, C, Monti, S, Rozza, D, Scire, C, Terslev, L, Bruyn, G, Boumans, D, Hartung, W, Hocevar, A, Milchert, M, Dohn, U, Mukhtyar, C, Aschwanden, M, Bosch, P, Camellino, D, Chrysidis, S, Ciancio, G, D'Agostino, M, Daikeler, T, Dasgupta, B, De Miguel, E, Diamantopoulos, A, Duftner, C, Agueda, A, Fredberg, U, Hanova, P, Hansen, I, Hauge, E, Iagnocco, A, Inanc, N, Juche, A, Karalilova, R, Kawamoto, T, Keller, K, Keen, H, Kermani, T, Kohler, M, Koster, M, Luqmani, R, Macchioni, P, Mackie, S, Naredo, E, Nielsen, B, Ogasawara, M, Pineda, C, Schafer, V, Seitz, L, Tomelleri, A, Torralba, K, Van Der Geest, K, Warrington, K, Schmidt, W, Dejaco C., Ponte C., Monti S., Rozza D., Scire C. A., Terslev L., Bruyn G. A. W., Boumans D., Hartung W., Hocevar A., Milchert M., Dohn U. Mo., Mukhtyar C. B., Aschwanden M., Bosch P., Camellino D., Chrysidis S., Ciancio G., D'Agostino M. A., Daikeler T., Dasgupta B., De Miguel E., Diamantopoulos A. P., Duftner C., Agueda A., Fredberg U., Hanova P., Hansen I. To., Hauge E. -M., Iagnocco A., Inanc N., Juche A., Karalilova R., Kawamoto T., Keller K. K., Keen H. I., Kermani T. A., Kohler M. J., Koster M., Luqmani R. A., Macchioni P., Mackie S. L., Naredo E., Nielsen B. D., Ogasawara M., Pineda C., Schafer V. S., Seitz L., Tomelleri A., Torralba K. D., Van Der Geest K. S. M., Warrington K. J., Schmidt W. A., Dejaco, C, Ponte, C, Monti, S, Rozza, D, Scire, C, Terslev, L, Bruyn, G, Boumans, D, Hartung, W, Hocevar, A, Milchert, M, Dohn, U, Mukhtyar, C, Aschwanden, M, Bosch, P, Camellino, D, Chrysidis, S, Ciancio, G, D'Agostino, M, Daikeler, T, Dasgupta, B, De Miguel, E, Diamantopoulos, A, Duftner, C, Agueda, A, Fredberg, U, Hanova, P, Hansen, I, Hauge, E, Iagnocco, A, Inanc, N, Juche, A, Karalilova, R, Kawamoto, T, Keller, K, Keen, H, Kermani, T, Kohler, M, Koster, M, Luqmani, R, Macchioni, P, Mackie, S, Naredo, E, Nielsen, B, Ogasawara, M, Pineda, C, Schafer, V, Seitz, L, Tomelleri, A, Torralba, K, Van Der Geest, K, Warrington, K, Schmidt, W, Dejaco C., Ponte C., Monti S., Rozza D., Scire C. A., Terslev L., Bruyn G. A. W., Boumans D., Hartung W., Hocevar A., Milchert M., Dohn U. Mo., Mukhtyar C. B., Aschwanden M., Bosch P., Camellino D., Chrysidis S., Ciancio G., D'Agostino M. A., Daikeler T., Dasgupta B., De Miguel E., Diamantopoulos A. P., Duftner C., Agueda A., Fredberg U., Hanova P., Hansen I. To., Hauge E. -M., Iagnocco A., Inanc N., Juche A., Karalilova R., Kawamoto T., Keller K. K., Keen H. I., Kermani T. A., Kohler M. J., Koster M., Luqmani R. A., Macchioni P., Mackie S. L., Naredo E., Nielsen B. D., Ogasawara M., Pineda C., Schafer V. S., Seitz L., Tomelleri A., Torralba K. D., Van Der Geest K. S. M., Warrington K. J., and Schmidt W. A.

Abstract

Objectives To develop an Outcome Measures in Rheumatology (OMERACT) ultrasonography score for monitoring disease activity in giant cell arteritis (GCA) and evaluate its metric properties. Methods The OMERACT Instrument Selection Algorithm was followed. Forty-nine members of the OMERACT ultrasonography large vessel vasculitis working group were invited to seven Delphi rounds. An online reliability exercise was conducted using images of bilateral common temporal arteries, parietal and frontal branches as well as axillary arteries from 16 patients with GCA and 7 controls. Sensitivity to change and convergent construct validity were tested using data from a prospective cohort of patients with new GCA in which ultrasound-based intima-media thickness (IMT) measurements were conducted at weeks 1, 3, 6, 12 and 24. Results Agreement was obtained (92.7%) for the OMERACT GCA Ultrasonography Score (OGUS), calculated as follows: sum of IMT measured in every segment divided by the rounded cut-off values of IMTs in each segment. The resulting value is then divided by the number of segments available. Thirty-five members conducted the reliability exercise, the interrater intraclass correlation coefficient (ICC) for the OGUS was 0.72-0.84 and the median intrareader ICC was 0.91. The prospective cohort consisted of 52 patients. Sensitivity to change between baseline and each follow-up visit up to week 24 yielded standardised mean differences from -1.19 to -2.16, corresponding to large and very large magnitudes of change, respectively. OGUS correlated moderately with erythrocyte sedimentation rate, C reactive protein and Birmingham Vasculitis Activity Score (corr coeff 0.37-0.48). Conclusion We developed a provisional OGUS for potential use in clinical trials.

Published
2023

15. Evaluation of the Synovial Effects of Biological and Targeted Synthetic DMARDs in Patients with Psoriatic Arthritis: A Systematic Literature Review and Meta-Analysis

Author

Ciliento, M, Venturelli, V, Schettini, N, Bertola, R, Garaffoni, C, Lanza, G, Gafa, R, Borghi, A, Corazza, M, Zabotti, A, Missiroli, S, Boncompagni, C, Patergnani, S, Perrone, M, Giorgi, C, Pinton, P, Govoni, M, Scire, C, Bortoluzzi, A, Silvagni, E, Ciliento M. S., Venturelli V., Schettini N., Bertola R., Garaffoni C., Lanza G., Gafa R., Borghi A., Corazza M., Zabotti A., Missiroli S., Boncompagni C., Patergnani S., Perrone M., Giorgi C., Pinton P., Govoni M., Scire C. A., Bortoluzzi A., Silvagni E., Ciliento, M, Venturelli, V, Schettini, N, Bertola, R, Garaffoni, C, Lanza, G, Gafa, R, Borghi, A, Corazza, M, Zabotti, A, Missiroli, S, Boncompagni, C, Patergnani, S, Perrone, M, Giorgi, C, Pinton, P, Govoni, M, Scire, C, Bortoluzzi, A, Silvagni, E, Ciliento M. S., Venturelli V., Schettini N., Bertola R., Garaffoni C., Lanza G., Gafa R., Borghi A., Corazza M., Zabotti A., Missiroli S., Boncompagni C., Patergnani S., Perrone M., Giorgi C., Pinton P., Govoni M., Scire C. A., Bortoluzzi A., and Silvagni E.

Abstract

The aims of this systematic literature review (SLR) were to identify the effects of approved biological and targeted synthetic disease modifying antirheumatic drugs (b/tsDMARDs) on synovial membrane of psoriatic arthritis (PsA) patients, and to determine the existence of histological/molecular biomarkers of response to therapy. A search was conducted on MEDLINE, Embase, Scopus, and Cochrane Library (PROSPERO:CRD42022304986) to retrieve data on longitudinal change of biomarkers in paired synovial biopsies and in vitro studies. A meta-analysis was conducted by adopting the standardized mean difference (SMD) as a measure of the effect. Twenty-two studies were included (19 longitudinal, 3 in vitro). In longitudinal studies, TNF inhibitors were the most used drugs, while, for in vitro studies, JAK inhibitors or adalimumab/secukinumab were assessed. The main technique used was immunohistochemistry (longitudinal studies). The meta-analysis showed a significant reduction in both CD3+ lymphocytes (SMD −0.85 [95% CI −1.23; −0.47]) and CD68+ macrophages (sublining, sl) (SMD −0.74 [−1.16; −0.32]) in synovial biopsies from patients treated for 4–12 weeks with bDMARDs. Reduction in CD3+ mostly correlated with clinical response. Despite heterogeneity among the biomarkers evaluated, the reduction in CD3+/CD68+sl cells during the first 3 months of treatment with TNF inhibitors represents the most consistent variation reported in the literature.

Published
2023

16. Which are the most frequently involved peripheral joints in calcium pyrophosphate crystal deposition at imaging? A systematic literature review and meta-analysis by the OMERACT ultrasound – CPPD subgroup

Author

Adinolfi, A, Sirotti, S, Sakellariou, G, Cipolletta, E, Filippucci, E, Porta, F, Zanetti, A, Ughi, N, Sarzi-Puttini, P, Scire, C, Keen, H, Pineda, C, Terslev, L, D'Agostino, M, Filippou, G, Adinolfi A., Sirotti S., Sakellariou G., Cipolletta E., Filippucci E., Porta F., Zanetti A., Ughi N., Sarzi-Puttini P., Scire C. A., Keen H., Pineda C., Terslev L., D'Agostino M. A., Filippou G., Adinolfi, A, Sirotti, S, Sakellariou, G, Cipolletta, E, Filippucci, E, Porta, F, Zanetti, A, Ughi, N, Sarzi-Puttini, P, Scire, C, Keen, H, Pineda, C, Terslev, L, D'Agostino, M, Filippou, G, Adinolfi A., Sirotti S., Sakellariou G., Cipolletta E., Filippucci E., Porta F., Zanetti A., Ughi N., Sarzi-Puttini P., Scire C. A., Keen H., Pineda C., Terslev L., D'Agostino M. A., and Filippou G.

Abstract

Objectives: To identify the prevalence of calcium pyrophosphate crystal deposition (CPPD) using ultrasound and conventional radiology at peripheral joints in patients with suspected or definite CPPD. Methods: A systematic literature search was performed in PubMed and Embase using pre-defined search strategies from inception to April 2021 to identify studies that evaluated conventional radiology and ultrasound in detecting CPPD at peripheral joints, including definite or suspected CPPD [Research question 1 (RQ1) and Research Question 2 (RQ2), respectively]. For the meta-analysis, the first, second, and third sub-analysis included studies with the knee, and knee or wrist as the index joint for CPPD (without restrictions on the reference standard) and synovial fluid analysis or histology as a reference standard (without restrictions on the index joint), respectively. Results: One-thousand eight hundred and twenty-seven manuscripts were identified, of which 94 articles were finally included. Twenty-two and seventy-two papers were included in RQ1 and RQ2, respectively. The knee had the highest prevalence for RQ1 and RQ2 by both conventional radiology and ultrasound, followed by the wrist with the highest prevalence for RQ1. The hand had the lowest CPPD prevalence. The third sub-analysis showed a higher CPPD prevalence on ultrasound than conventional radiology at the knee (only data available). Conclusion: Among all peripheral joints, the knees and wrists could be regarded as the target joints for CPPD detection by imaging. Furthermore, ultrasound seems to detect a higher number of calcium pyrophosphate deposits than conventional radiology, even when using a more restrictive reference standard.

Published
2023

17. Reliability and diagnostic accuracy of radiography for the diagnosis of calcium pyrophosphate deposition: performance of the novel definitions developed by an international multidisciplinary working group

Author

Sirotti, S, Becce, F, Sconfienza, L, Terslev, L, Naredo, E, Zufferey, P, Pineda, C, Gutierrez, M, Adinolfi, A, Serban, T, Maccarter, D, Mouterde, G, Zanetti, A, Scanu, A, Moller, I, Novo-Rivas, U, Largo, R, Sarzi-Puttini, P, Abhishek, A, Choi, H, Dalbeth, N, Pascart, T, Tedeschi, S, D'Agostino, M, Iagnocco, A, Keen, H, Scire, C, Filippou, G, Sirotti S., Becce F., Sconfienza L. M., Terslev L., Naredo E., Zufferey P., Pineda C., Gutierrez M., Adinolfi A., Serban T., MacCarter D., Mouterde G., Zanetti A., Scanu A., Moller I., Novo-Rivas U., Largo R., Sarzi-Puttini P., Abhishek A., Choi H. K., Dalbeth N., Pascart T., Tedeschi S. K., D'Agostino M. -A., Iagnocco A., Keen H. I., Scire C. A., Filippou G., Sirotti, S, Becce, F, Sconfienza, L, Terslev, L, Naredo, E, Zufferey, P, Pineda, C, Gutierrez, M, Adinolfi, A, Serban, T, Maccarter, D, Mouterde, G, Zanetti, A, Scanu, A, Moller, I, Novo-Rivas, U, Largo, R, Sarzi-Puttini, P, Abhishek, A, Choi, H, Dalbeth, N, Pascart, T, Tedeschi, S, D'Agostino, M, Iagnocco, A, Keen, H, Scire, C, Filippou, G, Sirotti S., Becce F., Sconfienza L. M., Terslev L., Naredo E., Zufferey P., Pineda C., Gutierrez M., Adinolfi A., Serban T., MacCarter D., Mouterde G., Zanetti A., Scanu A., Moller I., Novo-Rivas U., Largo R., Sarzi-Puttini P., Abhishek A., Choi H. K., Dalbeth N., Pascart T., Tedeschi S. K., D'Agostino M. -A., Iagnocco A., Keen H. I., Scire C. A., and Filippou G.

Abstract

Objectives: To assess the reliability and diagnostic accuracy of new radiographic definitions for calcium pyrophosphate deposition (CPPD) identification, developed by an international multidisciplinary working group. Methods: Patients with knee osteoarthritis scheduled for knee replacement were enrolled. Two radiologists and two rheumatologists assessed twice the images for presence/absence of CPPD on menisci, hyaline cartilage, tendons, joint capsule, synovial membrane, using the new definitions. In case of disagreement, a consensus decision was taken and considered for the assessment of diagnostic performance. Histological examination of specimens under compensated polarized light microscopy was the reference standard. Prevalence-adjusted bias-adjusted kappa (PABAK) was used to assess the reliability. Diagnostic performance statistics were calculated. Results: Sixty-seven participants were enrolled for the reliability study. The inter-observer reliability was substantial in most of the assessed structures when considering all 4 readers (kappa range 0.59 – 0.90), substantial to almost perfect among radiologists (kappa range 0.70-0.91), and moderate to almost perfect among rheumatologists (kappa range 0.46 – 0.88). The intra-observer reliability was substantial to almost perfect for all the observers (kappa range 0.70 – 1). Fifty-one patients were enrolled for the accuracy study. Radiography demonstrated to be specific for CPPD (92%), but sensitivity remained low in all sites and in the overall diagnosis (54%). Conclusion: The new imaging definitions of CPPD are highly specific against the gold standard of histological diagnosis; when described findings are present these definitions allow for a definite diagnosis of CPPD, rather than other calcium-containing crystal depositions; instead a negative finding does not exclude the diagnosis.

Published
2023

18. Musculoskeletal ultrasound may narrow the gap between patients and physicians in the assessment of rheumatoid arthritis disease activity

Author

Floris, A, Rozza, D, Zanetti, A, Carrara, G, Bellis, E, Cauli, A, Iagnocco, A, Scire, C, Piga, M, Batticciotto, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Vinghitti, C, Mastaglio, C, Scioscia, C, Russi, D, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Filippou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Parisi, S, Sabatino, V, Picerno, V, Floris A., Rozza D., Zanetti A., Carrara G., Bellis E., Cauli A., Iagnocco A., Scire C. A., Piga M., Batticciotto A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Vinghitti C., Mastaglio C., Scioscia C., Russi D., Luccioli F., Cavatorta F., Ceccarelli F., Sakellariou G., Filippou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M., Caprioli M., Gutierrez M., Draghessi A., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Parisi S., Sabatino V. D., Picerno V., Floris, A, Rozza, D, Zanetti, A, Carrara, G, Bellis, E, Cauli, A, Iagnocco, A, Scire, C, Piga, M, Batticciotto, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Vinghitti, C, Mastaglio, C, Scioscia, C, Russi, D, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Filippou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Parisi, S, Sabatino, V, Picerno, V, Floris A., Rozza D., Zanetti A., Carrara G., Bellis E., Cauli A., Iagnocco A., Scire C. A., Piga M., Batticciotto A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Vinghitti C., Mastaglio C., Scioscia C., Russi D., Luccioli F., Cavatorta F., Ceccarelli F., Sakellariou G., Filippou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M., Caprioli M., Gutierrez M., Draghessi A., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Parisi S., Sabatino V. D., and Picerno V.

Abstract

Objectives: To investigate the association between patient-physician discordance in the assessment of disease activity and residual US synovitis/tenosynovitis in a cohort of patients with RA in clinical remission. Methods: A post hoc analysis of the STARTER study, promoted by the Musculoskeletal-US (MSUS) Study Group of the Italian Society for Rheumatology, was performed using data from 361 consecutive patients with RA in clinical remission. The global assessment of disease activity by each patient (PGA) and evaluator/physician (EGA) was recorded on a 100-mm visual analogue scale. The PGA-EGA discordance was classified as positive (PGA>EGA) or negative (PGA

Published
2023

19. Relationship between the prevalence of subclinical tenosynovitis and treatment in patients with RA in clinical remission: STARTER study

Author

Parisi, S, Zanetti, A, Carrara, G, Scire, C, Iagnocco, A, Filippou, G, Batticciotto, A, Floris, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Venditti, C, Mastaglio, C, Scioscia, C, Rossi, D, Bellis, E, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Piga, M, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Di Sabatino, V, Picerno, V, Parisi S., Zanetti A., Carrara G., Scire C. A., Iagnocco A., Filippou G., Batticciotto A., Floris A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Venditti C., Mastaglio C., Scioscia C., Rossi D., Bellis E., Luccioli F., Cavatorta F. P., Ceccarelli F., Sakellariou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M. C., Caprioli M., Gutierrez M., Draghessi A., Piga M., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Di Sabatino V., Picerno V., Parisi, S, Zanetti, A, Carrara, G, Scire, C, Iagnocco, A, Filippou, G, Batticciotto, A, Floris, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Venditti, C, Mastaglio, C, Scioscia, C, Rossi, D, Bellis, E, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Piga, M, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Di Sabatino, V, Picerno, V, Parisi S., Zanetti A., Carrara G., Scire C. A., Iagnocco A., Filippou G., Batticciotto A., Floris A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Venditti C., Mastaglio C., Scioscia C., Rossi D., Bellis E., Luccioli F., Cavatorta F. P., Ceccarelli F., Sakellariou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M. C., Caprioli M., Gutierrez M., Draghessi A., Piga M., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Di Sabatino V., and Picerno V.

Abstract

Objective: This study is a sub-analysis from the patient cohort of the STARTER (Sonographic Tenosynovitis Assessment in RheumaToid arthritis patiEnts in Remission) study. The aim was to evaluate differences in ultrasound-detected joint and/or tendon involvement between patients receiving therapies based on a combination of conventional synthetic DMARDs (csDMARDs) and biologic DMARDs (bDMARDs) and those who were treated with either csDMARDs or bDMARDs in monotherapy. Material and methods: Four hundred and twenty-seven consecutive patients with a diagnosis of RA were recruited between October 2013 and June 2014. They were divided into three subgroups based on their therapy at baseline: patients with bDMARD in monotherapy, patients with csDMARD in monotherapy and patients in combination therapy (csDMARD + bDMARD). At baseline, 6 months and 12 months, a clinical examination (28 joint count) and an ultrasound evaluation were performed in each patient. A score of grey-scale (GS) and power Doppler (PD) synovitis and tenosynovitis was calculated based on the OMERACT scoring systems. Results: Two hundred and fifty-six patients completed the observation period: 48 patients from the bDMARD group (18.75%), 152 patients from the csDMARD group (59.38%) and 56 patients from csDMARD + bDMARD group (21.88%). The analysis showed that GS tenosynovitis and PD tenosynovitis are better controlled in combination therapy than they are ith csDMARD alone (P=0.025 and P=0.047, respectively); for PD synovitis, there was a better response in those who were treated with the combination therapy when compared with the patients receiving csDMARD (P=0.01) or bDMARD (P=0.02) alone. Conclusions: The analysis showed a lower prevalence of subclinical inflammatory manifestations detected with ultrasound imaging in those patients treated with the combination therapy than in those in monotherapy.

Published
2023

20. COVID-19 prognosis in systemic lupus erythematosus compared with rheumatoid arthritis and spondyloarthritis: Results from the CONTROL-19 Study by the Italian Society for Rheumatology

Author

Scirocco, C, Ferrigno, S, Andreoli, L, Fredi, M, Lomater, C, Moroni, L, Mosca, M, Raffeiner, B, Carrara, G, Landolfi, G, Rozza, D, Zanetti, A, Scire, C, Sebastiani, G, Scirocco C., Ferrigno S., Andreoli L., Fredi M., Lomater C., Moroni L., Mosca M., Raffeiner B., Carrara G., Landolfi G., Rozza D., Zanetti A., Scire C. A., Sebastiani G. D., Scirocco, C, Ferrigno, S, Andreoli, L, Fredi, M, Lomater, C, Moroni, L, Mosca, M, Raffeiner, B, Carrara, G, Landolfi, G, Rozza, D, Zanetti, A, Scire, C, Sebastiani, G, Scirocco C., Ferrigno S., Andreoli L., Fredi M., Lomater C., Moroni L., Mosca M., Raffeiner B., Carrara G., Landolfi G., Rozza D., Zanetti A., Scire C. A., and Sebastiani G. D.

Abstract

Introduction Data concerning SARS-CoV-2 in patients affected by SLE are contradicting. The aim of this study was to investigate disease-related differences in COVID-19 prognosis of patients affected by rheumatic diseases before vaccination; we tested the hypothesis that patients with SLE may have a different outcome compared with those with rheumatoid arthritis (RA) or spondyloarthritis (SPA). Methods We analysed data from the national CONTROL-19 Database with a retrospective, observational design, including rheumatic patients affected by COVID-19. The principal outcome measure was hospitalisation with death or mechanical ventilation. Differences between SLE, RA and SPA were analysed by univariable and multivariable logistic regression models. Results We included 103 patients with SLE (88.2% female, mean age 48.9 years, 50.4% active disease), 524 patients with RA (74.4% female, mean age 60.6 years, 59.7% active disease) and 486 patients with SPA (58.1% female, mean age 53.2 years, 58% active disease). Outcome prevalence was not different between patients with SLE and those with RA (SLE 24.5%, RA 25.6%), while patients with SPA showed a more favourable outcome compared with those with SLE (SPA 15.9%); data from the multivariable analysis confirmed this result. In SLE, age >65 years (OR 17.3, CI 5.51 to 63.16, p<0.001), hypertension (OR 6.2, CI 2.37 to 17.04, p<0.001) and prednisone (PDN) use (OR 3.8, CI 1.43 to 11.39, p=0.01) were associated with severe outcomes, whereas hydroxychloroquine use was found to be protective (OR 0.3, CI 0.14 to 0.91, p=0.03). Conclusion Our data suggest that patients with SLE and RA do not show a different COVID-19 outcome, while patients with SPA have a more favourable disease course compared with those with SLE. Risk of hospitalisation with ventilation or death was associated with age >65 years, hypertension and PDN use in patients with SLE.

Published
2023

21. Novel insights into the management of rheumatoid arthritis: one year in review 2023

Author

Garaffoni, C, Adinolfi, A, Bortoluzzi, A, Filippou, G, Giollo, A, Sakellariou, G, Silvagni, E, Sirotti, S, Ughi, N, Scire, C, Garaffoni C., Adinolfi A., Bortoluzzi A., Filippou G., Giollo A., Sakellariou G., Silvagni E., Sirotti S., Ughi N., Scire C. A., Garaffoni, C, Adinolfi, A, Bortoluzzi, A, Filippou, G, Giollo, A, Sakellariou, G, Silvagni, E, Sirotti, S, Ughi, N, Scire, C, Garaffoni C., Adinolfi A., Bortoluzzi A., Filippou G., Giollo A., Sakellariou G., Silvagni E., Sirotti S., Ughi N., and Scire C. A.

Abstract

New evidence from 2022 slightly changed some perspectives for rheumatoid arthritis (RA) management. Real-world data on the efficacy and safety of disease-modifying anti-rheumatic drugs strengthened the importance of tailoring treatment decisions based on patient characteristics. Moreover, the research of response biomarkers to therapy underlined the need for precision medicine and remote care applications showed an innovative outlook that supports a patient-centred approach. New developments in vaccinations led to the release of updated guidelines and to a consistent improvement in the prevention of vaccine-preventable infections. New literature data also reconsidered drug management in RA-associated interstitial lung disease and pregnancy. In this paper, the reviewers aim to present the most relevant studies published during the last year in the field of RA management.

Published
2023

22. Optimizing Patient Care: A Systematic Review of Multidisciplinary Approaches for SLE Management

Author

Galoppini, G, Marangoni, A, Cirilli, F, Ruffilli, F, Garaffoni, C, Govoni, M, Scire, C, Silvagni, E, Bortoluzzi, A, Galoppini G., Marangoni A., Cirilli F., Ruffilli F., Garaffoni C., Govoni M., Scire C. A., Silvagni E., Bortoluzzi A., Galoppini, G, Marangoni, A, Cirilli, F, Ruffilli, F, Garaffoni, C, Govoni, M, Scire, C, Silvagni, E, Bortoluzzi, A, Galoppini G., Marangoni A., Cirilli F., Ruffilli F., Garaffoni C., Govoni M., Scire C. A., Silvagni E., and Bortoluzzi A.

Abstract

Systemic lupus erythematosus (SLE) is characterized by multisystemic clinical manifestations ranging from a relatively mild involvement to potentially life-threatening complications. Due to this complexity, a multidisciplinary (MD) approach is the best strategy for optimizing patients’ care. The main aim of this systematic literature review (SLR) was to scrutinize the published data regarding the MD approach for the management of SLE patients. The secondary objective was to evaluate the outcomes of the MD approach in SLE patients. The Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were used. We performed an SLR to retrieve articles available in English or Italian listed in PubMed, Embase, Cinahl, and Cochrane Library concerning the MD approach used in observational studies and clinical trials. Four independent reviewers performed the study selection and data collection. Of 5451 abstracts evaluated, 19 studies were included in the SLR. The MD approach was most frequently described in the context of SLE pregnancy, reported in 10 papers. MD teams were composed of a rheumatologist, except for one cohort study; a gynecologist; a psychologist; a nurse; and other health professionals. MD approaches had a positive impact on pregnancy-related complications and disease flares and improved SLE psychological impact. Although international recommendations advise an MD approach for managing SLE, our review highlighted the paucity of data supporting this strategy, with most of the available evidence on the management of SLE during pregnancy.

Published
2023

24. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author

De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, S. L., Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, E., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, G. D., Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., and Rossella, T.

Subjects
Scleroderma, Systemic, Settore MED/16 - REUMATOLOGIA, Epidemiology, Immunology, Systemic, Autoimmunity, Autoimmune Disease, Autoimmune Diseases, Scleroderma, Rheumatology, Immunology and Allergy, Seasons, Human
Abstract

ObjectiveTo describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.MethodsData involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.ResultsAmong patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud’s phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1–16.5) than lcSSc (2 years, IQR 0–7), and dcSSc (1 year, IQR 0–3) (pConclusionThe ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.

Published
2023

25. Musculoskeletal ultrasound may narrow the gap between patients and physicians in the assessment of rheumatoid arthritis disease activity

Author

Floris A., Rozza D., Zanetti A., Carrara G., Bellis E., Cauli A., Iagnocco A., Scire C. A., Piga M., Batticciotto A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Vinghitti C., Mastaglio C., Scioscia C., Russi D., Luccioli F., Cavatorta F., Ceccarelli F., Sakellariou G., Filippou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M., Caprioli M., Gutierrez M., Draghessi A., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Parisi S., Sabatino V. D., Picerno V., Floris, A, Rozza, D, Zanetti, A, Carrara, G, Bellis, E, Cauli, A, Iagnocco, A, Scire, C, Piga, M, Batticciotto, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Vinghitti, C, Mastaglio, C, Scioscia, C, Russi, D, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Filippou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Parisi, S, Sabatino, V, and Picerno, V

Subjects
rheumatoid arthritis, remission, Rheumatology, patient–physician discordance, patient global assessment, Pharmacology (medical), patient-physician discordance, ultrasonography, RA
Abstract

Objectives To investigate the association between patient–physician discordance in the assessment of disease activity and residual US synovitis/tenosynovitis in a cohort of patients with RA in clinical remission. Methods A post hoc analysis of the STARTER study, promoted by the Musculoskeletal-US (MSUS) Study Group of the Italian Society for Rheumatology, was performed using data from 361 consecutive patients with RA in clinical remission. The global assessment of disease activity by each patient (PGA) and evaluator/physician (EGA) was recorded on a 100-mm visual analogue scale. The PGA-EGA discordance was classified as positive (PGA>EGA) or negative (PGA Results The mean (s.d.) PGA and EGA scores were 6.1 (7.1) and 8.8 (12) mm, respectively, with a median (IQR) absolute difference of 4 (0–10) mm. Positive and negative discordances were recorded in 39 (10.8%) and 65(18.0%) patients, respectively. The GS-S (adjOR 1.099) and PD-S (adjOR 1.167) scores were associated with positive discordance (P Conclusions Patient–physician discordance is associated with the lack of US remission in patients with RA and may represent a further indication for MSUS.

Published
2022

26. Factors associated with severe COVID-19 in people with idiopathic inflammatory myopathy: results from the COVID-19 Global Rheumatology Alliance physician-reported registry

Author

Yeoh, S, Gianfrancesco, M, Lawson-Tovey, S, Hyrich, K, Strangfeld, A, Gossec, L, Carmona, L, Mateus, E, Schafer, M, Richez, C, Hachulla, E, Holmqvist, M, Scire, C, Lorenz, H, Voll, R, Hasseli, R, Jayatilleke, A, Hsu, T, D'Silva, K, Pimentel-Quiroz, V, Vasquez Del Mercado, M, Shinjo, S, Neto, E, Junior, L, De Oliveira E Silva Montandon, A, Pons-Estel, G, Ornella, S, D'Angelo Exeni, M, Velozo, E, Jordan, P, Sirotich, E, Hausmann, J, Liew, J, Jacobsohn, L, Gore-Massy, M, Sufka, P, Grainger, R, Bhana, S, Wallace, Z, Robinson, P, Yazdany, J, Machado, P, Yeoh S. -A., Gianfrancesco M., Lawson-Tovey S., Hyrich K. L., Strangfeld A., Gossec L., Carmona L., Mateus E. F., Schafer M., Richez C., Hachulla E., Holmqvist M., Scire C. A., Lorenz H. -M., Voll R. E., Hasseli R., Jayatilleke A., Hsu T. Y. -T., D'Silva K. M., Pimentel-Quiroz V. R., Vasquez Del Mercado M., Shinjo S. K., Neto E. T. D. R., Junior L. F. D. R., De Oliveira E Silva Montandon A. C., Pons-Estel G. J., Ornella S., D'Angelo Exeni M. E., Velozo E., Jordan P., Sirotich E., Hausmann J. S., Liew J. W., Jacobsohn L., Gore-Massy M., Sufka P., Grainger R., Bhana S., Wallace Z., Robinson P. C., Yazdany J., MacHado P. M., Yeoh, S, Gianfrancesco, M, Lawson-Tovey, S, Hyrich, K, Strangfeld, A, Gossec, L, Carmona, L, Mateus, E, Schafer, M, Richez, C, Hachulla, E, Holmqvist, M, Scire, C, Lorenz, H, Voll, R, Hasseli, R, Jayatilleke, A, Hsu, T, D'Silva, K, Pimentel-Quiroz, V, Vasquez Del Mercado, M, Shinjo, S, Neto, E, Junior, L, De Oliveira E Silva Montandon, A, Pons-Estel, G, Ornella, S, D'Angelo Exeni, M, Velozo, E, Jordan, P, Sirotich, E, Hausmann, J, Liew, J, Jacobsohn, L, Gore-Massy, M, Sufka, P, Grainger, R, Bhana, S, Wallace, Z, Robinson, P, Yazdany, J, Machado, P, Yeoh S. -A., Gianfrancesco M., Lawson-Tovey S., Hyrich K. L., Strangfeld A., Gossec L., Carmona L., Mateus E. F., Schafer M., Richez C., Hachulla E., Holmqvist M., Scire C. A., Lorenz H. -M., Voll R. E., Hasseli R., Jayatilleke A., Hsu T. Y. -T., D'Silva K. M., Pimentel-Quiroz V. R., Vasquez Del Mercado M., Shinjo S. K., Neto E. T. D. R., Junior L. F. D. R., De Oliveira E Silva Montandon A. C., Pons-Estel G. J., Ornella S., D'Angelo Exeni M. E., Velozo E., Jordan P., Sirotich E., Hausmann J. S., Liew J. W., Jacobsohn L., Gore-Massy M., Sufka P., Grainger R., Bhana S., Wallace Z., Robinson P. C., Yazdany J., and MacHado P. M.

Abstract

Objectives To investigate factors associated with severe COVID-19 in people with idiopathic inflammatory myopathy (IIM). Methods Demographic data, clinical characteristics and COVID-19 outcome severity of adults with IIM were obtained from the COVID-19 Global Rheumatology Alliance physician-reported registry. A 3-point ordinal COVID-19 severity scale was defined: (1) no hospitalisation, (2) hospitalisation (and no death) and (3) death. ORs were estimated using multivariable ordinal logistic regression. Sensitivity analyses were performed using a 4-point ordinal scale: (1) no hospitalisation, (2) hospitalisation with no oxygen (and no death), (3) hospitalisation with oxygen/ventilation (and no death) and 4) death. Results Of 348 patients, 48% were not hospitalised, 39% were hospitalised (and did not die) and 13% died. Older age (OR=1.59/decade, 95% CI 1.31 to 1.91), high disease activity (OR=3.50, 95% CI 1.25 to 9.83; vs remission), ≥2 comorbidities (OR=2.63, 95% CI 1.39 to 4.98; vs none), prednisolone-equivalent dose >7.5 mg/day (OR=2.40, 95% CI 1.09 to 5.28; vs no intake) and exposure to rituximab (OR=2.71, 95% CI 1.28 to 5.72; vs conventional synthetic disease-modifying antirheumatic drugs only) were independently associated with severe COVID-19. In addition to these variables, in the sensitivity analyses, male sex (OR range: 1.65-1.83; vs female) was also significantly associated with severe outcomes, while COVID-19 diagnosis after 1 October 2020 (OR range: 0.51-0.59; vs on/before 15 June 2020) was significantly associated with less severe outcomes, but these associations were not significant in the main model (OR=1.57, 95% CI 0.95 to 2.59; and OR=0.61, 95% CI 0.37 to 1.00; respectively). Conclusions This is the first large registry data on outcomes of COVID-19 in people with IIM. Older age, male sex, higher comorbidity burden, high disease activity, prednisolone-equivalent dose >7.5 mg/day and rituximab exposure were associated with severe COVID-19. These

Published
2022

27. Adherence of Italian rheumatologists to the EULAR recommendations and outcomes in early rheumatoid arthritis patients after starting conventional DMARDs: Methotrexate in Italian patients wiTh Rheumatoid Arthritis (the MITRA study). A cohort study of the Italian Society for Rheumatology

Author

Fornaro, M, Benaglio, F, Montecucco, C, Raffeiner, B, Di Franco, M, Iannuccelli, C, Conigliaro, P, Lomater, C, Govoni, M, Silvagni, E, Zanetti, A, Parisi, S, Carrara, G, Scire, C, Caporali, R, Iannone, F, Fornaro M., Benaglio F., Montecucco C., Raffeiner B., Di Franco M., Iannuccelli C., Conigliaro P., Lomater C., Govoni M., Silvagni E., Zanetti A., Parisi S., Carrara G., Scire C. A., Caporali R., Iannone F., Fornaro, M, Benaglio, F, Montecucco, C, Raffeiner, B, Di Franco, M, Iannuccelli, C, Conigliaro, P, Lomater, C, Govoni, M, Silvagni, E, Zanetti, A, Parisi, S, Carrara, G, Scire, C, Caporali, R, Iannone, F, Fornaro M., Benaglio F., Montecucco C., Raffeiner B., Di Franco M., Iannuccelli C., Conigliaro P., Lomater C., Govoni M., Silvagni E., Zanetti A., Parisi S., Carrara G., Scire C. A., Caporali R., and Iannone F.

Abstract

Objective The aim of this study was to assess the real-life adherence of Italian rheumatologist to the 2013 EULAR recommendations and treatment outcome in rheumatoid arthritis (RA) patients who started a conventional synthetic disease-modifying anti-rheumatic drug (csDMARD). Methods The MITRA study is an Italian multicentre observational cohort focused on treatment naïve RA patients with early diagnosis recruited in an 18-month period starting from 2015. The data related to treatment with csDMARDs during the following 12 months follow-up were presented in this paper. Results Two-hundred and fifty-nine RA patients from MITRA cohort who had a follow-up visit and started a csDMARD were included in the prospective analysis. Methotrexate was started as first conventional DMARD in 224 (86.4%) patients. During the first year after starting conventional DMARDs, 175 (67.6%) RA patients reached the pre-specified target, which was DAS28 remission (<2.6) for 112 (43.2%) patients and LDA (<3.2) for 63 (24.3%) patients. Factors that negatively impacted the target achievement were fibromyalgia (HR: 0.2 [0.05-0.81]), HAQ-DI (HR: 0.72 [0.56-0.93]) and ESR (HR: 0.99 [0.99-1]). Globally, 33 (12.7%) patients started a biologic DMARD, while 61 out of 84 (72.6%) patients who had never reached the target remained on conventional DMARD. One-hundred and ninety-three adverse events (AEs) were recorded, the majority classified as mild (91 cases, 51%). Conclusion A high proportion of RA patients achieved the target during the first-year follow-up. However, a considerable portion of RA patients did not start a biological drug although the target was never reached. AEs remain frequent with conventional DMARDs, but the majority were mild.

Published
2022

28. Glucocorticoid tapering and associated outcome in patients with newly diagnosed systemic lupus erythematosus: the real-world GULP prospective observational study

Author

Floris, A, Chessa, E, Sebastiani, G, Prevete, I, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Bellisai, F, D'Alessandro, R, Zanetti, A, Carrara, G, Scire, C, Cauli, A, Piga, M, Floris A., Chessa E., Sebastiani G. D., Prevete I., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Bellisai F., D'Alessandro R., Zanetti A., Carrara G., Scire C. A., Cauli A., Piga M., Floris, A, Chessa, E, Sebastiani, G, Prevete, I, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Bellisai, F, D'Alessandro, R, Zanetti, A, Carrara, G, Scire, C, Cauli, A, Piga, M, Floris A., Chessa E., Sebastiani G. D., Prevete I., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Bellisai F., D'Alessandro R., Zanetti A., Carrara G., Scire C. A., Cauli A., and Piga M.

Abstract

Objective A subanalysis of the multicentre Early Lupus inception cohort was performed to investigate the real-world Glucocorticoids (GCs) Use in newly diagnosed systemic lupus erythematosus (SLE) Patients (GULP). Methods Patients starting prednisone (PDN) ≥5 mg/day and concomitant hydroxychloroquine or immunosuppressant within 12 months of SLE classification were enrolled. Core set variables were recorded at baseline and every 6 months, including changes in PDN dose, European Consensus Lupus Activity Measurement (ECLAM) and Systemic Lupus International Collaborating Clinics damage index. Regression models analysed predictors of tapering PDN<5 mg/day at any time and outcomes associated with different patterns of GCs tapering. Results The GULP study included 127 patients with SLE; 73 (57.5%) tapered and maintained PDN <5 mg/ day, and 17 (13.4%) discontinued PDN within a 2-year follow-up. Renal involvement (HR: 0.41; p=0.009) and lower C3 serum levels (HR: 1.04; p=0.025) predicted a lack of PDN tapering below 5 mg/day. High ECLAM scores were associated with a greater probability of increasing PDN dose (OR: 1.6; p=0.004), independently of daily intake. Disease relapse rate did not statistically differ (p=0.706) between patients tapering PDN <5 mg/day (42/99, 42.4%) and those tapering PDN without dropping below 5 mg/day (13/28, 46.4%). Every month on PDN <5 mg/day associated with lower damage accrual (IRR: 0.96; p=0.007), whereas never tapering PDN <5 mg/day associated with a higher risk of developing GC-related damage (OR 5.9; p=0.014). Conclusion Tapering PDN <5 mg/day was achieved and maintained in half of newly diagnosed patients with SLE and may represent a good balance between the need to prevent damage accrual and the risk of disease relapse.

Published
2022

29. Characteristics and Outcomes of People With Gout Hospitalized Due to COVID-19: Data From the COVID-19 Global Rheumatology Alliance Physician-Reported Registry

Author

Jatuworapruk, K, Montgomery, A, Gianfrancesco, M, Conway, R, Durcan, L, Graef, E, Jayatilleke, A, Keen, H, Kilian, A, Young, K, Carmona, L, Cogo, A, Duarte-Garcia, A, Gossec, L, Hasseli, R, Hyrich, K, Langlois, V, Lawson-Tovey, S, Malcata, A, Mateus, E, Schafer, M, Scire, C, Sigurdardottir, V, Sparks, J, Strangfeld, A, Xavier, R, Bhana, S, Gore-Massy, M, Hausmann, J, Liew, J, Sirotich, E, Sufka, P, Wallace, Z, Machado, P, Yazdany, J, Grainger, R, Robinson, P, Jatuworapruk K., Montgomery A., Gianfrancesco M., Conway R., Durcan L., Graef E. R., Jayatilleke A., Keen H., Kilian A., Young K., Carmona L., Cogo A. K., Duarte-Garcia A., Gossec L., Hasseli R., Hyrich K. L., Langlois V., Lawson-Tovey S., Malcata A., Mateus E. F., Schafer M., Scire C. A., Sigurdardottir V., Sparks J. A., Strangfeld A., Xavier R. M., Bhana S., Gore-Massy M., Hausmann J., Liew J. W., Sirotich E., Sufka P., Wallace Z., Machado P. M., Yazdany J., Grainger R., Robinson P. C., Jatuworapruk, K, Montgomery, A, Gianfrancesco, M, Conway, R, Durcan, L, Graef, E, Jayatilleke, A, Keen, H, Kilian, A, Young, K, Carmona, L, Cogo, A, Duarte-Garcia, A, Gossec, L, Hasseli, R, Hyrich, K, Langlois, V, Lawson-Tovey, S, Malcata, A, Mateus, E, Schafer, M, Scire, C, Sigurdardottir, V, Sparks, J, Strangfeld, A, Xavier, R, Bhana, S, Gore-Massy, M, Hausmann, J, Liew, J, Sirotich, E, Sufka, P, Wallace, Z, Machado, P, Yazdany, J, Grainger, R, Robinson, P, Jatuworapruk K., Montgomery A., Gianfrancesco M., Conway R., Durcan L., Graef E. R., Jayatilleke A., Keen H., Kilian A., Young K., Carmona L., Cogo A. K., Duarte-Garcia A., Gossec L., Hasseli R., Hyrich K. L., Langlois V., Lawson-Tovey S., Malcata A., Mateus E. F., Schafer M., Scire C. A., Sigurdardottir V., Sparks J. A., Strangfeld A., Xavier R. M., Bhana S., Gore-Massy M., Hausmann J., Liew J. W., Sirotich E., Sufka P., Wallace Z., Machado P. M., Yazdany J., Grainger R., and Robinson P. C.

Abstract

Objective To describe people with gout who were diagnosed with coronavirus disease 2019 (COVID-19) and hospitalized and to characterize their outcomes. Methods Data on patients with gout hospitalized for COVID-19 between March 12, 2020, and October 25, 2021, were extracted from the COVID-19 Global Rheumatology Alliance registry. Descriptive statistics were used to describe the demographics, comorbidities, medication exposures, and COVID-19 outcomes including oxygenation or ventilation support and death. Results One hundred sixty-three patients with gout who developed COVID-19 and were hospitalized were included. The mean age was 63 years, and 85% were male. The majority of the group lived in the Western Pacific Region (35%) and North America (18%). Nearly half (46%) had two or more comorbidities, with hypertension (56%), cardiovascular disease (28%), diabetes mellitus (26%), chronic kidney disease (25%), and obesity (23%) being the most common. Glucocorticoids and colchicine were used pre-COVID-19 in 11% and 12% of the cohort, respectively. Over two thirds (68%) of the cohort required supplemental oxygen or ventilatory support during hospitalization. COVID-19-related death was reported in 16% of the overall cohort, with 73% of deaths documented in people with two or more comorbidities. Conclusion This cohort of people with gout and COVID-19 who were hospitalized had high frequencies of ventilatory support and death. This suggests that patients with gout who were hospitalized for COVID-19 may be at risk of poor outcomes, perhaps related to known risk factors for poor outcomes, such as age and presence of comorbidity.

Published
2022

30. Impact of disease duration and gender on the sensitivity and specificity of 2015 ACR/EULAR classification criteria for gout. Cross-sectional results from an Italian multicentric study on the management of crystal-induced arthritis (ATTACk)

Author

Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Ceccarelli, F, Lucchetti, R, Bortoluzzi, A, Cimmino, M, Di Matteo, A, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Furini, F, Zanetti, A, Carrara, G, Scire, C, Doria, A, Ramonda, R, Lorenzin M., Ughi N., Ariani A., Raffeiner B., Ceccarelli F., Lucchetti R., Bortoluzzi A., Cimmino M. A., Di Matteo A., Frallonardo P., Hoxha A., Ortolan A., Favero M., Parisi S., Furini F., Zanetti A., Carrara G., Scire C. A., Doria A., Ramonda R., Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Ceccarelli, F, Lucchetti, R, Bortoluzzi, A, Cimmino, M, Di Matteo, A, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Furini, F, Zanetti, A, Carrara, G, Scire, C, Doria, A, Ramonda, R, Lorenzin M., Ughi N., Ariani A., Raffeiner B., Ceccarelli F., Lucchetti R., Bortoluzzi A., Cimmino M. A., Di Matteo A., Frallonardo P., Hoxha A., Ortolan A., Favero M., Parisi S., Furini F., Zanetti A., Carrara G., Scire C. A., Doria A., and Ramonda R.

Abstract

Objective We aimed to assess the performance of the 2015 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) gout classification criteria in an Italian cohort of patients with crystal-induced arthritis stratified by disease duration and gender in a real-life setting. Methods Consecutive patients referred to Rheumatology Units for suspected acute crystal-induced arthritis were enrolled in a multicentre cohort study by the Italian Society of Rheumatology which was designed to improve the management of crystal-induced arthritis (ATTACk). To test the performance of the criteria (sensitivity and specificity), the presence of monosodium urate (MSU) crystals in synovial fluid (SF) was used as gold standard. Subgroup analyses by gender and disease duration were performed. Results Two hundred and seventy-seven patients were enrolled. SF analysis was available in 137 (49%) patients. Complete SF analysis and ACR/EULAR scores were obtained in 44% of patients. MSU crystals were found in 66% of patients. The sensitivity and the specificity of all criteria sets were 78% (95%CI, 67–86) and 98% (95%CI, 87–100), respectively; only clinical criteria yielded 70% (95%CI, 59–80) sensitivity and 93% (95%CI, 80-98) specificity, respectively. In early-stage disease (<2 years), the sensitivity dropped to 58% (95%CI, 39-75), while the specificity was 100% (95%CI, 85–100). Conclusion The ACR/EULAR criteria showed good performance in patients presenting with acute arthritis; changes were observed when a subset of criteria were used, especially in early-stage disease.

Published
2022

31. Novel insights into the management of rheumatoid arthritis: one year in review 2022

Author

Garaffoni, C, Adinolfi, A, Bortoluzzi, A, Filippou, G, Giollo, A, Sakellariou, G, Sirotti, S, Ughi, N, Scire, C, Silvagni, E, Garaffoni C., Adinolfi A., Bortoluzzi A., Filippou G., Giollo A., Sakellariou G., Sirotti S., Ughi N., Scire C. A., Silvagni E., Garaffoni, C, Adinolfi, A, Bortoluzzi, A, Filippou, G, Giollo, A, Sakellariou, G, Sirotti, S, Ughi, N, Scire, C, Silvagni, E, Garaffoni C., Adinolfi A., Bortoluzzi A., Filippou G., Giollo A., Sakellariou G., Sirotti S., Ughi N., Scire C. A., and Silvagni E.

Abstract

New evidence for the treatment of rheumatoid arthritis (RA) has emerged during the last year. Specifically, updated guidelines on pharmacological and non-pharmacological management of RA have emphasised the necessity of global patient’s care, and have shifted the role of some older drugs, such as glucocorticoids and methotrexate. In addition, the long-term safety of Janus kinase inhibitors was investigated and reinforced. With respect to the coronavirus-19 pandemic, reassuring data on the efficacy and safety of vaccinations in the RA population were acquired, as well as on the potential role of telemedicine in RA management. Machine learning prediction models and biomarkers development have emerged as promising innovations in the area of precision/personalised medicine, appearing to encourage future expansion. In this narrative review, the authors aim to give their specific point of view on the most relevant and potentially impacting novelties published during 2021 and early 2022 in the context of RA management.

Published
2022

33. EULAR points to consider for the use of imaging to guide interventional procedures in patients with rheumatic and musculoskeletal diseases (RMDs)

Author

Dejaco, C, Machado, P, Carubbi, F, Bosch, P, Terslev, L, Tamborrini, G, Sconfienza, L, Scire, C, Ruetten, S, van Rompay, J, Proft, F, Pitzalis, C, Obradov, M, Moe, R, Mascarenhas, V, Malattia, C, Klauser, A, Kent, A, Jans, L, Hartung, W, Hammer, H, Duftner, C, Balint, P, Alunno, A, Baraliakos, X, Dejaco C., Machado P. M., Carubbi F., Bosch P., Terslev L., Tamborrini G., Sconfienza L. M., Scire C. A., Ruetten S., van Rompay J., Proft F., Pitzalis C., Obradov M., Moe R. H., Mascarenhas V. V., Malattia C., Klauser A. S., Kent A., Jans L., Hartung W., Hammer H. B., Duftner C., Balint P. V., Alunno A., Baraliakos X., Dejaco, C, Machado, P, Carubbi, F, Bosch, P, Terslev, L, Tamborrini, G, Sconfienza, L, Scire, C, Ruetten, S, van Rompay, J, Proft, F, Pitzalis, C, Obradov, M, Moe, R, Mascarenhas, V, Malattia, C, Klauser, A, Kent, A, Jans, L, Hartung, W, Hammer, H, Duftner, C, Balint, P, Alunno, A, Baraliakos, X, Dejaco C., Machado P. M., Carubbi F., Bosch P., Terslev L., Tamborrini G., Sconfienza L. M., Scire C. A., Ruetten S., van Rompay J., Proft F., Pitzalis C., Obradov M., Moe R. H., Mascarenhas V. V., Malattia C., Klauser A. S., Kent A., Jans L., Hartung W., Hammer H. B., Duftner C., Balint P. V., Alunno A., and Baraliakos X.

Abstract

Objectives To develop evidence-based Points to Consider (PtC) for the use of imaging modalities to guide interventional procedures in patients with rheumatic and musculoskeletal diseases (RMDs). Methods European Alliance of Associations for Rheumatology (EULAR) standardised operating procedures were followed. A systematic literature review was conducted to retrieve data on the role of imaging modalities including ultrasound (US), fluoroscopy, MRI, CT and fusion imaging to guide interventional procedures. Based on evidence and expert opinion, the task force (25 participants consisting of physicians, healthcare professionals and patients from 11 countries) developed PtC, with consensus obtained through voting. The final level of agreement was provided anonymously. Results A total of three overarching principles and six specific PtC were formulated. The task force recommends preference of imaging over palpation to guide targeted interventional procedures at peripheral joints, periarticular musculoskeletal structures, nerves and the spine. While US is the favoured imaging technique for peripheral joints and nerves, the choice of the imaging method for the spine and sacroiliac joints has to be individualised according to the target, procedure, expertise, availability and radiation exposure. All imaging guided interventions should be performed by a trained specialist using appropriate operational procedures, settings and assistance by technical personnel. Conclusion These are the first EULAR PtC to provide guidance on the role of imaging to guide interventional procedures in patients with RMDs.

Published
2022

34. The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases: insights after the first 5 years of the ERN ReCONNET

Author

Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Andersen, J, Arnaud, L, Avcin, T, Marsal Barril, S, Beretta, L, Bombardieri, S, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Corti, P, Costedoat-Chalumeau, N, Davidsone, Z, Doria, A, Fenech, C, Ferraris, A, Fischer-Betz, R, Fonseca, J, Frank, C, Gaglioti, A, Galetti, I, Guimaraes, V, Hachulla, E, Holmner, M, Houssiau, F, Iaccarino, L, Jacobsen, S, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Milas-Ahic, J, Moinzadeh, P, Montecucco, C, Mouthon, L, Muller-Ladner, U, Nagy, G, Patarata, E, Pileckyte, M, Pruunsild, C, Rednic, S, Romao, V, Schneider, M, Scire, C, Smith, V, Sulli, A, Tamirou, F, Tani, C, Taruscio, D, Taulaigo, A, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P, van Laar, J, Vieira, A, de Vries-Bouwstra, J, Zschocke, J, Cutolo, M, Mosca, M, Talarico R., Aguilera S., Alexander T., Amoura Z., Andersen J., Arnaud L., Avcin T., Marsal Barril S., Beretta L., Bombardieri S., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Cannizzo S., Cavagna L., Chaigne B., Cornet A., Corti P., Costedoat-Chalumeau N., Davidsone Z., Doria A., Fenech C., Ferraris A., Fischer-Betz R., Fonseca J. E., Frank C., Gaglioti A., Galetti I., Guimaraes V., Hachulla E., Holmner M., Houssiau F., Iaccarino L., Jacobsen S., Limper M., Malfait F., Mariette X., Marinello D., Martin T., Matthews L., Matucci-Cerinic M., Meyer A., Milas-Ahic J., Moinzadeh P., Montecucco C., Mouthon L., Muller-Ladner U., Nagy G., Patarata E., Pileckyte M., Pruunsild C., Rednic S., Romao V. C., Schneider M., Scire C. A., Smith V., Sulli A., Tamirou F., Tani C., Taruscio D., Taulaigo A. V., Tincani A., Ticciati S., Turchetti G., van Hagen P. M., van Laar J. M., Vieira A., de Vries-Bouwstra J. K., Zschocke J., Cutolo M., Mosca M., Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Andersen, J, Arnaud, L, Avcin, T, Marsal Barril, S, Beretta, L, Bombardieri, S, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Corti, P, Costedoat-Chalumeau, N, Davidsone, Z, Doria, A, Fenech, C, Ferraris, A, Fischer-Betz, R, Fonseca, J, Frank, C, Gaglioti, A, Galetti, I, Guimaraes, V, Hachulla, E, Holmner, M, Houssiau, F, Iaccarino, L, Jacobsen, S, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Milas-Ahic, J, Moinzadeh, P, Montecucco, C, Mouthon, L, Muller-Ladner, U, Nagy, G, Patarata, E, Pileckyte, M, Pruunsild, C, Rednic, S, Romao, V, Schneider, M, Scire, C, Smith, V, Sulli, A, Tamirou, F, Tani, C, Taruscio, D, Taulaigo, A, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P, van Laar, J, Vieira, A, de Vries-Bouwstra, J, Zschocke, J, Cutolo, M, Mosca, M, Talarico R., Aguilera S., Alexander T., Amoura Z., Andersen J., Arnaud L., Avcin T., Marsal Barril S., Beretta L., Bombardieri S., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Cannizzo S., Cavagna L., Chaigne B., Cornet A., Corti P., Costedoat-Chalumeau N., Davidsone Z., Doria A., Fenech C., Ferraris A., Fischer-Betz R., Fonseca J. E., Frank C., Gaglioti A., Galetti I., Guimaraes V., Hachulla E., Holmner M., Houssiau F., Iaccarino L., Jacobsen S., Limper M., Malfait F., Mariette X., Marinello D., Martin T., Matthews L., Matucci-Cerinic M., Meyer A., Milas-Ahic J., Moinzadeh P., Montecucco C., Mouthon L., Muller-Ladner U., Nagy G., Patarata E., Pileckyte M., Pruunsild C., Rednic S., Romao V. C., Schneider M., Scire C. A., Smith V., Sulli A., Tamirou F., Tani C., Taruscio D., Taulaigo A. V., Tincani A., Ticciati S., Turchetti G., van Hagen P. M., van Laar J. M., Vieira A., de Vries-Bouwstra J. K., Zschocke J., Cutolo M., and Mosca M.

Abstract

In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.

Published
2022

35. Reliability assessment of the definition of ultrasound enthesitis in SpA: results of a large, multicentre, international, web-based study

Author

Di Matteo, A, Cipolletta, E, Castaniti, G, Smerilli, G, Airoldi, C, Aydin, S, Becciolini, A, Bonfiglioli, K, Bruns, A, Carrara, G, Cazenave, T, Ciapetti, A, Cosatti, M, de Agustin, J, Di Carlo, M, Di Donato, E, Di Geso, L, Duran, E, Elliott, A, Estrach, C, Farisogullari, B, Fiorenza, A, Fodor, D, Gabba, A, Hernandez-Diaz, C, Huang, F, Hurnakova, J, Li, L, Jesus, D, Karadag, O, Martire, M, Massarotti, M, Michelena, X, Musca, A, Nair, J, Okano, T, Papalopoulos, I, Rosemffet, M, Rovisco, J, Rozza, D, Salaffi, F, Satulu, I, Scioscia, C, Scire, C, Sun, F, Tamas, M, Tanimura, S, Ventura-Rios, L, Voulgari, P, Vreju, F, Vukatana, G, Wong, E, Yang, J, Hereter, J, Zanetti, A, Grassi, W, Filippucci, E, Di Matteo A., Cipolletta E., Castaniti G. M. D., Smerilli G., Airoldi C., Aydin S. Z., Becciolini A., Bonfiglioli K., Bruns A., Carrara G., Cazenave T., Ciapetti A., Cosatti M. A., de Agustin J. J., Di Carlo M., Di Donato E., Di Geso L., Duran E., Elliott A., Estrach C., Farisogullari B., Fiorenza A., Fodor D., Gabba A., Hernandez-Diaz C., Huang F., Hurnakova J., Li L., Jesus D., Karadag O., Martire M. V., Massarotti M., Michelena X., Musca A. A., Nair J., Okano T., Papalopoulos I., Rosemffet M., Rovisco J., Rozza D., Salaffi F., Satulu I., Scioscia C., Scire C. A., Sun F., Tamas M. -M., Tanimura S., Ventura-Rios L., Voulgari P. V., Vreju F. A., Vukatana G., Wong E., Yang J., Hereter J. Z., Zanetti A., Grassi W., Filippucci E., Di Matteo, A, Cipolletta, E, Castaniti, G, Smerilli, G, Airoldi, C, Aydin, S, Becciolini, A, Bonfiglioli, K, Bruns, A, Carrara, G, Cazenave, T, Ciapetti, A, Cosatti, M, de Agustin, J, Di Carlo, M, Di Donato, E, Di Geso, L, Duran, E, Elliott, A, Estrach, C, Farisogullari, B, Fiorenza, A, Fodor, D, Gabba, A, Hernandez-Diaz, C, Huang, F, Hurnakova, J, Li, L, Jesus, D, Karadag, O, Martire, M, Massarotti, M, Michelena, X, Musca, A, Nair, J, Okano, T, Papalopoulos, I, Rosemffet, M, Rovisco, J, Rozza, D, Salaffi, F, Satulu, I, Scioscia, C, Scire, C, Sun, F, Tamas, M, Tanimura, S, Ventura-Rios, L, Voulgari, P, Vreju, F, Vukatana, G, Wong, E, Yang, J, Hereter, J, Zanetti, A, Grassi, W, Filippucci, E, Di Matteo A., Cipolletta E., Castaniti G. M. D., Smerilli G., Airoldi C., Aydin S. Z., Becciolini A., Bonfiglioli K., Bruns A., Carrara G., Cazenave T., Ciapetti A., Cosatti M. A., de Agustin J. J., Di Carlo M., Di Donato E., Di Geso L., Duran E., Elliott A., Estrach C., Farisogullari B., Fiorenza A., Fodor D., Gabba A., Hernandez-Diaz C., Huang F., Hurnakova J., Li L., Jesus D., Karadag O., Martire M. V., Massarotti M., Michelena X., Musca A. A., Nair J., Okano T., Papalopoulos I., Rosemffet M., Rovisco J., Rozza D., Salaffi F., Satulu I., Scioscia C., Scire C. A., Sun F., Tamas M. -M., Tanimura S., Ventura-Rios L., Voulgari P. V., Vreju F. A., Vukatana G., Wong E., Yang J., Hereter J. Z., Zanetti A., Grassi W., and Filippucci E.

Abstract

Objectives. To investigate the reliability of the OMERACT US Task Force definition of US enthesitis in SpA. Methods. In this web exercise, based on the evaluation of 101 images and 39 clips of the main entheses of the lower limbs, the elementary components included in the OMERACT definition of US enthesitis in SpA (hypoechoic areas, entheseal thickening, power Doppler signal at the enthesis, enthesophytes/calcifications, bone erosions) were assessed by 47 rheumatologists from 37 rheumatology centres in 15 countries. Inter- and intra-observer reliability of the US components of enthesitis was calculated using Light’s kappa, Cohen’s kappa, Prevalence And Bias Adjusted Kappa (PABAK) and their 95% CIs. Results. Bone erosions and power Doppler signal at the enthesis showed the highest overall inter-reliability [Light’s kappa: 0.77 (0.76–0.78), 0.72 (0.71–0.73), respectively; PABAK: 0.86 (0.86–0.87), 0.73 (0.73–0.74), respectively], followed by enthesophytes/calcifications [Light’s kappa: 0.65 (0.64–0.65), PABAK: 0.67 (0.67–0.68)]. This was moderate for entheseal thickening [Light’s kappa: 0.41 (0.41–0.42), PABAK: 0.41 (0.40–0.42)], and fair for hypoechoic areas [Light’s kappa: 0.37 (0.36–0.38); PABAK: 0.37 (0.37–0.38)]. A similar trend was observed in the intra-reliability exercise, although this was characterized by an overall higher degree of reliability for all US elementary components compared with the inter-observer evaluation. Conclusions. The results of this multicentre, international, web-based study show a good reliability of the OMERACT US definition of bone erosions, power Doppler signal at the enthesis and enthesophytes/calcifications. The low reliability of entheseal thickening and hypoechoic areas raises questions about the opportunity to revise the definition of these two major components for the US diagnosis of enthesitis.

Published
2022

36. Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Author

Ferri, C, De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri C., De Angelis R., Giuggioli D., Bajocchi G., Dagna L., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Guiducci S., Girelli F., Riccieri V., Zanatta E., Bosello S., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Masini F., Gigante A., Bellando-Randone S., Pellegrino G., Pigatto E., Lazzaroni M. G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Doria A., Iannone F., Salvarani C., Matucci-Cerinic M., Ferri, C, De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri C., De Angelis R., Giuggioli D., Bajocchi G., Dagna L., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Guiducci S., Girelli F., Riccieri V., Zanatta E., Bosello S., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Masini F., Gigante A., Bellando-Randone S., Pellegrino G., Pigatto E., Lazzaroni M. G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Doria A., Iannone F., Salvarani C., and Matucci-Cerinic M.

Abstract

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature. Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 ± 26.9 yrs.; mean disease duration 8.9 ± 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas. Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches. Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities.

Published
2022

37. Impact of rheumatoid arthritis and methotrexate on pregnancy outcomes: retrospective cohort study of the Italian Society for Rheumatology

Author

Zanetti, A, Zambon, A, Scire, C, Bortoluzzi, A, Zanetti A., Zambon A., Scire C. A., Bortoluzzi A., Zanetti, A, Zambon, A, Scire, C, Bortoluzzi, A, Zanetti A., Zambon A., Scire C. A., and Bortoluzzi A.

Abstract

OBJECTIVE: To evaluate the impact of rheumatoid arthritis (RA) and methotrexate (MTX) on the probability of becoming pregnant, pregnancy losses, elective termination of pregnancy (TOP) and congenital malformations. METHODS: A retrospective cohort study on administrative healthcare databases was conducted. Three patients' cohorts were enrolled among childbearing-age women. The first cohort included patients with RA starting MTX between July 2004 and December 2011. The second cohort included patients with RA without MTX treatment randomly selected from the same population (ratio 1:1). Finally, a cohort of subjects without RA was identified (ratio 1:4). Multivariate logistic regression models were implemented, ORs and 95% CI were reported. RESULTS: The two matched RA cohorts included 3564 patients with MTX and without MTX. The cohort without RA included 14 256 subjects. In the three cohorts, the proportion of women achieving pregnancy during follow-up was 6.3%, 9.1% and 11.9%, respectively. Congenital malformations were very rare in all cohorts. RA women treated with MTX at any time before conception showed significantly higher risks of pregnancy losses than non-RA women (OR (95% CI) 2.22 (1.40 to 3.45)). We observed a significant positive relationship between the exposure to MTX in the 3 months window before conception and increased risk of elective TOP (OR (95% CI) 4.77 (1.08 to 19.40)). CONCLUSION: MTX-treated patients appeared to be the cohort with the highest risk of pregnancy losses. The positive association with elective TOP and exposure to MTX in the three months window before conception in patients with RA reinforces the need for adequate preconception counselling to avoid unplanned pregnancies.

Published
2022

38. Increased COVID-19 mortality in patients with rheumatic diseases: results from the CONTROL-19 study by the Italian Society for Rheumatology

Author

Zanetti, A, Carrara, G, Landolfi, G, Rozza, D, Chighizola, C, Alunno, A, Andreoli, L, Caporali, R, Gerli, R, Sebastiani, G, Valesini, G, Sinigaglia, L, Raffeiner, B, Lomater, C, Caprioli, M, Fredi, M, Romeo, N, Cuomo, G, Vadacca, M, Scire, C, Zanetti A., Carrara G., Landolfi G., Rozza D., Chighizola C. B., Alunno A., Andreoli L., Caporali R., Gerli R., Sebastiani G. D., Valesini G., Sinigaglia L., Raffeiner B., Lomater C., Caprioli M., Fredi M., Romeo N., Cuomo G., Vadacca M., Scire C. A., Zanetti, A, Carrara, G, Landolfi, G, Rozza, D, Chighizola, C, Alunno, A, Andreoli, L, Caporali, R, Gerli, R, Sebastiani, G, Valesini, G, Sinigaglia, L, Raffeiner, B, Lomater, C, Caprioli, M, Fredi, M, Romeo, N, Cuomo, G, Vadacca, M, Scire, C, Zanetti A., Carrara G., Landolfi G., Rozza D., Chighizola C. B., Alunno A., Andreoli L., Caporali R., Gerli R., Sebastiani G. D., Valesini G., Sinigaglia L., Raffeiner B., Lomater C., Caprioli M., Fredi M., Romeo N., Cuomo G., Vadacca M., and Scire C. A.

Abstract

Objective To investigate differences in coronavirus disease 2019 (COVID-19) mortality between patients with rheumatic musculoskeletal diseases (RMD) and the general population in Italy. Methods We analysed the data from the national surveillance study promoted by the Italian Society for Rheumatology (CONTROL-19 database) including patients with RMD and COVID-19 between 26 March 2020 and 29 November 2020, compared with official data from the Italian population (within the same period) adjusted for age, sex and geographic location. The main outcome of the analyses was mortality. The relationship between RMD and mortality was analysed using adjusted logistic models and sensitivity analyses were conducted to support the robustness of our results. Results We included 668 RMD patients (62.7% with inflammatory arthritis, 28.6% with systemic autoimmune diseases), who had a mean age of 58.4 years and of which 66% were female. Compared to the general population, the RMD population showed an increased risk of death (OR 3.10 (95% CI 2.29–4.12)), independently from the differences in age and sex distribution. Even after considering the potential influence of surveillance bias, the OR was 2.08 (95% CI: 1.55–2.73). Such excess of risk was more evident in the subgroup of younger patients, and more consistent in women. Subjects with systemic autoimmune diseases showed a higher risk of death than patients with any other RMDs. Conclusions Patients with RMD and COVID-19 infection evidenced a significant increase in mortality during the first pandemic phases in Italy. These findings support the need for strong SARS-CoV-2 prevention in patients with rheumatic diseases.

Published
2022

39. Influence of initial glucocorticoid co-medication on mortality and hospitalization in early inflammatory arthritis: an investigation by record linkage of clinical and administrative databases

Author

Sakellariou, G, Scire, C, Rumi, F, Carrara, G, Zanetti, A, Cerra, C, Migliazza, S, Bugatti, S, Montecucco, C, Sakellariou G., Scire C. A., Rumi F., Carrara G., Zanetti A., Cerra C., Migliazza S., Bugatti S., Montecucco C., Sakellariou, G, Scire, C, Rumi, F, Carrara, G, Zanetti, A, Cerra, C, Migliazza, S, Bugatti, S, Montecucco, C, Sakellariou G., Scire C. A., Rumi F., Carrara G., Zanetti A., Cerra C., Migliazza S., Bugatti S., and Montecucco C.

Abstract

Background: While low-dose oral glucocorticoids (GCs) are recommended in the management of early arthritis, their impact on mortality is unclear. The aim of this study is to evaluate the effect of GCs on mortality in patients with early arthritis, by linking clinical and administrative databases. Methods: The study included patients with new-onset rheumatoid arthritis (RA) or undifferentiated arthritis (2005–2010), who received DMARDs (MTX in RA or UA with poor prognosis, hydroxychloroquine in UA) and were alive at the second year of follow-up. Low-dose GCs could be prescribed. Clinical and administrative data were linked from Administrative Health Databases (AHD) of the corresponding province, which provided us with information on drug delivery, comorbidities, hospitalization, and mortality. The effect of GCs in the first year was defined using a dichotomous variable or a 3-level categorization (not delivered, ≤7.5 mg/day, or >7.5 mg/day of prednisone) on all-cause mortality, assessed with Cox regression, either crude or adjusted for age, gender, Charlson Comorbidity Index (CCI) or single comorbidities, ACPA, HAQ, and MTX in the first year. A secondary analysis of the effect of GCs on related hospitalizations (for cardiovascular events, diabetes, serious infections, osteoporotic fractures) was also carried. Results: Four hundred forty-nine patients were enrolled (mean age 58.59, RA 65.03%) of which 51 (11.36%) died during the study. The median (IQR) follow-up was equal to 103.91 (88.03–126.71) months. Treatments with GCs were formally prescribed to 198 patients (44.10%) at ≤7.5 mg/day, although by the end of the study such treatments were received by 257 patients (57.24%); 88 patients (19.6%) were treated with GCs at >7.5 mg/day. In adjusted analyses, the GC delivery (HR, 95% CI 1.35 (0.74, 2.47)) did not significantly predict mortality — both at a low (HR, 95% CI 1.41 (0.73, 2.71)) and at a high (HR, 95% CI 1.23 (0.52, 2.92)) dosage. When “all-cause hospital

Published
2022

40. Musculoskeletal ultrasound for treating rheumatoid arthritis to target-a systematic literature review

Author

Silvagni, E, Zandonella Callegher, S, Mauric, E, Chiricolo, S, Schreiber, N, Tullio, A, Zabotti, A, Scire, C, Dejaco, C, Sakellariou, G, Silvagni E., Zandonella Callegher S., Mauric E., Chiricolo S., Schreiber N., Tullio A., Zabotti A., Scire C. A., Dejaco C., Sakellariou G., Silvagni, E, Zandonella Callegher, S, Mauric, E, Chiricolo, S, Schreiber, N, Tullio, A, Zabotti, A, Scire, C, Dejaco, C, Sakellariou, G, Silvagni E., Zandonella Callegher S., Mauric E., Chiricolo S., Schreiber N., Tullio A., Zabotti A., Scire C. A., Dejaco C., and Sakellariou G.

Abstract

OBJECTIVE: We aimed to systematically review the literature to retrieve evidence on the diagnostic and prognostic value of musculoskeletal ultrasound for a treat to target (T2T) approach in RA. METHODS: Eight research questions were developed addressing the role of ultrasound (including different ultrasound scores and elementary lesions) for diagnosis, monitoring and prognosis of RA. PubMed and EMBASE were searched (2005-2020). Articles on RA and reporting data on musculoskeletal ultrasound were included and extracted according to the underlying questions, and risk of bias assessed according to the study design. RESULTS: Out of 4632 records, 60 articles were included. Due to clinical heterogeneity, meta-analysis was not possible. Ultrasound better predicted disease relapses with respect to clinical examination in patients in remission, while both methods performed similarly in predicting response to therapy, achievement of remission and radiographic progression. Ultrasound was superior to clinical examination in diagnosing joint involvement using another imaging modality, such as magnetic resonance imaging, as reference. Limited ultrasound scores performed like more extensive evaluations for the detection of joint inflammation and for outcome prediction. Higher ultrasound scores of synovitis were linked to poor outcomes at all disease stages, but a specific cut-off distinguishing between low- and high-risk groups did not emerge. CONCLUSIONS: These data confirm the pivotal role of ultrasound when evaluating synovial inflammation and when identifying RA patients at higher risk of relapse. Further research is needed to better define the role of ultrasound in a T2T management strategy in moderately-to-highly active RA.

Published
2022

41. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology

Author

De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Dall'Ara, F, Lazzaroni, M, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Risa, A, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri, C, De Angelis R., Giuggioli D., Bajocchi G., Dagna L., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Guiducci S., Girelli F., Riccieri V., Zanatta E., Bosello S., Cavazzana I., Ingegnoli F., Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Masini F., Gigante A., Bellando-Randone S., Pellegrino G., Pigatto E., Dall'Ara F., Lazzaroni M. G., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., Furini F., Vultaggio L., Parisi S., Peroni C. L., Risa A. M., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Doria A., Iannone F., Salvarani C., Matucci-Cerinic M., Ferri C., De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Dall'Ara, F, Lazzaroni, M, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Risa, A, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri, C, De Angelis R., Giuggioli D., Bajocchi G., Dagna L., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Guiducci S., Girelli F., Riccieri V., Zanatta E., Bosello S., Cavazzana I., Ingegnoli F., Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Masini F., Gigante A., Bellando-Randone S., Pellegrino G., Pigatto E., Dall'Ara F., Lazzaroni M. G., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., Furini F., Vultaggio L., Parisi S., Peroni C. L., Risa A. M., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Doria A., Iannone F., Salvarani C., Matucci-Cerinic M., and Ferri C.

Abstract

OBJECTIVE: There is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics. METHODS: A multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared. RESULTS: The overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs. CONCLUSION: Our study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex.

Published
2022

42. Defining anti-synthetase syndrome: a systematic literature review

Author

Zanframundo, G, Faghihi-Kashani, S, Scire, C, Bonella, F, Corte, T, Doyle, T, Fiorentino, D, Gonzalez-Gay, M, Hudson, M, Kuwana, M, Lundberg, I, Mammen, A, Mchugh, N, Miller, F, Monteccucco, C, Oddis, C, Rojas-Serrano, J, Schmidt, J, Selva-O'Callaghan, A, Werth, V, Sakellariou, G, Aggarwal, R, Cavagna, L, Zanframundo G., Faghihi-Kashani S., Scire C. A., Bonella F., Corte T. J., Doyle T. J., Fiorentino D., Gonzalez-Gay M. A., Hudson M., Kuwana M., Lundberg I. E., Mammen A., McHugh N., Miller F. W., Monteccucco C., Oddis C. V., Rojas-Serrano J., Schmidt J., Selva-O'Callaghan A., Werth V. P., Sakellariou G., Aggarwal R., Cavagna L., Zanframundo, G, Faghihi-Kashani, S, Scire, C, Bonella, F, Corte, T, Doyle, T, Fiorentino, D, Gonzalez-Gay, M, Hudson, M, Kuwana, M, Lundberg, I, Mammen, A, Mchugh, N, Miller, F, Monteccucco, C, Oddis, C, Rojas-Serrano, J, Schmidt, J, Selva-O'Callaghan, A, Werth, V, Sakellariou, G, Aggarwal, R, Cavagna, L, Zanframundo G., Faghihi-Kashani S., Scire C. A., Bonella F., Corte T. J., Doyle T. J., Fiorentino D., Gonzalez-Gay M. A., Hudson M., Kuwana M., Lundberg I. E., Mammen A., McHugh N., Miller F. W., Monteccucco C., Oddis C. V., Rojas-Serrano J., Schmidt J., Selva-O'Callaghan A., Werth V. P., Sakellariou G., Aggarwal R., and Cavagna L.

Abstract

Anti-synthetase syndrome (ASSD) is a heterogeneous autoimmune disease characterised by multi-system involvement with a wide variety of manifestations. Validated classification criteria are necessary to improve recognition and prevent misclassification, especially given the lack of reliable and standardised autoantibody testing. We systematically reviewed the literature to analyse proposed ASSD criteria, characteristics, and diagnostic performance. Methods We searched PubMed and Embase databases (01/01/1984 to 06/11/2018) and the ACR and EULAR meeting abstracts (2017-2018). Sensitivities, specificities, positive, negative likelihood ratios and risk of bias were calculated for ASSD criteria and key variables reported in the literature. We performed meta-analysis when appropriate. Results We retrieved 4,358 studies. We found 85 proposed ASSD criteria from a total of 82 studies. All but one study included anti-synthetase autoantibody (ARS) positivity in the ASSD criteria. Most studies required only one ASSD feature plus anti-ARS to define ASSD (n=64, 78%), whereas 16 studies required more than one ASSD variable plus anti-ARS. The only criteria not including anti-ARS positivity required 5 ASSD clinical features. We found limited data and wide variability in the diagnostic performance of each variable and definition proposed in the literature. Given these limitations we only meta-analysed the performance of individual muscle biopsy and clinical variables in diagnosing ASSD, which performed poorly. Conclusion The current ASSD criteria include a variety of serological, clinical, and histological features with wide variability amongst proposed definitions and the performance of these definitions has not been tested. This systematic literature review suggests the need for additional data and consensus-driven classification criteria for ASSD.

Published
2022

43. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.

Abstract

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

44. Efficacy and Drug Survival after Switching from Etanercept to the Biosimilar SB4: A Real-Life Long-Term Study

Author

Parisi, S, Becciolini, A, Ditto, M, Rozza, D, Zanetti, A, Lagana, A, Peroni, C, Centanaro Di Vittorio, C, Degiovanni, R, Realmuto, C, Scire, C, Priora, M, Di Donato, E, Santilli, D, Mozzani, F, Lucchini, G, Ariani, A, Gardelli, L, Girelli, F, Arrigoni, E, Plate, I, Bravi, E, Paroli, M, Caccavale, R, Salvarani, C, Sandri, G, Lumetti, F, Volpe, A, Marchetta, A, Fusaro, E, Parisi S., Becciolini A., Ditto M. C., Rozza D., Zanetti A., Lagana A., Peroni C. L., Centanaro Di Vittorio C., Degiovanni R., Realmuto C., Scire C. A., Priora M., Di Donato E., Santilli D., Mozzani F., Lucchini G., Ariani A., Gardelli L., Girelli F., Arrigoni E., Plate I., Bravi E., Paroli M., Caccavale R., Salvarani C., Sandri G., Lumetti F., Volpe A., Marchetta A., Fusaro E., Parisi, S, Becciolini, A, Ditto, M, Rozza, D, Zanetti, A, Lagana, A, Peroni, C, Centanaro Di Vittorio, C, Degiovanni, R, Realmuto, C, Scire, C, Priora, M, Di Donato, E, Santilli, D, Mozzani, F, Lucchini, G, Ariani, A, Gardelli, L, Girelli, F, Arrigoni, E, Plate, I, Bravi, E, Paroli, M, Caccavale, R, Salvarani, C, Sandri, G, Lumetti, F, Volpe, A, Marchetta, A, Fusaro, E, Parisi S., Becciolini A., Ditto M. C., Rozza D., Zanetti A., Lagana A., Peroni C. L., Centanaro Di Vittorio C., Degiovanni R., Realmuto C., Scire C. A., Priora M., Di Donato E., Santilli D., Mozzani F., Lucchini G., Ariani A., Gardelli L., Girelli F., Arrigoni E., Plate I., Bravi E., Paroli M., Caccavale R., Salvarani C., Sandri G., Lumetti F., Volpe A., Marchetta A., and Fusaro E.

Abstract

We evaluated the 3-year drug survival and efficacy of the biosimilar SB4/Benepali in rheumatoid arthritis (RA), psoriatic arthritis (PsA) and ankylosing spondylitis (AS) patients, pre-viously treated with etanercept (ETA). Drug survival rate was calculated using the Kaplan–Meier method and Cox proportional hazard models were developed to examine predictors of SB4 discontin-uation. 236 patients (120 RA, 80 PsA and 36 AS), aged 60.7 ± 13.8 years and with an ETA duration of 4.1 ± 3.4 years were included. The 3-year retention rate for SB4 was 94.4%, 88% and 86% in AS, RA and PsA patients, respectively, with no difference between groups. Patients without comorbid disease had higher retention rates vs. patients with comorbid disease (90% vs. 60%, p < 0.0001). Disease activity, as measured by DAS28, DAPSA and BASDAI remained stable over the 3 years. Comorbid disease (hazard ratio; HR: 4.06, p < 0.0001) and HAQ at baseline (HR: 2.42, p = 0.0024) significantly increased the risk of SB4 discontinuation, while previous ETA duration was negatively associated with SB4 discontinuation (HR: 0.97, p = 0.0064). Forty-one (17.4%) patients left the study due to the interruption of the SB4 treatment, 31 (75.6%) discontinued due to inefficacy and 10 (24.4%) due to adverse events. This real-life study confirms the similar efficacy profile of ETA with long-term retention and a good safety profile in inflammatory arthritis patients.

Published
2022

45. Relevant domains and outcome measurement instruments in neuropsychiatric systemic lupus erythematosus: a systematic literature review

Author

Silvagni, E, Chessa, E, Bergossi, F, D'Amico, M, Furini, F, Guerrini, G, Cauli, A, Scire, C, Bertsias, G, Govoni, M, Piga, M, Bortoluzzi, A, Silvagni E., Chessa E., Bergossi F., D'Amico M. E., Furini F., Guerrini G., Cauli A., Scire C. A., Bertsias G., Govoni M., Piga M., Bortoluzzi A., Silvagni, E, Chessa, E, Bergossi, F, D'Amico, M, Furini, F, Guerrini, G, Cauli, A, Scire, C, Bertsias, G, Govoni, M, Piga, M, Bortoluzzi, A, Silvagni E., Chessa E., Bergossi F., D'Amico M. E., Furini F., Guerrini G., Cauli A., Scire C. A., Bertsias G., Govoni M., Piga M., and Bortoluzzi A.

Abstract

Objectives: Although neuropsychiatric involvement in SLE (NPSLE) is one of the most complex and troubling manifestations of the disease, validated outcome instruments to be used as sensitive endpoints in controlled clinical trials are lacking. We performed a systematic literature review (SLR) to identify outcome measurement instruments and domains used to assess NPSLE. Methods: The Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were used. Articles available in English (1967-2020), listed in PubMed, Embase, PsycINFO, Cochrane Library and the EULAR outcome measures library were screened. All domains and outcome measurement instruments were characterized according to the OMERACT Filter 2.1, considering core areas (manifestations/abnormalities, life impact, death/lifespan, societal/resource use) and contextual factors. Results: Of 3392 abstracts evaluated, 83 studies were included in the SLR (15 974 patients, females 89.9%). Eligible studies included domains and instruments pertinent to all core areas defined by the OMERACT, except for 'societal/resource use'. The most common core areas were 'manifestations/abnormalities', covering 10 domains pertinent to laboratory and instrumental markers, indexes and neuropsychiatric dimension (cognitive, neurologic and psychiatric field), and 'life impact', covering 7 domains related to physical function (from both the perspective of the patient and the physician), pain and quality of life. Conclusion: Our study revealed great heterogeneity in the instruments derived from populations with NPSLE and none of these had high-quality evidence. This supports the need to develop and further validate a core domain set and outcome measurement instruments to promote clinical research in this field, enhancing comparability across studies.

Published
2022

46. Persistence of bDMARD therapy in Rheumatoid Arthritis after first-line TNF-inhibitor failure: the RECORD study of the Italian Society for Rheumatology

Author

Carrara, G, Argnani, L, Zanetti, A, Zabotti, A, Silvagni, E, Sebastiani, G, Sebastiani, M, Scire, C, Carrara G., Argnani L., Zanetti A., Zabotti A., Silvagni E., Sebastiani G. D., Sebastiani M., Scire C. A., Carrara, G, Argnani, L, Zanetti, A, Zabotti, A, Silvagni, E, Sebastiani, G, Sebastiani, M, Scire, C, Carrara G., Argnani L., Zanetti A., Zabotti A., Silvagni E., Sebastiani G. D., Sebastiani M., and Scire C. A.

Abstract

Objective: The optimal choice of a second biological disease-modifying anti-rheumatic drug (bDMARD) after failure with first line tumour necrosis factor inhibitor (TNFi) represents a critical therapeutic challenge. This study aims to evaluate the persistence with treatment using second line bDMARDs with different mechanisms of action in rheumatoid arthritis (RA) patients with inadequate response to first line TNFi. Method: A retrospective cohort study on administrative healthcare databases was conducted. We analysed the relationship between different bDMARDs and persistence with treatment in RA patients who started second line bDMARD therapy according to two different strategies: cycling (second TNFi) or switching [change in mechanism of action: abatacept (ABA), tocilizumab (TCZ), and rituximab (RTX)] with or without concomitant conventional synthetic (cs) DMARDs. Results: The cohort comprised 1434 patients. The mean age was 53.8 years and 1142 (79.6%) were women. Among second line bDMARDs, 969 patients (67.6%) started TNFi, 204 (14.2%) ABA, 145 (10.1%) RTX, and 116 (8.1%) TCZ. A bDMARD was prescribed as monotherapy in 359 patients (25.0%). The switching strategy showed a lower overall discontinuation rate [hazard ratio (HR) 0.72], while switching compared to cycling showed significantly better survival for ABA (HR 0.61) and RTX (HR 0.76), but no significant difference for TCZ (HR 0.82). A lower impact of better drug survival in the switching strategy occurred in patients with concurrent methotrexate. Conclusions: Among RA patients failing a first TNFi, switching is associated with marginally better persistence, in particular for ABA and RTX, with only marginal differences in patients on concurrent csDMARDs.

Published
2022

47. Cost-Effectiveness of the Early Arthritis Clinic Organizational Model

Author

Zanetti, A, Sakellariou, G, Zambon, A, Carrara, G, Argnani, L, Mantovani, L, Cortesi, P, Bugatti, S, Montecucco, C, Scire', C, Mantovani, LG, Cortesi, PA, Scire', CA, Zanetti, A, Sakellariou, G, Zambon, A, Carrara, G, Argnani, L, Mantovani, L, Cortesi, P, Bugatti, S, Montecucco, C, Scire', C, Mantovani, LG, Cortesi, PA, and Scire', CA

Abstract

Objective: Early diagnosis and tight control improve outcomes of rheumatoid arthritis (RA). However, whether establishing an early arthritis clinic (EAC) is sustainable for national health systems is not known. This analysis aimed to compare effectiveness and costs of an EAC compared to patients followed by the current standard of care. Methods: A retrospective study on administrative health databases of patients with a new diagnosis of RA was conducted: 430 patients followed in an EAC were enrolled, and 4 non-EAC controls were randomly matched for each. During 2 years of follow-up, the mean health care costs (outpatient, inpatient, pharmaceutical, and global) and 3 effectiveness measures (number and length of hospitalization and quality of care) of the EAC and non-EAC were estimated. The incremental cost-effectiveness ratio was calculated as well as the cost-effectiveness acceptability curve. Results: The cohorts included patients with a mean age of 55.4 years, and 1,506 patients (70%) were female. The mean pharmaceutical (2,602 versus 1,945 euros) and outpatient (2,447 versus 1,778 euros) costs were higher in the EAC cohort. Conversely, a higher rate of non-EAC patients had a low adherence to quality-of-care indicators. The expected number of hospitalizations and the length of stay were statistically significantly higher in the non-EAC versus EAC. Conclusion: Despite an expected increase in outpatient costs (visits and diagnostic tests) and pharmaceutical costs, the reduction in terms of number and length of hospitalizations and the higher adherence to international quality-of-care guidelines support the effectiveness of the EAC model.

Published
2023

48. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author

De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., Rossella, T., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., Sebastiani G. D., De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., Rossella, T., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., and Sebastiani G. D.

Abstract

Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. Methods Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets. Results Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). Conclusion The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.

Published
2023

49. The 2023 ACR/EULAR classification criteria for calcium pyrophosphate deposition disease

Author

Abhishek, A, Tedeschi, S, Pascart, T, Latourte, A, Dalbeth, N, Neogi, T, Fuller, A, Rosenthal, A, Becce, F, Bardin, T, Ea, H, Filippou, G, Fitzgerald, J, Iagnocco, A, Lioté, F, Mccarthy, G, Ramonda, R, Richette, P, Sivera, F, Andrés, M, Cipolletta, E, Doherty, M, Pascual, E, Perez-Ruiz, F, So, A, Jansen, T, Kohler, M, Stamp, L, Yinh, J, Adinolfi, A, Arad, U, Aung, T, Benillouche, E, Bortoluzzi, A, Dau, J, Maningding, E, Fang, M, Figus, F, Filippucci, E, Haslett, J, Janssen, M, Kaldas, M, Kimoto, M, Leamy, K, Navarro, G, Sarzi-Puttini, P, Scire', C, Silvagni, E, Sirotti, S, Stack, J, Truong, L, Xie, C, Yokose, C, Hendry, A, Terkeltaub, R, Taylor, W, Choi, H, Tedeschi, SK, Ea, HK, McCarthy, GM, Jansen, TL, Kohler, MJ, Stamp, LK, Fang, MA, Figus, FA, Navarro, GM, Stack, JR, Hendry, AM, Taylor, WJ, Choi, HK, Abhishek, A, Tedeschi, S, Pascart, T, Latourte, A, Dalbeth, N, Neogi, T, Fuller, A, Rosenthal, A, Becce, F, Bardin, T, Ea, H, Filippou, G, Fitzgerald, J, Iagnocco, A, Lioté, F, Mccarthy, G, Ramonda, R, Richette, P, Sivera, F, Andrés, M, Cipolletta, E, Doherty, M, Pascual, E, Perez-Ruiz, F, So, A, Jansen, T, Kohler, M, Stamp, L, Yinh, J, Adinolfi, A, Arad, U, Aung, T, Benillouche, E, Bortoluzzi, A, Dau, J, Maningding, E, Fang, M, Figus, F, Filippucci, E, Haslett, J, Janssen, M, Kaldas, M, Kimoto, M, Leamy, K, Navarro, G, Sarzi-Puttini, P, Scire', C, Silvagni, E, Sirotti, S, Stack, J, Truong, L, Xie, C, Yokose, C, Hendry, A, Terkeltaub, R, Taylor, W, Choi, H, Tedeschi, SK, Ea, HK, McCarthy, GM, Jansen, TL, Kohler, MJ, Stamp, LK, Fang, MA, Figus, FA, Navarro, GM, Stack, JR, Hendry, AM, Taylor, WJ, and Choi, HK

Abstract

Objective Calcium pyrophosphate deposition (CPPD) disease is prevalent and has diverse presentations, but there are no validated classification criteria for this symptomatic arthritis. The American College of Rheumatology (ACR) and EULAR have developed the first-ever validated classification criteria for symptomatic CPPD disease. Methods Supported by the ACR and EULAR, a multinational group of investigators followed established methodology to develop these disease classification criteria. The group generated lists of candidate items and refined their definitions, collected de-identified patient profiles, evaluated strengths of associations between candidate items and CPPD disease, developed a classification criteria framework, and used multi-criterion decision analysis to define criteria weights and a classification threshold score. The criteria were validated in an independent cohort. Results Among patients with joint pain, swelling, or tenderness (entry criterion) whose symptoms are not fully explained by an alternative disease (exclusion criterion), the presence of crowned dens syndrome or calcium pyrophosphate crystals in synovial fluid are sufficient to classify a patient as having CPPD disease. In the absence of these findings, a score>56 points using weighted criteria, comprising clinical features, associated metabolic disorders, and results of laboratory and imaging investigations, can be used to classify as CPPD disease. These criteria had a sensitivity of 92.2% and specificity of 87.9% in the derivation cohort (190 CPPD cases, 148 mimickers), whereas sensitivity was 99.2% and specificity was 92.5% in the validation cohort (251 CPPD cases, 162 mimickers). Conclusion The 2023 ACR/EULAR CPPD disease classification criteria have excellent performance characteristics and will facilitate research in this field.

Published
2023

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