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1. 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

2. 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

3. 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

4. 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

5. 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

6. 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

7. 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

8. 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

9. 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

10. 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

11. 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

12. 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

13. 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

14. Risk of acute arterial and venous thromboembolic events in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

Author: 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, Emmi, G, Bettiol, Alessandra, Sinico, Renato Alberto, Schiavon, Franco, Monti, Sara, Bozzolo, Enrica Paola, Franceschini, Franco, Govoni, Marcello, Lunardi, Claudio, Guida, Giuseppe, Lopalco, Giuseppe, Paolazzi, Giuseppe, Vacca, Angelo, Gregorini, Gina, Leccese, Pietro, Piga, Matteo, Conti, Fabrizio, Fraticelli, Paolo, Quartuccio, Luca, Alberici, Federico, Salvarani, Carlo, Bettio, Silvano, Negrini, Simone, Selmi, Carlo, Sciascia, Savino, Moroni, Gabriella, Colla, Loredana, Manno, Carlo, Urban, Maria Letizia, Vannacci, Alfredo, Pozzi, Maria Rosa, Fabbrini, Paolo, Polti, Stefano, Felicetti, Mara, Marchi, Maria Rita, Padoan, Roberto, Delvino, Paolo, Caporali, Roberto, Montecucco, Carlomaurizio, Dagna, Lorenzo, Cariddi, Adriana, Toniati, Paola, Tamanini, Silvia, Furini, Federica, Bortoluzzi, Alessandra, Tinazzi, Elisa, Delfino, Lorenzo, Badiu, Iuliana, Rolla, Giovanni, Venerito, Vincenzo, Iannone, Florenzo, Berti, Alvise, Bortolotti, Roberto, Racanelli, Vito, Jeannin, Guido, Padula, Angela, Cauli, Alberto, Priori, Roberta, Gabrielli, Armando, Bond, Milena, Tedesco, Martina, Pazzola, Giulia, Tomietto, Paola, Pellecchio, Marco, Marvisi, Chiara, Maritati, Federica, Palmisano, Alessandra, Dejaco, Christian, Willeit, Johann, Kiechl, Stefan, Olivotto, Iacopo, Willeit, Peter, Prisco, Domenico, Vaglio, Augusto, Emmi, Giacomo, 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, Emmi, G, Bettiol, Alessandra, Sinico, Renato Alberto, Schiavon, Franco, Monti, Sara, Bozzolo, Enrica Paola, Franceschini, Franco, Govoni, Marcello, Lunardi, Claudio, Guida, Giuseppe, Lopalco, Giuseppe, Paolazzi, Giuseppe, Vacca, Angelo, Gregorini, Gina, Leccese, Pietro, Piga, Matteo, Conti, Fabrizio, Fraticelli, Paolo, Quartuccio, Luca, Alberici, Federico, Salvarani, Carlo, Bettio, Silvano, Negrini, Simone, Selmi, Carlo, Sciascia, Savino, Moroni, Gabriella, Colla, Loredana, Manno, Carlo, Urban, Maria Letizia, Vannacci, Alfredo, Pozzi, Maria Rosa, Fabbrini, Paolo, Polti, Stefano, Felicetti, Mara, Marchi, Maria Rita, Padoan, Roberto, Delvino, Paolo, Caporali, Roberto, Montecucco, Carlomaurizio, Dagna, Lorenzo, Cariddi, Adriana, Toniati, Paola, Tamanini, Silvia, Furini, Federica, Bortoluzzi, Alessandra, Tinazzi, Elisa, Delfino, Lorenzo, Badiu, Iuliana, Rolla, Giovanni, Venerito, Vincenzo, Iannone, Florenzo, Berti, Alvise, Bortolotti, Roberto, Racanelli, Vito, Jeannin, Guido, Padula, Angela, Cauli, Alberto, Priori, Roberta, Gabrielli, Armando, Bond, Milena, Tedesco, Martina, Pazzola, Giulia, Tomietto, Paola, Pellecchio, Marco, Marvisi, Chiara, Maritati, Federica, Palmisano, Alessandra, Dejaco, Christian, Willeit, Johann, Kiechl, Stefan, Olivotto, Iacopo, Willeit, Peter, Prisco, Domenico, Vaglio, Augusto, and Emmi, Giacomo
Published: 2021

15. Early Lupus Project: one-year follow-up of an Italian cohort of patients with systemic lupus erythematosus of recent onset

Author: Sebastiani, G, Prevete, I, Iuliano, A, Piga, M, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Tincani, A, Fredi, M, Conti, F, Spinelli, F, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scire, C, Mathieu, A, Sebastiani G. D., Prevete I., Iuliano A., Piga M., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Tincani A., Fredi M., Conti F., Spinelli F. R., Galeazzi M., Bellisai F., Zanetti A., Carrara G., Scire C. A., Mathieu A., Sebastiani, G, Prevete, I, Iuliano, A, Piga, M, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Tincani, A, Fredi, M, Conti, F, Spinelli, F, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scire, C, Mathieu, A, Sebastiani G. D., Prevete I., Iuliano A., Piga M., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Tincani A., Fredi M., Conti F., Spinelli F. R., Galeazzi M., Bellisai F., Zanetti A., Carrara G., Scire C. A., and Mathieu A.
Abstract: Objective: To describe the clinical and serological features of a prospectively followed cohort of early diagnosed systemic lupus erythematosus (SLE) patients during a one-year follow-up period. Methods: SLE patients with disease duration less than 12 months were consecutively enrolled in a multicentre, prospective study. At study entry and then every 6 months, a large panel of data was recorded. Results: Of 260 patients enrolled, 185 had at least 12 months of follow-up; of these, 84.3% were female, 92.4% were Caucasians. Mean diagnostic delay was about 20 months; higher values of European Consensus Lupus Activity Measurement (ECLAM) and of organs/systems involved were both associated with shorter diagnostic delay. Clinical and serological parameters improved after study entry. However, patients' quality of life deteriorated and cardiovascular risk factors significantly increased. About one-third of patients with active disease at study entry went into remission (ECLAM = 0). Negative predictors for remission were: oral ulcers, arthritis, low C4, anti-SSB (Ro) antibodies and therapy with mycophenolate. There was a widespread use of glucocorticoids both at baseline and during follow-up. Conclusion: Clinical symptoms and serological parameters improve during the first period after diagnosis. However, patients’ quality of life deteriorates. The widespread use of glucocorticoids is probably the reason for the early significant increase of some cardiovascular risk factors.
Published: 2018

16. Clinical and ultrasonographic predictors for achieving minimal disease activity in patients with psoriatic arthritis: The UPSTREAM (Ultrasound in PSoriatic arthritis TREAtMent) prospective observational study protocol

Author: Canzoni, M, Piga, M, Zabotti, A, Scire, C, Carrara, G, Olivieri, I, Iagnocco, A, Canzoni M., Piga M., Zabotti A., Scire C. A., Carrara G., Olivieri I., Iagnocco A., Canzoni, M, Piga, M, Zabotti, A, Scire, C, Carrara, G, Olivieri, I, Iagnocco, A, Canzoni M., Piga M., Zabotti A., Scire C. A., Carrara G., Olivieri I., and Iagnocco A.
Abstract: Introduction Psoriatic arthritis (PsA) occurs in 10%-15% of people with psoriasis and accounts for 10%-20% of early arthritis clinics referral. Only a few prognostic factors of therapeutic response in patients with PsA have been identified. In the last years, the role of imaging has grown up and the European League Against Rheumatism recognised that ultrasound (US) has higher sensitivity than clinical examination to detect inflammatory disease activity. The aims of the Ultrasound in PSoriatic arthritis TREAtMent (UPSTREAM) study are to integrate clinic and US in order to inform whether US has provide an added prognostic value in PsA. Methods and analysis UPSTREAM is an observational prospective cohort study enrolling patients with PsA having clinically active joint disease and starting a new course of therapy. The primary objective is to evaluate the additional value of US over clinical examination in detecting patients achieving minimal disease activity after 6 months. Data will be obtained at baseline and at standard clinical follow-up visits. Patient's clinical assessment will be performed according to the core set proposed by the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis-Outcome Measures in Rheumatology. Sonographic evaluations will be performed by expert sonographers in 42 joints, 36 tendons, 12 entheses and 2 bursae, according to a score that will be purposely developed for PsA by the US Study Group of the Italian Society for Rheumatology. The UPSTREAM study will identify clinical and US predictors of response to treatment in patients with PsA and active peripheral arthritis starting a new course of therapy. Ethics and dissemination Ethic approval for this study has been obtained from the institutional review board (IRB)/independent ethics committee (IEC) Comitato Etico Lazio 1 (Prot. N 198 02-02-2017) and then locally from the IRB/IEC of each participating centre. Results will be published in relevant scientific journals and be di
Published: 2018

17. Early Lupus Project: one-year follow-up of an Italian cohort of patients with systemic lupus erythematosus of recent onset

Author: Sebastiani, G, Prevete, I, Iuliano, A, Piga, M, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Tincani, A, Fredi, M, Conti, F, Spinelli, F, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scire, C, Mathieu, A, Sebastiani G. D., Prevete I., Iuliano A., Piga M., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Tincani A., Fredi M., Conti F., Spinelli F. R., Galeazzi M., Bellisai F., Zanetti A., Carrara G., Scire C. A., Mathieu A., Sebastiani, G, Prevete, I, Iuliano, A, Piga, M, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Tincani, A, Fredi, M, Conti, F, Spinelli, F, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scire, C, Mathieu, A, Sebastiani G. D., Prevete I., Iuliano A., Piga M., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Tincani A., Fredi M., Conti F., Spinelli F. R., Galeazzi M., Bellisai F., Zanetti A., Carrara G., Scire C. A., and Mathieu A.
Abstract: Objective: To describe the clinical and serological features of a prospectively followed cohort of early diagnosed systemic lupus erythematosus (SLE) patients during a one-year follow-up period. Methods: SLE patients with disease duration less than 12 months were consecutively enrolled in a multicentre, prospective study. At study entry and then every 6 months, a large panel of data was recorded. Results: Of 260 patients enrolled, 185 had at least 12 months of follow-up; of these, 84.3% were female, 92.4% were Caucasians. Mean diagnostic delay was about 20 months; higher values of European Consensus Lupus Activity Measurement (ECLAM) and of organs/systems involved were both associated with shorter diagnostic delay. Clinical and serological parameters improved after study entry. However, patients' quality of life deteriorated and cardiovascular risk factors significantly increased. About one-third of patients with active disease at study entry went into remission (ECLAM = 0). Negative predictors for remission were: oral ulcers, arthritis, low C4, anti-SSB (Ro) antibodies and therapy with mycophenolate. There was a widespread use of glucocorticoids both at baseline and during follow-up. Conclusion: Clinical symptoms and serological parameters improve during the first period after diagnosis. However, patients’ quality of life deteriorates. The widespread use of glucocorticoids is probably the reason for the early significant increase of some cardiovascular risk factors.
Published: 2018

18. Clinical and ultrasonographic predictors for achieving minimal disease activity in patients with psoriatic arthritis: The UPSTREAM (Ultrasound in PSoriatic arthritis TREAtMent) prospective observational study protocol

Author: Canzoni, M, Piga, M, Zabotti, A, Scire, C, Carrara, G, Olivieri, I, Iagnocco, A, Canzoni M., Piga M., Zabotti A., Scire C. A., Carrara G., Olivieri I., Iagnocco A., Canzoni, M, Piga, M, Zabotti, A, Scire, C, Carrara, G, Olivieri, I, Iagnocco, A, Canzoni M., Piga M., Zabotti A., Scire C. A., Carrara G., Olivieri I., and Iagnocco A.
Abstract: Introduction Psoriatic arthritis (PsA) occurs in 10%-15% of people with psoriasis and accounts for 10%-20% of early arthritis clinics referral. Only a few prognostic factors of therapeutic response in patients with PsA have been identified. In the last years, the role of imaging has grown up and the European League Against Rheumatism recognised that ultrasound (US) has higher sensitivity than clinical examination to detect inflammatory disease activity. The aims of the Ultrasound in PSoriatic arthritis TREAtMent (UPSTREAM) study are to integrate clinic and US in order to inform whether US has provide an added prognostic value in PsA. Methods and analysis UPSTREAM is an observational prospective cohort study enrolling patients with PsA having clinically active joint disease and starting a new course of therapy. The primary objective is to evaluate the additional value of US over clinical examination in detecting patients achieving minimal disease activity after 6 months. Data will be obtained at baseline and at standard clinical follow-up visits. Patient's clinical assessment will be performed according to the core set proposed by the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis-Outcome Measures in Rheumatology. Sonographic evaluations will be performed by expert sonographers in 42 joints, 36 tendons, 12 entheses and 2 bursae, according to a score that will be purposely developed for PsA by the US Study Group of the Italian Society for Rheumatology. The UPSTREAM study will identify clinical and US predictors of response to treatment in patients with PsA and active peripheral arthritis starting a new course of therapy. Ethics and dissemination Ethic approval for this study has been obtained from the institutional review board (IRB)/independent ethics committee (IEC) Comitato Etico Lazio 1 (Prot. N 198 02-02-2017) and then locally from the IRB/IEC of each participating centre. Results will be published in relevant scientific journals and be di
Published: 2018

19. Ultrasonography in psoriatic arthritis: Which sites should we scan?

Author: Zabotti, A, Piga, M, Canzoni, M, Sakellariou, G, Iagnocco, A, Scire, C, Adinolfi, A, Azzolin, I, Bandinelli, F, Batticciotto, A, Boffini, N, Bortoluzzi, A, Carrara, G, Cavatorta, F, Cagnotto, G, Caprioli, M, Colaci, M, De Lucia, O, Delle Sedie, A, Denaro, V, Di Matteo, A, Di Sabatino, V, Epis, O, Hoxha, A, Farina, I, Germano, G, Filippou, G, Filippucci, E, Focherini, M, Gabba, A, Idolazzi, L, La Paglia, G, Luccioli, F, Macchioni, P, Magnani, M, Massarotti, M, Mastaglio, C, Navarini, L, Parisi, S, Diamanti, A, Picerno, V, Piras, M, Porta, F, Possemato, N, Prevete, I, Raffeiner, B, Ramonda, R, Ravagnani, V, Rossi, D, Rossi, S, Scolieri, P, Santoboni, G, Scioscia, C, Terenzi, R, Tinazzi, I, Toscano, C, Venditti, C, Volpe, A, Vukatana, G, Zanframundo, G, Zabotti A., Piga M., Canzoni M., Sakellariou G., Iagnocco A., Scire C. A., Adinolfi A., Azzolin I., Bandinelli F., Batticciotto A., Boffini N., Bortoluzzi A., Carrara G., Cavatorta F. P., Cagnotto G., Caprioli M., Colaci M., De Lucia O., Delle Sedie A., Denaro V., Di Matteo A., Di Sabatino V., Epis O. M., Hoxha A., Farina I., Germano G., Filippou G., Filippucci E., Focherini M. C., Gabba A., Idolazzi L., La Paglia G. M. C., Luccioli F., Macchioni P., Magnani M., Massarotti M., Mastaglio C., Navarini L., Parisi S., Diamanti A. P., Picerno V., Piras M., Porta F., Possemato N., Prevete I., Raffeiner B., Ramonda R., Ravagnani V., Rossi D., Rossi S., Scolieri P., Santoboni G., Scioscia C., Terenzi R., Tinazzi I., Toscano C., Venditti C., Volpe A., Vukatana G., Zanframundo G., Zabotti, A, Piga, M, Canzoni, M, Sakellariou, G, Iagnocco, A, Scire, C, Adinolfi, A, Azzolin, I, Bandinelli, F, Batticciotto, A, Boffini, N, Bortoluzzi, A, Carrara, G, Cavatorta, F, Cagnotto, G, Caprioli, M, Colaci, M, De Lucia, O, Delle Sedie, A, Denaro, V, Di Matteo, A, Di Sabatino, V, Epis, O, Hoxha, A, Farina, I, Germano, G, Filippou, G, Filippucci, E, Focherini, M, Gabba, A, Idolazzi, L, La Paglia, G, Luccioli, F, Macchioni, P, Magnani, M, Massarotti, M, Mastaglio, C, Navarini, L, Parisi, S, Diamanti, A, Picerno, V, Piras, M, Porta, F, Possemato, N, Prevete, I, Raffeiner, B, Ramonda, R, Ravagnani, V, Rossi, D, Rossi, S, Scolieri, P, Santoboni, G, Scioscia, C, Terenzi, R, Tinazzi, I, Toscano, C, Venditti, C, Volpe, A, Vukatana, G, Zanframundo, G, Zabotti A., Piga M., Canzoni M., Sakellariou G., Iagnocco A., Scire C. A., Adinolfi A., Azzolin I., Bandinelli F., Batticciotto A., Boffini N., Bortoluzzi A., Carrara G., Cavatorta F. P., Cagnotto G., Caprioli M., Colaci M., De Lucia O., Delle Sedie A., Denaro V., Di Matteo A., Di Sabatino V., Epis O. M., Hoxha A., Farina I., Germano G., Filippou G., Filippucci E., Focherini M. C., Gabba A., Idolazzi L., La Paglia G. M. C., Luccioli F., Macchioni P., Magnani M., Massarotti M., Mastaglio C., Navarini L., Parisi S., Diamanti A. P., Picerno V., Piras M., Porta F., Possemato N., Prevete I., Raffeiner B., Ramonda R., Ravagnani V., Rossi D., Rossi S., Scolieri P., Santoboni G., Scioscia C., Terenzi R., Tinazzi I., Toscano C., Venditti C., Volpe A., Vukatana G., and Zanframundo G.
Published: 2018

20. The predictive role of ultrasound-detected tenosynovitis and joint synovitis for flare in patients with rheumatoid arthritis in stable remission. Results of an Italian multicentre study of the Italian Society for Rheumatology Group for Ultrasound: The STARTER study

Author: Filippou, G, Sakellariou, G, Scire, C, Carrara, G, Rumi, F, Bellis, E, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Scioscia, C, Venditti, C, Volpe, A, Iagnocco, A, Filippou G., Sakellariou G., Scire C. A., Carrara G., Rumi F., Bellis E., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., MacChioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Scioscia C., Venditti C., Volpe A., Iagnocco A., Filippou, G, Sakellariou, G, Scire, C, Carrara, G, Rumi, F, Bellis, E, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Scioscia, C, Venditti, C, Volpe, A, Iagnocco, A, Filippou G., Sakellariou G., Scire C. A., Carrara G., Rumi F., Bellis E., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., MacChioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Scioscia C., Venditti C., Volpe A., and Iagnocco A.
Abstract: Objective To define the role of ultrasound (US) for the assessment of patients with rheumatoid arthritis (RA) in clinical remission, including joint and tendon evaluation. Methods A multicentre longitudinal study has been promoted by the US Study Group of the Italian Society for Rheumatology. 25 Italian centres participated, enrolling consecutive patients with RA in clinical remission. All patients underwent complete clinical assessment (demographic data, disease characteristics, laboratory exams, clinical assessment of 28 joints and patient/physician-reported outcomes) and Power Doppler (PD) US evaluation of wrist, metacarpalphalangeal joints, proximal interphalangeal joints and synovial tendons of the hands and wrists at enrolment, 6 and 12 months. The association between clinical and US variables with flare, disability and radiographic progression was evaluated by univariable and adjusted logistic regression models. Results 361 patients were enrolled, the mean age was 56.20 (±13.31) years and 261 were women, with a mean disease duration of 9.75 (±8.07) years. In the 12 months follow-up, 98/326 (30.1%) patients presented a disease flare. The concurrent presence of PD positive tenosynovitis and joint synovitis predicted disease flare, with an OR (95% CI) of 2.75 (1.45 to 5.20) in crude analyses and 2.09 (1.06 to 4.13) in adjusted analyses. US variables did not predict the worsening of function or radiographic progression. US was able to predict flare at 12 months but not at 6 months. Conclusions PD positivity in tendons and joints is an independent risk factor of flare in patients with RA in clinical remission. Musculoskeletal ultrasound evaluation is a valuable tool to monitor and help decision making in patients with RA in clinical remission.
Published: 2018

21. Linee di indirizzo operative per la conciliazione delle attività sanitarie e giudiziarie nell'ambito dei trapianti di organi e tessuti a scopo terapeutico: 'PROCUREMENT e PROCURE', Centro Nazionale Trapianti

Author: Aurelio, MT, Aurelio, M, Balestra, L, Bettoni, C, Cardillo, M, Cerri, N, Cossolini, M, De Feo, T, Dettori, G, Ferri, F, La Torraca, R, Marchesi, M, Milanesi, F, Pea, E, Persutti, R, Piccolo, G, Piga, M, Scagliusi, M, Siciliano, T, Terenghi, P, Morgutti, M, Vescovi, S, Aurelio, MT, De Feo, TM, Milanesi, FC, Piga, MA, Aurelio, MT, Aurelio, M, Balestra, L, Bettoni, C, Cardillo, M, Cerri, N, Cossolini, M, De Feo, T, Dettori, G, Ferri, F, La Torraca, R, Marchesi, M, Milanesi, F, Pea, E, Persutti, R, Piccolo, G, Piga, M, Scagliusi, M, Siciliano, T, Terenghi, P, Morgutti, M, Vescovi, S, Aurelio, MT, De Feo, TM, Milanesi, FC, and Piga, MA
Published: 2020

22. Linee di indirizzo operative per la conciliazione delle attività sanitarie e giudiziarie nell'ambito dei trapianti di organi e tessuti a scopo terapeutico: 'PROCUREMENT e PROCURE', Centro Nazionale Trapianti

Author: Aurelio, MT, Aurelio, M, Balestra, L, Bettoni, C, Cardillo, M, Cerri, N, Cossolini, M, De Feo, T, Dettori, G, Ferri, F, La Torraca, R, Marchesi, M, Milanesi, F, Pea, E, Persutti, R, Piccolo, G, Piga, M, Scagliusi, M, Siciliano, T, Terenghi, P, Morgutti, M, Vescovi, S, Aurelio, MT, De Feo, TM, Milanesi, FC, Piga, MA, Aurelio, MT, Aurelio, M, Balestra, L, Bettoni, C, Cardillo, M, Cerri, N, Cossolini, M, De Feo, T, Dettori, G, Ferri, F, La Torraca, R, Marchesi, M, Milanesi, F, Pea, E, Persutti, R, Piccolo, G, Piga, M, Scagliusi, M, Siciliano, T, Terenghi, P, Morgutti, M, Vescovi, S, Aurelio, MT, De Feo, TM, Milanesi, FC, and Piga, MA
Published: 2020

23. Comparative study between two European inception cohorts of patients with early systemic lupus erythematosus

Author: Prevete, I, Espinosa, G, Bellisai, F, Bortoluzzi, A, Conti, F, Fredi, M, Fonseca-Aizpuru, E, de Viedma, V, Gonzalez-Garcia, A, Gonzalez-Leon, R, Iaccarino, L, Iannone, F, Marin-Ballve, A, Mitjavila, F, Pallares, L, Piga, M, Rios-Garces, R, Suarez, S, Tani, C, Zanetti, A, Ruiz-Irastorza, G, Sebastiani, G, Fonseca-Aizpuru, EM, de Viedma, VG, Sebastiani, GD, Prevete, I, Espinosa, G, Bellisai, F, Bortoluzzi, A, Conti, F, Fredi, M, Fonseca-Aizpuru, E, de Viedma, V, Gonzalez-Garcia, A, Gonzalez-Leon, R, Iaccarino, L, Iannone, F, Marin-Ballve, A, Mitjavila, F, Pallares, L, Piga, M, Rios-Garces, R, Suarez, S, Tani, C, Zanetti, A, Ruiz-Irastorza, G, Sebastiani, G, Fonseca-Aizpuru, EM, de Viedma, VG, and Sebastiani, GD
Abstract: Objective To compare the main characteristics of two inception cohorts (Italian [ITC] and Spanish [SPC]) cohorts of patients with systemic lupus erythematosus (SLE) at the time of diagnosis and at one year of follow-up. Methods Demographic, clinical and immunological characteristics, and treatments at SLE diagnosis and at 12 months of follow-up of ITC and SPC were compared. Results One hundred and sixty-four patients in the ITC and 231 patients in the SPC were compared. the patients from ITC were younger at SLE diagnosis (41.1±15.0 years vs. 46.4±15.6 years; p<0.001) and had a higher prevalence of arthritis (62.8% vs. 45.5%; p=0.001), serositis (25.6% vs. 16.0%; p=0.026), neurological involvement (7.9% vs. 1.7%; p=0.006), and immunological abnormalities (anti-dsDNA, anti-Sm, antiphospholipid antibodies) (93.9% vs. 77.8%; p<0.001). Conversely, photosensitivity (29.5% in ITC vs. 45.9% in SPC; p=0.001) and oral ulcers (12.4% vs. 30.3%; p<0.001) were more frequent at onset of SLE in the Spanish patients. At the first 12 months of follow-up, these differences were maintained. At SLE onset, more Italian patients received glucocorticoids (85.4% vs. 50.2%; p<0.001) and immunosuppressive agents. At 12 months of follow-up, more Spanish patients were treated with antimalarials (75.6% in ITC vs. 90.0% in SPC; p<0.001). Conversely, the use of glucocorticoids was lower in SPC (89.0% in ITC vs. 57.1% in SPC; p<0.001). Conclusion These cohorts presented different profiles in terms of pattern of organ/system involvement and disease treatment, possibly as a consequence of patient selection or different disease management approaches between Italy and Spain.
Published: 2020

24. Comparison of early vs. delayed anakinra treatment in patients with adult onset Still's disease and effect on clinical and laboratory outcomes

Author: Vitale, A, Cavalli, G, Ruscitti, P, Sota, J, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Cavallaro, E, Massaro, Mg, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Fornaro, M, Paladini, A, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Frediani, B, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Grosso, S, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L., Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Cavalli, G, Ruscitti, P, Sota, J, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Cavallaro, E, Massaro, Mg, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Fornaro, M, Paladini, A, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Frediani, B, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Grosso, S, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L., Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: Background: Aim of this study was to search for any difference in the outcome of patients with adult onset Still's disease (AOSD) treated with anakinra (ANK) in relation with the interval between disease onset and the start of anti-interleukin(IL)-1 treatment and according with the different lines of ANK treatment. Patients and Methods: One hundred and forty-one AOSD patients treated with ANK have been retrospectively assessed. Statistically significant differences (p < 0.05) were analyzed in the frequency of ANK effectiveness, primary or secondary inefficacy to ANK and rate of resolution of clinical and laboratory AOSD manifestations after 3, 6, and 12 months since ANK treatment according with different lines of treatment and different times between AOSD onset and start of ANK. Results: No significant differences were identified in the ANK effectiveness and frequency of primary or secondary inefficacy for patients starting ANK within 6 months (p = 0.19, p = 0.14, and p = 0.81, respectively) or 12 months (p = 0.37, p = 0.23, and p = 0.81, respectively) since AOSD onset compared with patients starting ANK thereafter; no significant differences were identified in ANK effectiveness and primary or secondary inefficacy according with different lines of ANK treatment (p = 0.06, p = 0.19, and p = 0.13, respectively). Patients starting ANK within 6 and 12 months since AOSD onset showed a significantly quicker decrease of erythrocyte sedimentation rate and C-reactive protein than observed among patients undergoing ANK treatment after 6 and 12 months. The number of swollen joints at the 3 month follow-up visit was significantly lower among patients undergoing ANK within 6 months since AOSD onset (p = 0.01), while no significance was identified at the 6 and 12 month assessments (p = 0.23 and p = 0.45, respectively). At the 3 and 6 month visits, the number of swollen joints was significantly higher among patients previously treated with conventional and biological disease mo
Published: 2020

25. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author: Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel
Abstract: Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.
Published: 2019

26. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author: Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel
Abstract: Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.
Published: 2019

27. Long-term retention rate of anakinra in adult onset Still’s disease and predictive factors for treatment response.

Author: Vitale, A, Cavalli, G, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Sota, J, Cavallaro, E, Massaro, Maria Grazia, Ruscitti, P, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Ferraccioli, Gianfranco, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Lapadula, G, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L, Ferraccioli G (ORCID:0000-0001-6246-2428), Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Cavalli, G, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Sota, J, Cavallaro, E, Massaro, Maria Grazia, Ruscitti, P, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Ferraccioli, Gianfranco, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Lapadula, G, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L, Ferraccioli G (ORCID:0000-0001-6246-2428), Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: We have evaluated long-term retention rate of anakinra in adult onset Still disease and predictive factors for treatment response. The risk of losing anakinra efficacy increases along with the number of swollen joints at the start of therapy, while the typical skin rash is a negative predictor of anakinra withdrawal.
Published: 2019

28. Long-term retention rate of anakinra in adult onset Still's disease and predictive factors for treatment response

Author: Vitale, A, Cavalli, G, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Sota, J, Cavallaro, E, Massaro, Mg, Ruscitti, P, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Ferraccioli, Gianfranco, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Lapadula, G, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L, Ferraccioli G (ORCID:0000-0001-6246-2428), Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Cavalli, G, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Sota, J, Cavallaro, E, Massaro, Mg, Ruscitti, P, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Ferraccioli, Gianfranco, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Lapadula, G, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L, Ferraccioli G (ORCID:0000-0001-6246-2428), Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: Background: Anakinra (ANA) is an effective treatment choice in patients with adult onset Still’s disease (AOSD); however, withdrawal owing to long-term remission as well as lack or loss of efficacy and adverse events may variably impact treatment survival. Objectives: Aims of this study were: i) to assess the drug retention rate (DRR) of ANA during a long-term follow-up looking for any difference related to the line of biologic treatment, the concomitant use of conventional disease modifying anti-rheumatic drugs (cDMARDs) and the different type of AOSD (systemic versus chronic articular); ii) to identify predictive factors of lack of efficacy, loss of efficacy and ANA withdrawal owing to long-term remission. Methods: AOSD patients classified according with Yamaguchi criteria and treated with ANA were retrospectively enrolled in 18 Italian tertiary Centers. Demographic, laboratory, clinical and therapeutic data related to the start of ANA (baseline), the 3-month assessment and the last follow-up visit while on ANA treatment were retrospectively collected and statistically analyzed. Results: One hundred and forty-one AOSD patients (48 males, 93 females) treated with ANA for a mean period of 35.96±36.05 months were enrolled. The overall DRR of ANA was 44.6% and 30.5% at the 60- and 120-month assessments, respectively, with no significant differences between: i) biologic naïve patients and those previously treated with other biologics (log-rank p=0.97); ii) monotherapy and concomitant use of cDMARDs (log-rank p=0.45); iii) systemic and chronic articular types of AOSD (log-rank p=0.67). No variables collected at baseline could predict primary inefficacy, while the number of swollen joints at baseline was significantly associated with secondary inefficacy (p=0.01, OR=1.194, C.I. 1.043-1.367). The typical AOSD skin rash was negatively related with ANA withdrawal owing to long-term remission (p=0.03, OR=0.224, C.I. 0.058-0.863). Conclusions: Long-term DRR of ANA has been f
Published: 2019

29. Ultrasound-detected tenosynovitis independently associates with patient-reported flare in patients with rheumatoid arthritis in clinical remission: Results from the observational study STARTER of the Italian Society for Rheumatology

Author: Bellis, E, Scire, C, Carrara, G, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Filippou, G, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Sakellariou, G, Scioscia, C, Venditti, C, Volpe, A, Matucci-Cerinic, M, Iagnocco, A, Bellis E., Scire C. A., Carrara G., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Filippou G., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., Macchioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Sakellariou G., Scioscia C., Venditti C., Volpe A., Matucci-Cerinic M., Iagnocco A., Bellis, E, Scire, C, Carrara, G, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Filippou, G, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Sakellariou, G, Scioscia, C, Venditti, C, Volpe, A, Matucci-Cerinic, M, Iagnocco, A, Bellis E., Scire C. A., Carrara G., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Filippou G., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., Macchioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Sakellariou G., Scioscia C., Venditti C., Volpe A., Matucci-Cerinic M., and Iagnocco A.
Abstract: Objectives. This study aimed to estimate the prevalence of US-detected tenosynovitis in RA patients in clinical remission and to explore its clinical correlates.Methods. A total of 427 RA patients in clinical remission were consecutively enrolled from 25 Italian rheumatology centres. Tenosynovitis and synovitis were scored by US grey scale (GS) and power Doppler (PD) semi-quantitative scoring systems at wrist and hand joints. Complete clinical assessment was performed by rheumatologists blinded to the US results. A flare questionnaire was used to assess unstable remission (primary outcome), HAQ for functional disability and radiographic erosions for damage (secondary outcomes). Cross-sectional relationships between the presence of each US finding and outcome variables are presented as odds ratios (ORs) and 95% CIs, both crude and adjusted for pre-specified confounders.Results. The prevalence of tenosynovitis in clinical remission was 52.5% (95% CI 0.48, 0.57) for GS and 22.7% (95% CI 0.19, 0.27) for PD, while the prevalence of synovitis was 71.6% (95% CI 0.67, 0.76) for GS and 42% (95% CI 0.37, 0.47) for PD. Among clinical correlates, PD tenosynovitis associated with lower remission duration and morning stiffness while PD synovitis did not. Only PD tenosynovitis showed a significant association with the flare questionnaire [OR 1.95 (95% CI 1.17, 3.26)]. No cross-sectional associations were found with the HAQ. The presence of radiographic erosions associated with GS and PD synovitis but not with tenosynovitis.Conclusions. US-detected tenosynovitis is a frequent finding in RA patients in clinical remission and associates with unstable remission.
Published: 2016

30. Ultrasound-detected tenosynovitis independently associates with patient-reported flare in patients with rheumatoid arthritis in clinical remission: Results from the observational study STARTER of the Italian Society for Rheumatology

Author: Bellis, E, Scire, C, Carrara, G, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Filippou, G, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Sakellariou, G, Scioscia, C, Venditti, C, Volpe, A, Matucci-Cerinic, M, Iagnocco, A, Bellis E., Scire C. A., Carrara G., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Filippou G., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., Macchioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Sakellariou G., Scioscia C., Venditti C., Volpe A., Matucci-Cerinic M., Iagnocco A., Bellis, E, Scire, C, Carrara, G, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Filippou, G, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Sakellariou, G, Scioscia, C, Venditti, C, Volpe, A, Matucci-Cerinic, M, Iagnocco, A, Bellis E., Scire C. A., Carrara G., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Filippou G., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., Macchioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Sakellariou G., Scioscia C., Venditti C., Volpe A., Matucci-Cerinic M., and Iagnocco A.
Abstract: Objectives. This study aimed to estimate the prevalence of US-detected tenosynovitis in RA patients in clinical remission and to explore its clinical correlates.Methods. A total of 427 RA patients in clinical remission were consecutively enrolled from 25 Italian rheumatology centres. Tenosynovitis and synovitis were scored by US grey scale (GS) and power Doppler (PD) semi-quantitative scoring systems at wrist and hand joints. Complete clinical assessment was performed by rheumatologists blinded to the US results. A flare questionnaire was used to assess unstable remission (primary outcome), HAQ for functional disability and radiographic erosions for damage (secondary outcomes). Cross-sectional relationships between the presence of each US finding and outcome variables are presented as odds ratios (ORs) and 95% CIs, both crude and adjusted for pre-specified confounders.Results. The prevalence of tenosynovitis in clinical remission was 52.5% (95% CI 0.48, 0.57) for GS and 22.7% (95% CI 0.19, 0.27) for PD, while the prevalence of synovitis was 71.6% (95% CI 0.67, 0.76) for GS and 42% (95% CI 0.37, 0.47) for PD. Among clinical correlates, PD tenosynovitis associated with lower remission duration and morning stiffness while PD synovitis did not. Only PD tenosynovitis showed a significant association with the flare questionnaire [OR 1.95 (95% CI 1.17, 3.26)]. No cross-sectional associations were found with the HAQ. The presence of radiographic erosions associated with GS and PD synovitis but not with tenosynovitis.Conclusions. US-detected tenosynovitis is a frequent finding in RA patients in clinical remission and associates with unstable remission.
Published: 2016

31. La donazione d'organi 'samaritana': problemi etico-deontologici e giuridici

Author: Badengo, S, Marchesi, M, Zoja, R, Piga, M, Piga, M A, Badengo, S, Marchesi, M, Zoja, R, Piga, M, and Piga, M A
Abstract: The so called “Samaritan donation” is meant as non-directed altruistic donation, where the donor does not choose the recipient. It’s an uncommon practice in Italy, as in many over other countries. An critical hesitation to develop these type of transplantation programs is the fear of incentivize the recruitment of disadvantaged people to provide organs for paying recipients; another are doubts related to motivations (possible psychiatric or psychological illness). All Sa- maritan donors should be always carefully assessed by an independent Authority, to make sure the donor knows and understands what is involved, is not under any pressure to donate and is giving consent freely and voluntarily. Samaritan donation should not be intended as an alterna- tive to after-death-donation, but its exceptionality should contribute toward increasing aware- ness of people and the amount of after-death-donations.
Published: 2013

32. La professione del sociologo nel panorama internazionale

Author: Dallago, B, Bruschi, A, Rampazi, M, Negrelli, S, Scaglia, A, Barone, C, Ruggera, L, Di Nicola, P, Gubert, R, Pollini, G, Piga, M, Pretto, A, Artieri Boccia, G, Borrelli, D, Gavrila, M, Galesi, D, Carboni, A, Gianera, A, Perino, A, Manna, E, Savonardo, L, Gherardi, S, Fazzi, L, Fischietti, G, NEGRELLI, SERAFINO, Dallago, B, Bruschi, A, Rampazi, M, Negrelli, S, Scaglia, A, Barone, C, Ruggera, L, Di Nicola, P, Gubert, R, Pollini, G, Piga, M, Pretto, A, Artieri Boccia, G, Borrelli, D, Gavrila, M, Galesi, D, Carboni, A, Gianera, A, Perino, A, Manna, E, Savonardo, L, Gherardi, S, Fazzi, L, Fischietti, G, and NEGRELLI, SERAFINO
Published: 2015

33. Development and validation of a new algorithm for attribution of neuropsychiatric events in systemic lupus erythematosus

Author: Bortoluzzi, A, Scirè, Ca, Bombardieri, S, Caniatti, L, Conti, F, De Vita, S, Doria, A, Ferraccioli, Gianfranco, Gremese, Elisa, Mansutti, E, Mathieu, A, Mosca, M, Padovan, M, Piga, M, Tincani, A, Tola, Mr, Tomietto, P, Valesini, G, Zen, M, Govoni, M., Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Gremese, Elisa (ORCID:0000-0002-2248-1058), Bortoluzzi, A, Scirè, Ca, Bombardieri, S, Caniatti, L, Conti, F, De Vita, S, Doria, A, Ferraccioli, Gianfranco, Gremese, Elisa, Mansutti, E, Mathieu, A, Mosca, M, Padovan, M, Piga, M, Tincani, A, Tola, Mr, Tomietto, P, Valesini, G, Zen, M, Govoni, M., Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), and Gremese, Elisa (ORCID:0000-0002-2248-1058)
Abstract: The aim of this study was to develop and validate an algorithm to assist the attribution of neuropsychiatric (NP) events to underlying disease in SLE patients.
Published: 2015

34. Development and validation of a new algorithm for attribution of neuropsychiatric events in systemic lupus erythematosus

Author: Bortoluzzi, A, Scirè, Ca, Bombardieri, S, Caniatti, L, Conti, F, De Vita, S, Doria, A, Ferraccioli, Gianfranco, Gremese, Elisa, Mansutti, E, Mathieu, A, Mosca, M, Padovan, M, Piga, M, Tincani, A, Tola, Mr, Tomietto, P, Valesini, G, Zen, M, Govoni, M., Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Gremese, Elisa (ORCID:0000-0002-2248-1058), Bortoluzzi, A, Scirè, Ca, Bombardieri, S, Caniatti, L, Conti, F, De Vita, S, Doria, A, Ferraccioli, Gianfranco, Gremese, Elisa, Mansutti, E, Mathieu, A, Mosca, M, Padovan, M, Piga, M, Tincani, A, Tola, Mr, Tomietto, P, Valesini, G, Zen, M, Govoni, M., Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), and Gremese, Elisa (ORCID:0000-0002-2248-1058)
Abstract: The aim of this study was to develop and validate an algorithm to assist the attribution of neuropsychiatric (NP) events to underlying disease in SLE patients.
Published: 2014

35. Factors and comorbidities associated with first neuropsychiatric event in systemic lupus erythematosus: does a risk profile exist? A large multicentre retrospective cross-sectional study on 959 Italian patients

Author: Govoni, M, Bombardieri, S, Bortoluzzi, A, Caniatti, L, Casu, C, Conti, F, De Vita, S, Doria, A, Farina, I, Ferraccioli, Gianfranco, Gremese, Elisa, Mansutti, E, Mosca, M, Padovan, M, Piga, M, Tincani, A, Tola, Mr, Tomietto, P, Taglietti, M, Trotta, F, Valesini, G, Zen, M, Mathieu, A., Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Gremese, Elisa (ORCID:0000-0002-2248-1058), Govoni, M, Bombardieri, S, Bortoluzzi, A, Caniatti, L, Casu, C, Conti, F, De Vita, S, Doria, A, Farina, I, Ferraccioli, Gianfranco, Gremese, Elisa, Mansutti, E, Mosca, M, Padovan, M, Piga, M, Tincani, A, Tola, Mr, Tomietto, P, Taglietti, M, Trotta, F, Valesini, G, Zen, M, Mathieu, A., Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), and Gremese, Elisa (ORCID:0000-0002-2248-1058)
Abstract: To analyse risk factors and comorbidities potentially associated with CNS involvement in a large cohort of Italian patients affected by SLE.
Published: 2012

36. Structure-Property Interplay of Proton Conducting Membranes Based on Poly-2,2-(2,6-pyridine)-5,5-bibenzimidazole, SiO2-Im and H3PO4 for High Temperature Fuel Cells

Author: Di Noto, V, Piga, M, Giffin, G, Quartarone, E, Righetti, P, Mustarelli, P, Magistris, A, Giffin, GA, Di Noto, V, Piga, M, Giffin, G, Quartarone, E, Righetti, P, Mustarelli, P, Magistris, A, and Giffin, GA
Abstract: Polybenzimidazoles (PBIs) are among the polymers of choice to prepare membranes for high temperature polymer fuel cells. Poly-2,2′(2,6-pyridine) -5,5′-bibenzimidazole (PBI5N), doped with H3PO4, and acid-doped PBI5N containing 10 wt% of imidazole-functionalized silica membranes were studied with thermogravimetric analysis, differential scanning calorimetry, dynamic-mechanical analysis, infrared spectroscopy, and broadband electric spectroscopy to examine the structure-property relationships. Key results show that: (1) doped PBI5N membranes show thermal decomposition starting at 120 °C, while pristine PBI5N is stable up to 300 °C; (2) the presence of filler increases the acid uptake and decreases the crystallinity of PBI5N; (3) the addition of phosphoric acid reduces the mechanical properties of the membrane, while the addition of filler has the opposite effect; (4) acid-doped membranes have conductivity values on the order of 10 -2-10-3 S cm-1; and (5) membranes exhibit a Vogel-Tamman-Fulcher (VTF) type proton conduction mechanism, where proton hopping is coupled with the segmental motion of the polymer chain. Infrared spectroscopy combined with DFT quantum mechanical calculations was used to assign the experimental spectrum of PBI5N
Published: 2011

37. Tecniche di procreazione assistita nelle coppie HIV-discordanti

Author: Morini, O, Piga, M, Schillaci, D, MORINI, OSVALDO, Piga, MA, SCHILLACI, DANIELA ROBERTA, Morini, O, Piga, M, Schillaci, D, MORINI, OSVALDO, Piga, MA, and SCHILLACI, DANIELA ROBERTA
Published: 1996

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