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2. AB1041 IMPACT OF ANIFROLUMAB ON DISEASE BURDEN IN SYSTEMIC LUPUS ERYTHEMATOSUS: REAL-WORLD DATA FROM A MULTICENTRIC COHORT

Author

Cardelli, C., primary, Tani, C., additional, Stagnaro, C., additional, Elefante, E., additional, Zucchi, D., additional, Mazzarella, O., additional, Moroni, L., additional, Piga, M., additional, Ceccarelli, F., additional, Fasano, S., additional, De Marchi, G., additional, Urban, M. L., additional, Ramirez, G. A., additional, Chessa, E., additional, Biancalana, E., additional, Emmi, G., additional, Quartuccio, L., additional, Ciccia, F., additional, Conti, F., additional, Cauli, A., additional, Dagna, L., additional, and Mosca, M., additional

Published
2024
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3. AB1129 PATIENTS AND PHYSICIANS TREATMENT PREFERENCES FOR SYSTEMIC LUPUS ERYTHEMATOSUS: A COMPARATIVE ANALYSIS OF COMPLEMENTARY DISCRETE CHOICE EXPERIMENTS

Author

Piga, M., primary, Quartuccio, L., additional, Atzeni, F., additional, Callori, M., additional, Doria, A., additional, Emmi, G., additional, Franceschini, F., additional, Gerosa, M., additional, Mosca, M., additional, Pasqualetti, P., additional, Pelissero, R., additional, Sebastiani, G. D., additional, Conti, F., additional, and Govoni, M., additional

Published
2024
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4. AB1195 NINTEDANIB RESTORES ENDOTHELIAL FUNCTION IN PATIENTS WITH SYSTEMIC SCLEROSIS: PRELIMINARY FINDINGS OF A MONOCENTRIC STUDY

Author

Benfaremo, D., primary, Zaccone, V., additional, Sistoni Pepparoni, M., additional, Contegiacomo, S., additional, Bigoni, S., additional, Tana, C., additional, Ferraioli, Y., additional, Pacenti, N., additional, Pantaleoni, A., additional, Mea, D., additional, Piga, M. A., additional, Svegliati Baroni, S., additional, Agarbati, S., additional, Paolini, C., additional, Mozzicafreddo, M., additional, Spadoni, T., additional, Fogante, M., additional, Zuccatosta, L., additional, Argalia, G., additional, and Moroncini, G., additional

Published
2024
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5. POS0747 DIFFERENCES AND SIMILARITIES IN CARDIOVASCULAR RISK ESTIMATION WITH FIVE DIFFERENT PREDICTION MODELS IN RHEUMATOID ARTHRITIS: DATA FROM THE CARDIOVASCULAR OBESITY AND RHEUMATIC DISEASE STUDY GROUP OF THE ITALIAN SOCIETY OF RHEUMATOLOGY

Author

Cacciapaglia, F., primary, Bartoloni, E., additional, Erre, G. L., additional, Piga, M., additional, Sakellariou, G., additional, Viapiana, O., additional, Manfredi, A., additional, Gremese, E., additional, Spinelli, F. R., additional, and Atzeni, F., additional

Published
2024
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7. Gene Expression Profiling of Monozygotic Twins Affected by Psoriatic Arthritis

Author

Angioni MM, Floris A, Cangemi I, Congia M, Chessa E, Orrù S, Piga M, and Cauli A

Subjects
psoriatic arthritis, gene expression profiling, diseases in twin, genetic, environment-gene interaction, joint erosions, Diseases of the musculoskeletal system, RC925-935
Abstract

Maria Maddalena Angioni,1 Alberto Floris,1 Ignazio Cangemi,1 Mattia Congia,1 Elisabetta Chessa,1 Sandro Orrù,2 Matteo Piga,1 Alberto Cauli1 1Rheumatology Unit, Department of Medical Sciences and Public Health, University of Cagliari, University Clinic AOU, Cagliari, 09042, Italy; 2Medical Genetics, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, 09042, ItalyCorrespondence: Maria Maddalena AngioniRheumatology Unit, Department of Medical Sciences and Public Health, University of Cagliari, University Clinic AOU Cagliari, S.S. 554, Bivio per Sestu, Monserrato, Cagliari, 09042, ItalyTel +39 07051093340Email m.maddalena.angioni@gmail.comIntroduction: Psoriatic Arthritis (PsA) is a multifactorial disease, where the relative burden of genetic, epigenetic and environmental factors in clinical course and damage accrual is not yet definitively clarified. In clinical practice, there is a real need for useful candidate biomarkers in PsA diagnosis and disease progression, by exploring its underlying transcriptomic and epigenomic mechanisms. This work aims to profile the transcriptome in monozygotic (MZ) twins with psoriatic arthritis (PsA) highly concordant for clinical presentation, but discordant for the radiographic outcomes’ severity.Methods: We describe i) the clinical case of two MZ twins; ii) their comparative gene expression profiling (HTA 2.0 Affymetrix) and iii) signal pathways and pathophysiological processes in which differentially expressed genes are involved (in silico analysis by the IPA software, QIAGEN).Results: One hundred sixty-three transcripts and 36 coding genes (28 up and 8 down) were differentially expressed between twins, and in the brother with the most erosive form, the transcriptomic profiling highlights the overexpression of genes known to be involved in immunomodulatory processes and on a broad spectrum of PsA manifestations.Discussion: Twins’ clinical cases are still a gold mine in medical research: twin brothers are ideal experimental models in estimating the relative importance of genetic versus nongenetic components as determinants of complex phenotypes, non-Mendelian and multifactorial diseases as PsA.Keywords: psoriatic arthritis, gene expression profiling, diseases in twin, genetic, environment–gene interaction, joint erosions

Published
2021

8. 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

9. 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

10. 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

11. Assessment Of Circulating Endothelial Cells And Their Progenitors As Potential Biomarkers Of Disease Activity And Damage Accrual In Behçet’s Syndrome

Author

Floris A, Piga M, Pinna S, Angioni MM, Congia M, Mascia P, Chessa E, Cangemi I, Mathieu A, and Cauli A

Subjects
Circulating Endothelial Cells (CECs), Endothelial Progenitor Cells (EPCs), Behçet’s Syndrome., Diseases of the musculoskeletal system, RC925-935
Abstract

Alberto Floris, Matteo Piga, Silvia Pinna, Maria Maddalena Angioni, Mattia Congia, Piero Mascia, Elisabetta Chessa, Ignazio Cangemi, Alessandro Mathieu, Alberto Cauli Rheumatology Unit, AOU University Clinic and University of Cagliari, Cagliari, ItalyCorrespondence: Matteo PigaRheumatology Unit, University Clinic AOU of Cagliari, SS 554, Monserrato, CA 09042, ItalyTel +390706754069Fax +39070513157Email matteopiga@unica.itPurpose: To explore the potential role of circulating endothelial cells (CECs) and their progenitors (EPCs) as biomarkers of disease activity and damage accrual in patients with Behçet’s syndrome (BS), by using a standardised and reliable flow cytometry protocol.Patients and methods: CECs and EPCs were assessed in 32 BS patients and 11 gender/age/smoking habits matched healthy controls (HC). They were identified by flow cytometry as alive/nucleated/CD45-negative/CD34-bright/CD146-positive and alive/nucleated/CD45-negative/CD34-bright/CD309-positive events, respectively. In BS patients, demographic and clinical features, including disease activity (assessed by Behçet’s disease current disease activity form, BDCAF) and irreversible damage accrual (by the vasculitis damage index, VDI) were recorded. Uni- and multivariate analysis were performed to compare the CECs and EPCs concentrations in BS vs HC and to identify potential associations with demographic or clinical features.Results: The CECs concentration was significantly higher in the BS patients than HCs [median (IQR) 15.0 (7.5–23.0) vs 6.0 (2.0–13.0) CECs/mL, p=0.024]. In BS patients, no significant associations were found between CECs and demographic features, present and past clinical manifestations, BDCAF score and ongoing treatment. A significant association was observed between CECs and organ damage, as assessed by the VDI (rho 0.356, p=0.045). Higher levels of CECs were especially associated with vascular damage [median (IQR) 23.0 (14.0–47.0) vs 13.0 (6.0–19.0) CECs/mL, p=0.011], including arterial aneurysm and stenosis, complicated venous thrombosis, cerebrovascular accident. The concentration of EPCs did not significantly differ between the BS and HC [median 26.5 (13.0–46.0) vs 19.0 (4.0–42.0) EPCs/mL, p=0.316] and no significant associations were observed between their levels and any clinical characteristic.Conclusion: Our study suggests that the CECs concentration is significantly higher in BS than healthy subjects, and it mainly correlates with vascular damage. A longitudinal extension of the present study on a wider cohort would be useful to validate the potential role of CECs as a marker or, hopefully, predictor of vascular damage in BS.Keywords: circulating endothelial cells, CECs, endothelial progenitor cells, EPCs, Behçet’s syndrome

Published
2019

12. OP0027 APPLICATION OF PREVENTIVE CV RISK STRATEGIES FOR DYSLIPIDEMIA IN RHEUMATOID ARTHRITIS PATIENTS: DATA FROM THE CARDIOVASCULAR OBESITY AND RHEUMATIC DISEASES (CORDIS) STUDY GROUP OF THE ITALIAN SOCIETY OF RHEUMATOLOGY

Author

Cacciapaglia, F., primary, Piga, M., additional, Erre, G. L., additional, Sakellariou, G., additional, Manfredi, A., additional, Viapiana, O., additional, Gremese, E., additional, Spinelli, F. R., additional, Atzeni, F., additional, and Bartoloni, E., additional

Published
2023
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15. POS1172 GEOGRAPHICAL VARIABILITY OF ORGAN DAMAGE BURDEN IN A LARGE INTERNATIONAL MULTICENTER COHORT OF PATIENTS WITH BEHÇET’S SYNDROME: PRELIMINARY RESULTS OF THE PROBE STUDY

Author

Floris, A., primary, Chadli, S., additional, Lamouna, A., additional, Hegazy, M. T., additional, Yagiz Ozogul, Y., additional, Ozguler, Y., additional, Serpa Pinto, L., additional, Alikhani, M., additional, Oliveira, L., additional, Sota, J., additional, Lopalco, G., additional, Poddighe, D., additional, Kougkas, N., additional, Khabbazi Oskouei, A., additional, Laconi, R., additional, Wagner Silva de Souza, A., additional, Lo Monaco, A., additional, Espinosa, G., additional, Butendieck, R., additional, Govoni, M., additional, Issayeva, B., additional, Iannone, F., additional, Fabiani, C. M., additional, Cantarini, L., additional, Vasconcelos, C., additional, Shahram, F., additional, Correia, J., additional, Tazi Mezalek, Z., additional, Ragab, G., additional, Arnaud, L., additional, Cauli, A., additional, Hatemi, G., additional, and Piga, M., additional

Published
2023
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18. AB0558 ANIFROLUMAB IN REFRACTORY SYSTEMIC LUPUS ERYTHEMATOSUS: A REAL-LIFE, MULTICENTER STUDY

Author

Trentin, F., primary, Tani, C., additional, Cauli, A., additional, Ceccarelli, F., additional, Ciccia, F., additional, Conti, F., additional, Coladonato, L., additional, Dagna, L., additional, De Marchi, G., additional, Emmi, G., additional, Fasano, S., additional, Gatto, M., additional, Moroni, L., additional, Riccio, F., additional, Piga, M., additional, Zen, M., additional, and Mosca, M., additional

Published
2023
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19. 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

20. The AutoInflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases

Author

Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, Conti G, Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, and Conti G

Abstract

Objective: The present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network. Methods: This is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform. Results: AIDA project will share knowledge and expertise on mAIDs. Since its start, 118 centers from 24 countries and 4 continents have joined the AIDA project. Fifty-nine centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 337 users (122 Principal Investigators, 210 Site Investigators, 2 Lead Investigators, and 3 data managers). The Registry collects baseline and follow-up data using 3,748 fields organized into 21 instruments, which include demographics, patient history, symptoms, trigger/risk factors, therapies, and healthcare information for mAIDs patients. Conclusions: The AIDA mAIDs Registry, acts both as a research tool for future collaborative real-life studies on mAIDs and as a service to connect all the figures called to participate. On this basis, the registry is expected to play a pivotal role in generating new scientific evidence on this group of rare diseases, substantially improving the management of patients, and optimizing the impact on the healthcare system. NCT 05200715 available at https://clinicaltrials.gov

Published
2022

21. 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

22. 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

23. 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

24. Severe neuropsychiatric systemic lupus erythematosus successfully treated with rituximab: an alternative to standard of care

Author

Chessa E, Piga M, Floris A, Mathieu A, and Cauli A

Subjects
Systemic lupus erythematosus, neuropsychiatric lupus, rituximab, demyelinating syndrome, brain MRI., Diseases of the musculoskeletal system, RC925-935
Abstract

Elisabetta Chessa, Matteo Piga, Alberto Floris, Alessandro Mathieu, Alberto Cauli Rheumatology Unit, University Clinic AOU of Cagliari, Cagliari, Italy Abstract: Demyelinating syndrome secondary to systemic lupus erythematosus (DS-SLE) is a rare encephalomyelitis burden with a high risk of disability and death. We report on a 49-year-old Caucasian woman with systemic lupus erythematosus (SLE) complicated by severe cognitive dysfunction, brainstem disease, cranial nerve palsies, weakness and numbness in limbs and multiple discrete magnetic resonance imaging (MRI) areas of damage within the white matter of semioval centers, temporal lobe, external capsule, claustrum, subinsular regions and midbrain. She also had multiple mononeuritis diagnosed through sensory and motor nerve conduction study. She was diagnosed with severe DS-SLE prominently involving the brain and was treated with 500 mg methylprednisolone (PRE) pulses for 3 consecutive days, followed by one single pulse of 500 mg cyclophosphamide, and 1 g rituximab, which was then repeated 14 days later. PRE 25 mg/day, rapidly tapered to 7.5 mg/day in 6 months, and mycophenolate mofetil 1 g/day were prescribed as maintenance therapy. She had progressive and sustained improvement in neurological symptoms with almost complete resolution of brain MRI lesions after 1 year. B-cell depleting therapy could be considered as a possible alternative to standard of care in the management of severe inflammatory neuropsychiatric SLE but it should be associated with a conventional immunosuppressant as maintenance treatment to reduce the risk of flare and reduce corticosteroids dose. Keywords: systemic lupus erythematosus, neuropsychiatric lupus, rituximab, demyelinating syndrome, brain MRI

Published
2017

25. A patient-driven registry on Behçet's disease: the AIDA for patients pilot project

Author

Gaggiano, C, Del Bianco, A, Sota, J, Gentileschi, S, Ruscitti, P, Giacomelli, R, Piga, M, Crisafulli, F, Monti, S, Emmi, G, De Paulis, A, Vitale, A, Tarsia, M, Caggiano, V, Nuzzolese, R, Parretti, V, Fabiani, C, Lopalco, G, Maier, A, Cattalini, M, Rigante, Donato, Govoni, M, Li Gobbi, F, Guiducci, S, Parronchi, P, Marino, A, Ciccia, F, Maggio, Mc, Aragona, E, Bartoloni, E, Iagnocco, A, Viapiana, O, Sebastiani, Gd, Guerriero, S, Insalaco, A, Del Giudice, E, Conti, G, Barone, P, Olivieri, An, Brucato, A, Carubbi, F, Triggianese, P, Mauro, A, Tosi, Gm, Fonollosa, A, Giardini, Ham, Ragab, G, Tharwat, S, Hernández-Rodríguez, J, Sfikakis, Pp, Laskari, K, Karamanakos, A, Espinosa, G, Shahram, F, Direskeneli, H, Hinojosa-Azaola, A, Opris-Belinski, D, Almaghlouth, Ia, Hatemi, G, Eksin, Ma, Önen, F, Więsik-Szewczyk, E, Akkoç, N, Tufan, A, Şahin, A, Erten, Ş, Ozen, S, Batu, Ed, Frediani, B, Balistreri, A, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Del Bianco, A, Sota, J, Gentileschi, S, Ruscitti, P, Giacomelli, R, Piga, M, Crisafulli, F, Monti, S, Emmi, G, De Paulis, A, Vitale, A, Tarsia, M, Caggiano, V, Nuzzolese, R, Parretti, V, Fabiani, C, Lopalco, G, Maier, A, Cattalini, M, Rigante, Donato, Govoni, M, Li Gobbi, F, Guiducci, S, Parronchi, P, Marino, A, Ciccia, F, Maggio, Mc, Aragona, E, Bartoloni, E, Iagnocco, A, Viapiana, O, Sebastiani, Gd, Guerriero, S, Insalaco, A, Del Giudice, E, Conti, G, Barone, P, Olivieri, An, Brucato, A, Carubbi, F, Triggianese, P, Mauro, A, Tosi, Gm, Fonollosa, A, Giardini, Ham, Ragab, G, Tharwat, S, Hernández-Rodríguez, J, Sfikakis, Pp, Laskari, K, Karamanakos, A, Espinosa, G, Shahram, F, Direskeneli, H, Hinojosa-Azaola, A, Opris-Belinski, D, Almaghlouth, Ia, Hatemi, G, Eksin, Ma, Önen, F, Więsik-Szewczyk, E, Akkoç, N, Tufan, A, Şahin, A, Erten, Ş, Ozen, S, Batu, Ed, Frediani, B, Balistreri, A, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Introduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet’s disease (BD). Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry. Results: Respondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behçet’s Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0–30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1–50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 ± 1.1 (range – 1.8–4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status (p < 0.001), the presence of any major organ involvement (p < 0.031), the presence of gastro-intestinal (p < 0.001), neurological (p = 0.012) and musculoskeletal (p = 0.022) symptoms, recurrent fever (p = 0.002), and headache (p < 0.001) were associated to a higher number of accesses to the healthcare system. Multiple linear regression showed that the BDQoL score could significantly predict the global socioeconomic impact of BD (F = 14.519, OR 1.162 [CI 0.557–1.766], p < 0.001). Discussion: Preliminary results from the AID

Published
2023

26. Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry

Author

Gaggiano, C, Maselli, A, Sfikakis, Pp, Laskari, K, Ragab, G, Hegazy, Mt, Laymouna, Ah, Lopalco, G, Almaghlouth, Ia, Asfina, Kn, Alahmed, O, Giardini Mayrink, Ha, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, Mc, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, An, Tufan, A, Karadeniz, H, Kardaş, Rc, La Torre, F, Cardinale, F, Marino, A, Guerriero, S, Ruscitti, P, Tarsia, M, Vitale, A, Caggiano, V, Telesca, S, Iannone, F, Parretti, V, Frassi, M, Aragona, E, Ciccia, F, Wiesik-Szewczyk, E, Ionescu, R, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Maselli, A, Sfikakis, Pp, Laskari, K, Ragab, G, Hegazy, Mt, Laymouna, Ah, Lopalco, G, Almaghlouth, Ia, Asfina, Kn, Alahmed, O, Giardini Mayrink, Ha, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, Mc, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, An, Tufan, A, Karadeniz, H, Kardaş, Rc, La Torre, F, Cardinale, F, Marino, A, Guerriero, S, Ruscitti, P, Tarsia, M, Vitale, A, Caggiano, V, Telesca, S, Iannone, F, Parretti, V, Frassi, M, Aragona, E, Ciccia, F, Wiesik-Szewczyk, E, Ionescu, R, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet’s syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behçet’s Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.2%). The median age at onset was 10.0 years (IQR 7.7). The median follow-up duration was 21.8 years (IQR 23.3). Recurrent oral (100%) and genital ulcers (67.6%) and pseudofolliculitis (56.8%) were the most common symptoms associated with MSM. At disease onset, 31 subjects had arthritis (83.8%), 33 arthralgia (89.2%), and 14 myalgia (37.8%). Arthritis was monoarticular in 9/31 cases (29%), oligoarticular in 10 (32.3%), polyarticular in 5 (16.1%), axial in 7 (22.6%). Over time, arthritis became chronic-recurrent in 67.7% of cases and 7/31 patients had joint erosions (22.6%). The median Behçet's Syndrome Overall Damage Index was 0 (range 0–4). Colchicine was inefcacious for MSM in 4/14 cases (28.6%), independently from the type of MSM (p=0.46) or the concomitant therapy (p=0.30 for cDMARDs, p=1.00 for glucocorticoids); cDMARDs and bDMARDs were inefcacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefcacy (p=0.014). To conclude, MSM in children with BS are frequently associated with recurrent ulcers and pseudofolliculitis. Arthritis is mostly mono- or oligoarticular, but sacroiliitis is not unusual. Prognosis of this subset of BS is overall favorable, though the presence of myalgia negatively afects response to biologic therapies.

Published
2023

27. La fatigue est indépendamment associée à l’activité de la maladie évaluée par le Physician Global Assessment (PGA) chez les patients atteints de lupus systémique

Author

Mertz, P., primary, Piga, M., additional, Chessa, E., additional, Amoura, Z., additional, Voll, R., additional, Schwarting, A., additional, Maurier, F., additional, Blaison, G., additional, Bonnotte, B., additional, Poindron, V., additional, Fiehn, C., additional, Lorenz, H.M., additional, Korganow, A.S., additional, Sibilia, J., additional, Martin, T., additional, and Arnaud, L., additional

Published
2022
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29. Current perspective on the role of the interleukin-23/interleukin-17 axis in inflammation and disease (chronic arthritis and psoriasis)

Author

Cauli A, Piga M, Floris A, and Mathieu A

Subjects
TH17, IL-17, IL-23, Psoriasis, Psoriatic Arthritis, Ankylosing Spondylitis, Immunologic diseases. Allergy, RC581-607
Abstract

Alberto Cauli, Matteo Piga, Alberto Floris, Alessandro Mathieu Rheumatology Unit, Department of Medical Sciences, Policlinico of the University of Cagliari, Monserrato, Cagliari, Italy Abstract: TH17 is a lymphocyte subset, which is characterized by its polarization to secrete interleukin (IL)-17. IL-23 is the pivotal mediator responsible for TH17 differentiation and the IL-23/IL-17 axis has been strongly implicated in the pathogenesis of several immune mediated diseases, in particular chronic arthritis and skin psoriasis. This review will summarize the basic immunology and the new monoclonal antibodies, which antagonize this pathway allowing a new therapeutic approach. Keywords: TH17, IL-17, IL-23, psoriasis, psoriatic arthritis, ankylosing spondylitis

Published
2015

31. S14.1 The effect of anti-ribosomal-p and anti-dweys antibodies on depression and behavioral cognitive processes in systemic lupus erythematosus : an integrated clinical and functional MRI study

Author

Chessa, E, primary, Porcu, M, additional, Pintus, E, additional, Perra, A, additional, Floris, A, additional, Angioni, MM, additional, Congia, M, additional, Carta, MG, additional, Saba, L, additional, Mathieu, A, additional, Cauli, A, additional, and Piga, M, additional

Published
2022
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32. PO.6.133 Dynamical trajectory of glucocorticoid tapering and discontinuation in real-world patients with newly diagnosed systemic lupus erythematosus: the gulp study

Author

Piga, M, primary, Chessa, E, additional, Floris, A, additional, Sebastiani, GD, additional, Prevete, I, additional, Iannone, F, additional, Coladonato, L, additional, Govoni, M, additional, Bortoluzzi, A, additional, Mosca, M, additional, Tani, C, additional, Doria, A, additional, Iaccarino, L, additional, Franceschini, F, additional, Fredi, M, additional, Conti, F, additional, Spinelli, FR, additional, Bellisai, F, additional, D’Alessandro, R, additional, Zanetti, A, additional, Carrara, G, additional, Scirè, CA, additional, and Cauli, A, additional

Published
2022
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33. Clinical potential of apremilast in the treatment of psoriatic arthritis

Author

Cauli A, Porru G, Piga M, Vacca A, Dessole G, and Mathieu A

Subjects
Psoriatic Arthritis, Apremilast, Therapy., Immunologic diseases. Allergy, RC581-607
Abstract

Alberto Cauli, Giovanni Porru, Matteo Piga, Alessandra Vacca, Grazia Dessole, Alessandro MathieuRheumatology Unit, Department of Medical Sciences, Policlinico of University of Cagliari, Monserrato, ItalyAbstract: Psoriatic arthritis (PsA) is a frequent chronic inflammatory disease characterized by joint and skin involvement, and by typical extra-articular manifestations. Although the pathogenesis of PsA is still under investigation, the available evidence suggests the importance of the patient's genetic background, microbial or environmental triggers, and an imbalance in the adaptive and acquired immune system, resulting in the production of inflammatory mediators. New therapeutic approaches have been proposed, among them the use of modulators of intracellular signals and gene transcription such as PDE4-inhibiting compounds, which are able to modulate the activity of transcription factors such as CREB and NF-κB and therefore the synthesis of inflammatory mediators, resulting in immunoregulation. This paper summarizes the mechanism of action of apremilast, a PDE4 inhibitor, and the clinical data available on its clinical efficacy and safety profile in the treatment of PsA patients.Keywords: psoriatic arthritis, apremilast, therapy

Published
2014

34. AB0636 Relationship between organ damage and impairment of health-related quality of life in patients with Behçet’s Syndrome: results from a longitudinal extension of the BODI Project.

Author

Floris, A., primary, Laconi, R., additional, Espinosa, G., additional, Lopalco, G., additional, Serpa Pinto, L., additional, Kougkas, N., additional, Sota, J., additional, Lo Monaco, A., additional, Govoni, M., additional, Cantarini, L., additional, Bertsias, G., additional, Correia, J., additional, Iannone, F., additional, Cervera, R., additional, Vasconcelos, C., additional, Mathieu, A., additional, Cauli, A., additional, and Piga, M., additional

Published
2022
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35. POS0787 THE EFFECT OF ANTI-RIBOSOMAL-P AND ANTI-NR2 ANTIBODIES ON FUNCTIONAL BRAIN MRI NETWORKS IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH DEPRESSION AND BEHAVIORAL COGNITIVE DISORDERS.

Author

Chessa, E., primary, Piga, M., additional, Porcu, M., additional, Pintus, E., additional, Perra, A., additional, Serafini, C., additional, Floris, A., additional, Congia, M., additional, Angioni, M. M., additional, Carta, M. G., additional, Saba, L., additional, Mathieu, A., additional, and Cauli, A., additional

Published
2022
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36. AB0630 Assessment of organ damage accrual in Behçet's Syndrome over 2-year follow-up: results from the BODI Project longitudinal extension.

Author

Floris, A., primary, Laconi, R., additional, Espinosa, G., additional, Lopalco, G., additional, Serpa Pinto, L., additional, Kougkas, N., additional, Sota, J., additional, Lo Monaco, A., additional, Govoni, M., additional, Cantarini, L., additional, Bertsias, G., additional, Correia, J., additional, Iannone, F., additional, Cervera, R., additional, Vasconcelos, C., additional, Mathieu, A., additional, Cauli, A., additional, and Piga, M., additional

Published
2022
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37. AB0631 Impact of Behçet’s Syndrome on work activity and productivity: results from a sub-analysis of the BODI Project cohort.

Author

Laconi, R., primary, Floris, A., additional, Espinosa, G., additional, Lopalco, G., additional, Serpa Pinto, L., additional, Kougkas, N., additional, Sota, J., additional, Lo Monaco, A., additional, Govoni, M., additional, Cantarini, L., additional, Bertsias, G., additional, Correia, J., additional, Iannone, F., additional, Cervera, R., additional, Vasconcelos, C., additional, Mathieu, A., additional, Cauli, A., additional, and Piga, M., additional

Published
2022
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39. Risk factors of damage in early diagnosed systemic lupus erythematosus: results of the Italian multicentre Early Lupus Project inception cohort

Author

Piga, M, Floris, A, 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, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scirè, C, Mathieu, A, Piga M, Floris A, Sebastiani GD, 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 FR8, Galeazzi M, Bellisai F, Zanetti A, Carrara G, Scirè CA, Mathieu A, Piga, M, Floris, A, 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, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scirè, C, Mathieu, A, Piga M, Floris A, Sebastiani GD, 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 FR8, Galeazzi M, Bellisai F, Zanetti A, Carrara G, Scirè CA, and Mathieu A

Abstract

Objective: To investigate risk factors for damage development in a prospective inception cohort of early diagnosed SLE patients. Methods: The Early Lupus Project recruited an inception cohort of patients within 12 months of SLE classification (1997 ACR criteria). At enrolment and every 6 months thereafter, the SLICC/ACR Damage Index was recorded. The contribution of baseline and time-varying covariates to the development of damage, defined as any SLICC/ACR Damage Index increase from 0 to ≥1, was assessed using univariate analysis. Forward-backward Cox regression models were fitted with covariates with P < 0.05 to identify factors independently associated with the risk of damage development. Results: Overall, 230 patients with a mean (s.d.) age of 36.5 (14.4) years were eligible for this study; the mean number of visits per patient was 5.3 (2.7). There were 51 (22.2%) patients with SLICC/ACR Damage Index ≥1 after 12 months, 59 (25.6%) after 24 months and 67 (29.1%) after 36 months. Dyslipidaemia [P = 0.001; hazard ratio (HR) 2.9; 95% CI 1.5, 5.6], older age (P = 0.001; HR 3.0; 95% CI 1.6, 5.5), number of organs/systems involved (P = 0.002; HR 1.4; 95% CI 1.1, 1.8) and cardiorespiratory involvement (P = 0.041; HR 1.9; 95% CI 1.0, 3.7) were independently associated with an increased risk of developing damage. Risk profiles for damage development differed for glucocorticoid-related and-unrelated damage. HCQ use (P = 0.005; HR 0.4; 95% CI 0.2, 0.8) reduced the risk of glucocorticoid-unrelated damage. Conclusion: We identified risk factors of damage development, but little effect of glucocorticoids, in this early SLE cohort. Addressing modifiable risk factors from the time of SLE diagnosis might improve patient outcomes.

Published
2020

41. Development and implementation of the AIDA International Registry for patients with Behçet's disease

Author

Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ö, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L &amp, Autoinflammatory Diseases Alliance (AIDA), Network, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ö, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L &amp, Autoinflammatory Diseases Alliance (AIDA), Network, Conti G (ORCID:0000-0002-8566-9365), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet’s disease (BD). Methods: The Registry is a clinical physician-driven population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Results: Starting from January 31st to November 23rd, 2021, 99 centres from 20 countries in 4 continents have been involved. Forty-eight of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 265 users (99 Principal Investigators, 162 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5474 fields organised into 15 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: The development of the AIDA International Registry for BD patients will facilitate the collection of standardised data leading to real-world evidence, enabling international multicentre collaborative research through data sharing, international consultation, dissemination of knowledge, inclusion of patients and families, and ultimately optimisation of scientific efforts and implementation of standardised care.

Published
2022

42. Development and implementation of the AIDA International Registry for patients with VEXAS syndrome

Author

Vitale, A, Caggiano, V, Della Casa, F, Hernández-Rodríguez, J, Frassi, M, Monti, S, Tufan, A, Telesca, S, Conticini, E, Ragab, G, Lopalco, G, Almaghlouth, I, Pereira, Rmr, Yildirim, D, Cattalini, M, Marino, A, Giani, T, La Torre, F, Ruscitti, P, Aragona, E, Wiesik-Szewczyk, E, Del Giudice, E, Sfikakis, Pp, Govoni, M, Emmi, G, Maggio, Mc, Giacomelli, R, Ciccia, F, Conti, Giorgio, Ait-Idir, D, Lomater, C, Sabato, V, Piga, M, Sahin, A, Opris-Belinski, D, Ionescu, R, Bartoloni, E, Franceschini, F, Parronchi, P, de Paulis, A, Espinosa, G, Maier, A, Sebastiani, Gd, Insalaco, A, Shahram, F, Sfriso, P, Minoia, F, Alessio, M, Makowska, J, Hatemi, G, Akkoç, N, Li Gobbi, F, Gidaro, A, Olivieri, An, Al-Mayouf, Sm, Erten, S, Gentileschi, S, Vasi, I, Tarsia, M, Mahmoud, Aaa, Frediani, B, Fares Alzahrani, M, Laymouna, Ah, Ricci, F, Cardinale, F, Jahnz-Rózyk, K, Tosi, Gm, Crisafulli, F, Balistreri, A, Dagostin, Ma, Ghanema, M, Gaggiano, C, Sota, J, Di Cola, I, Fabiani, C, Giardini, Ham, Renieri, A, Fabbiani, A, Carrer, A, Bocchia, M, Caroni, F, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Caggiano, V, Della Casa, F, Hernández-Rodríguez, J, Frassi, M, Monti, S, Tufan, A, Telesca, S, Conticini, E, Ragab, G, Lopalco, G, Almaghlouth, I, Pereira, Rmr, Yildirim, D, Cattalini, M, Marino, A, Giani, T, La Torre, F, Ruscitti, P, Aragona, E, Wiesik-Szewczyk, E, Del Giudice, E, Sfikakis, Pp, Govoni, M, Emmi, G, Maggio, Mc, Giacomelli, R, Ciccia, F, Conti, Giorgio, Ait-Idir, D, Lomater, C, Sabato, V, Piga, M, Sahin, A, Opris-Belinski, D, Ionescu, R, Bartoloni, E, Franceschini, F, Parronchi, P, de Paulis, A, Espinosa, G, Maier, A, Sebastiani, Gd, Insalaco, A, Shahram, F, Sfriso, P, Minoia, F, Alessio, M, Makowska, J, Hatemi, G, Akkoç, N, Li Gobbi, F, Gidaro, A, Olivieri, An, Al-Mayouf, Sm, Erten, S, Gentileschi, S, Vasi, I, Tarsia, M, Mahmoud, Aaa, Frediani, B, Fares Alzahrani, M, Laymouna, Ah, Ricci, F, Cardinale, F, Jahnz-Rózyk, K, Tosi, Gm, Crisafulli, F, Balistreri, A, Dagostin, Ma, Ghanema, M, Gaggiano, C, Sota, J, Di Cola, I, Fabiani, C, Giardini, Ham, Renieri, A, Fabbiani, A, Carrer, A, Bocchia, M, Caroni, F, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: The aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination. Methods: The present registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients’ management; the registry may potentially communicate with other on-line tools dedicated to VEXAS syndrome, enhancing international collaboration and data sharing for research purposes. The registry is handy enough to be easily modified to meet future needs regarding VEXAS syndrome. Results: To date (April 2022), 105 Centers from 23 Countries in 4 continents have been involved; 287 users (106 Principal Investigators, 177 Site Investigators, 2 Lead Investigators, and 2 data managers) may already enter the registry for data collection and sharing. The registry includes 4950 fields organised into 18 instruments designed to fully describe patient’s details about demographics, clinical manifestations, symptoms, histologic details about skin and bone marrow biopsies and aspirate, laboratory features, complications, comorbidities, therapies, and healthcare access. Conclusions: This international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease in a relatively short time with the final goal to obtain real-world evidence data for daily clinical practice. This project can be found on https://clinicaltrials.gov NCT 05200715

Published
2022

43. Estimated 10-year cardiovascular risk in a large Italian cohort of rheumatoid arthritis patients: Data from the Cardiovascular Obesity and Rheumatic DISease (CORDIS) Study Group

Author

Cacciapaglia, F., Spinelli, F. R., Piga, M., Erre, G. L., Sakellariou, G., Manfredi, A., Viapiana, O., Fornaro, M., Colella, S., Floris, A., Mangoni, A. A., Castagna, F., Vacchi, Caterina, Orsolini, G., Bugatti, S., Cafaro, G., Cauli, A., Gremese, Elisa, Atzeni, F., Bartoloni, E., Vacchi C., Gremese E. (ORCID:0000-0002-2248-1058), Cacciapaglia, F., Spinelli, F. R., Piga, M., Erre, G. L., Sakellariou, G., Manfredi, A., Viapiana, O., Fornaro, M., Colella, S., Floris, A., Mangoni, A. A., Castagna, F., Vacchi, Caterina, Orsolini, G., Bugatti, S., Cafaro, G., Cauli, A., Gremese, Elisa, Atzeni, F., Bartoloni, E., Vacchi C., and Gremese E. (ORCID:0000-0002-2248-1058)

Abstract

Background: Several cardiovascular (CV) risk algorithms are available to predict CV events in the general population. However, their performance in patients with rheumatoid arthritis (RA) might differ from the general population. This cross-sectional multicentre study aimed to estimate the 10-year CV risk using two different algorithms in a large RA cohort and in patients with osteoarthritis (OA). Methods: In a consecutive series of RA patients and matched OA controls without prior CV events, clinical and serologic data and traditional CV risk factors were recorded. The 10-year CV risk was assessed with the Systematic COronary Risk Evaluation (SCORE) and the “Progetto Cuore” algorithms. Results: 1,467 RA patients and 342 OA subjects were included. RA patients were more frequently diabetic (9.9% vs 6.4%; p=0.04) and smokers (20.4% vs 12.5%; p=0.002) but had lower prevalence of obesity (15% vs 21%; p=0.003). Dyslipidaemia was more prevalent in OA (32.5% vs 21.7%; p<0.0001). The 10-year estimated CV risk was 1.6% (95%CI 1.3-1.9) in RA and 1.4% (95%CI 1.3-1.6) in OA (p=0.002) according to SCORE and 6.5% (95%CI 6.1-6.9) in RA and 4.4% (95%CI 3.9-5.1) in OA (p<0.001) according to “Progetto Cuore”. Regardless of the score used, RA patients had a 3- to-4-fold increased 10-year risk of CV events compared to OA subjects. Conclusion: RA patients have a significantly higher 10-year risk of CV events than OA subjects. In addition to effective disease control and joint damage prevention, specific protective measures targeting modifiable traditional CV risk factors should be implemented in RA.

Published
2022

44. 'Capitolo 5. Istituzioni'

Author

Piga, M. L., Bertolini, S., Calloni, M., D’Amico, M., Gammaitoni, M., Musumeci, R., Naldini, M., Picardi, I., and Ruspini, E.

Published
2022

45. Risk of acute arterial and venous thromboembolic events in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Author

Bettiol, A., Sinico, R. A., Schiavon, F., Monti, S., Bozzolo, E. P., Franceschini, F., Govoni, M., Lunardi, C., Guida, G., Lopalco, G., Paolazzi, G., Vacca, A., Gregorini, G., Leccese, P., Piga, M., Conti, F., Fraticelli, P., Quartuccio, L., Alberici, F., Salvarani, C., Bettio, S., Negrini, S., Selmi, C., Sciascia, S., Moroni, G., Colla, L., Manno, C., Urban, M. L., Vannacci, A., Pozzi, M. R., Fabbrini, P., Polti, S., Felicetti, M., Marchi, M. R., Padoan, R., Delvino, P., Caporali, R., Montecucco, C., Dagna, L., Cariddi, A., Toniati, P., Tamanini, S., Furini, F., Bortoluzzi, A., Tinazzi, E., Delfino, L., Badiu, I., Rolla, G., Venerito, V., Iannone, F., Berti, A., Bortolotti, R., Racanelli, V., Jeannin, G., Padula, A., Cauli, A., Priori, R., Gabrielli, A., Bond, M., Tedesco, M., Pazzola, G., Tomietto, P., Pellecchio, M., Marvisi, C., Maritati, F., Palmisano, A., Dejaco, C., Willeit, J., Kiechl, S., Olivotto, I., Willeit, P., Prisco, D., Vaglio, A., Emmi, G., Bargagli, E., Becatti, M., Beccalli, M., Bello, F., Bozzao, F., Canti, V., Cassia, M. A., Cassone, G., Catanoso, M., Chieco-Bianchi, F., Clari, R., Coladonato, L., De Santis, M., Di Scala, G., Fagni, F., Fenaroli, P., Fiorillo, C., Floris, A., Fornaro, M., Galli, E., Generali, E., Giliberti, M., Lascaro, N., Leccese, I., Mattioli, I., Olivieri, B., Osti, N., Peyronel, F., Radin, M., Righetti, G., Salvati, S., Silvestri, E., Susca, N., Tamburini, C., Taurisano, G., Trezzi, B., Trivioli, G., Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, and Emmi, G

Subjects
Pulmonary and Respiratory Medicine, Burden of disease, Humans, Churg-Strauss Syndrome, Granulomatosis with Polyangiitis, Venous Thromboembolism, Venous Thrombosis, Churg-strauss syndrome, Criminology, NO, 03 medical and health sciences, 0302 clinical medicine, Medicine, 030212 general & internal medicine, Vascular inflammation, business.industry, Conflict of interest, Cytoplasmic antibody, medicine.disease, 030228 respiratory system, Wegener granulomatosis, arterial and venous thromboembolic events, Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome), Organ involvement, business, Production team
Abstract

Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg-Strauss syndrome) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) characterised by respiratory manifestations and systemic organ involvement [1]. Particularly, cardiac manifestations occur in 40–60% of patients, representing the leading cause of mortality [2]. Recent reports suggest that venous thromboembolic events might also represent a consistent burden of disease [3, 4], as already known for the other AAVs [5–7], possibly due to eosinophil-mediated vascular inflammation [5]. Nevertheless, the occurrence of arterial and venous thrombotic events (AVTE) has never been systematically explored in EGPA. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Alessandra Bettiol Conflict of interest: Renato Alberto Sinico Conflict of interest: Franco Schiavon Conflict of interest: Sara Monti Conflict of interest: Enrica Paola Bozzolo Conflict of interest: Franco Franceschini Conflict of interest: Marcello Govoni Conflict of interest: Claudio Lunardi Conflict of interest: Giuseppe Guida Conflict of interest: Giuseppe Lopalco Conflict of interest: Giuseppe Paolazzi Conflict of interest: Angelo Vacca Conflict of interest: Gina Gregorini Conflict of interest: Pietro Leccese Conflict of interest: Matteo Piga Conflict of interest: Fabrizio Conti Conflict of interest: Paolo Fraticelli Conflict of interest: Luca Quartuccio Conflict of interest: Federico Alberici Conflict of interest: Carlo Salvarani Conflict of interest: Silvano Bettio Conflict of interest: Simone Negrini Conflict of interest: Carlo Selmi Conflict of interest: Savino Sciascia Conflict of interest: Gabriella Moroni Conflict of interest: Loredana Colla Conflict of interest: Carlo Manno Conflict of interest: Maria Letizia Urban Conflict of interest: Alfredo Vannacci Conflict of interest: Maria Rosa Pozzi Conflict of interest: Paolo Fabbrini Conflict of interest: Stefano Polti Conflict of interest: Mara Felicetti Conflict of interest: Maria Rita Marchi Conflict of interest: Roberto Padoan Conflict of interest: Paolo Delvino Conflict of interest: Roberto Caporali Conflict of interest: Carlomaurizio Montecucco Conflict of interest: Lorenzo Dagna Conflict of interest: Adriana Cariddi Conflict of interest: Paola Toniati Conflict of interest: Dr. Tamanini reports other from Glaxo Smith Kline, outside the submitted work. Conflict of interest: Federica Furini Conflict of interest: Alessandra Bortoluzzi Conflict of interest: Elisa Tinazzi Conflict of interest: Lorenzo Delfino Conflict of interest: Iuliana Badiu Conflict of interest: Giovanni Rolla Conflict of interest: Vincenzo Venerito Conflict of interest: Florenzo Iannone Conflict of interest: Alvise Berti Conflict of interest: Roberto Bortolotti Conflict of interest: Vito Racanelli Conflict of interest: Guido Jeannin Conflict of interest: Angela Padula Conflict of interest: Alberto Cauli Conflict of interest: Roberta Priori Conflict of interest: Armando Gabrielli Conflict of interest: Milena Bond Conflict of interest: Martina Tedesco Conflict of interest: Giulia Pazzola Conflict of interest: Paola Tomietto Conflict of interest: Marco Pellecchio Conflict of interest: Chiara Marvisi Conflict of interest: Federica Maritati Conflict of interest: Alessandra Palmisano Conflict of interest: Christian Dejaco Conflict of interest: Johann Willeit Conflict of interest: Stefan Kiechl Conflict of interest: Iacopo Olivotto Conflict of interest: Peter Willeit Conflict of interest: Domenico Prisco Conflict of interest: Augusto Vaglio Conflict of interest: Giacomo Emmi

Published
2020

46. Erratum: Management of adult-onset Still's disease with interleukin-1 inhibitors: Evidence- And consensus-based statements by a panel of Italian experts (Arthritis Res Ther (2019) 21:275 DOI: 10.1186/s13075-019-2021-9)

Author

Colafrancesco, S., Manara, M., Bortoluzzi, A., Serban, T., Bianchi, G., Cantarini, L., Ciccia, F., Dagna, L., Govoni, M., Montecucco, C., Priori, R., Ravelli, A., Sfriso, P., Sinigaglia, L., Alivernini, S., Baldissera, E., Bartoloni, E., Berti, A., Bugatti, S., Camellino, D., Cammelli, D., Caporali, R., Caso, F., Cavallaro, E., Cavalli, G., Colaci, M., Costa, L., Di Scala, G., Emmi, G., Frassi, M., Gerli, R., Giacomelli, R., Gremese, E., Iannone, F., Lapadula, G., Leveghi, L., Lopalco, G., Manna, R., Marotto, D., Mathieu, A., Neri, R., Patisso, I., Piga, M., Punzi, L., Romano, M., Ruscitti, P., Salvarani, C., Scarpa, R., Scrivo, R., Talarico, R., Verrecchia, E., Viapiana, O., Vitale, A., Vitiello, G., Colafrancesco, S., Manara, M., Bortoluzzi, A., Serban, T., Bianchi, G., Cantarini, L., Ciccia, F., Dagna, L., Govoni, M., Montecucco, C., Priori, R., Ravelli, A., Sfriso, P., Sinigaglia, L., Alivernini, S., Baldissera, E., Bartoloni, E., Berti, A., Bugatti, S., Camellino, D., Cammelli, D., Caporali, R., Caso, F., Cavallaro, E., Cavalli, G., Colaci, M., Costa, L., Di Scala, G., Emmi, G., Frassi, M., Gerli, R., Giacomelli, R., Gremese, E., Iannone, F., Lapadula, G., Leveghi, L., Lopalco, G., Manna, R., Marotto, D., Mathieu, A., Neri, R., Patisso, I., Piga, M., Punzi, L., Romano, M., Ruscitti, P., Salvarani, C., Scarpa, R., Scrivo, R., Talarico, R., Verrecchia, E., Viapiana, O., Vitale, A., and Vitiello, G.

Abstract

Following publication of the original article [1], it was brought to our attention that the AOSD Consensus Group was incorrectly tagged and therefore not searchable. The publishers apologize for this error.

Published
2020

48. OP0223 DEVELOPMENT AND PRELIMINARY VALIDATION OF ULTRASONOGRAPHIC DISEASE ACTIVITY AND DAMAGE SCORES IN PSORIATIC ARTHRITIS PATIENTS: RESULTS FROM THE UPSTREAM (ULTRASOUND IN PSORIATIC ARTHRITIS TREATMENT) STUDY

Author

Zabotti, A., primary, Piga, M., additional, Zanetti, A., additional, Canzoni, M., additional, Boffini, N., additional, Picerno, V., additional, Zanframundo, G., additional, Silvagni, E., additional, Giovannini, I., additional, Raffeiner, B., additional, Scolieri, P., additional, Mancini, P., additional, Parisi, S., additional, Bortoluzzi, A., additional, Sakellariou, G., additional, De Lucia, O., additional, Tinazzi, I., additional, Figus, F., additional, Idolazzi, L., additional, Lorenzin, M., additional, Callegher, S. Z., additional, Cauli, A., additional, Carrara, G., additional, Scirè, C. A., additional, and Iagnocco, A., additional

Published
2021
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50. POS0751 COMORBIDITY AND LONG-TERM OUTCOME IN PATIENTS WITH CONGENITAL HEART BLOCK: PRELIMINARY DATA OF THE ITALIAN REGISTRY ON THE IMMUNE-MEDIATED CONGENITAL HEART BLOCK

Author

Fredi, M., primary, Rizzo, G., additional, Andreoli, L., additional, Bacco, B., additional, Bertero, T., additional, Bortoluzzi, A., additional, Ceccarelli, F., additional, Cimaz, R., additional, Conigliaro, P., additional, Corradi, F., additional, De Vita, S., additional, DI Poi, E., additional, Elefante, E., additional, Emmi, G., additional, Gerosa, M., additional, Govoni, M., additional, Hoxha, A., additional, Lojacono, A., additional, Marrani, E., additional, Marozio, L., additional, Mathieu, A., additional, Mosca, M., additional, Melissa, P., additional, Picchi, C., additional, Piga, M., additional, Priori, R., additional, Ramoni, V., additional, Ruffatti, A., additional, Simonini, G., additional, Tani, C., additional, Tonello, M., additional, Trespidi, L., additional, Urban, M. L., additional, Vezzoli, M., additional, Zatti, S., additional, Calza, S., additional, Brucato, A., additional, Franceschini, F., additional, and Tincani, A., additional

Published
2021
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