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1. Exposure to air pollution as an environmental determinant of how SjÃ¶gren's disease is expressed at diagnosis

Author: Brito-Zeron, P., Flores-Chavez, A., Ng, W. -F, Horvath, I. Fanny, Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Ozkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Abacar, K., Bartoloni, E., Rischmueller, M., Reis-de Oliveira, F., Trevisani, V. Fernandes Moca, Jurcut, C., Fugmann, Cecilia, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Szanto, A., Farris, A. D., Villa, M., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Inanc, N., Ramos-Casals, M., Brito-Zeron, P., Flores-Chavez, A., Ng, W. -F, Horvath, I. Fanny, Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Ozkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Abacar, K., Bartoloni, E., Rischmueller, M., Reis-de Oliveira, F., Trevisani, V. Fernandes Moca, Jurcut, C., Fugmann, Cecilia, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Szanto, A., Farris, A. D., Villa, M., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Inanc, N., and Ramos-Casals, M.
Abstract: ObjectiveTo analyse how the potential exposure to air pollutants can influence the key components at the time of diagnosis of Sjogren's phenotype (epidemiological profile, sicca symptoms, and systemic disease). MethodsFor the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Air pollution indexes per country were defined according to the OECD (1990-2021), including emission data of nitrogen and sulphur oxides (NO/SO), particulate matter (PM2.5 and 1.0), carbon monoxide (CO) and volatile organic compounds (VOC) calculated per unit of GDP, Kg per 1000 USD.ResultsThe results of the chi-square tests of independence for each air pollutant with the frequency of dry eyes at diagnosis showed that, except for one, all variables exhibited p-values <0.0001. The most pronounced disparities emerged in the dry eye prevalence among individuals inhabiting countries with the highest NO/SO exposure, a surge of 4.61 percentage points compared to other countries, followed by CO (3.59 points), non-methane (3.32 points), PM2.5 (3.30 points), and PM1.0 (1.60 points) exposures. Concerning dry mouth, individuals residing in countries with worse NO/SO exposures exhibited a heightened frequency of dry mouth by 2.05 percentage points (p<0.0001), followed by non-methane exposure (1.21 percentage points increase, p=0.007). Individuals inhabiting countries with the worst NO/SO, CO, and PM2.5 pollution levels had a higher mean global ESSDAI score than those in lower-risk nations (all p-values <0.0001). When systemic disease was stratified according to DAS into low, moderate, and high systemic activity levels, a heightened proportion of individuals manifesting moderate/severe systemic activity was observed in countries with worse exposures to NO/SO, CO, and PM2.5 pollutant levels. ConclusionFor the first time, we suggest that pollution levels could
Published: 2023
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2. Influence of exposure to climate-related hazards in the phenotypic expression of primary SjÃ¶gren's syndrome

Author: Flores-Chavez, A., Brito-Zeron, P., Ng, W. -f., Szanto, A., Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Oezkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Inanc, N., Bartoloni, E., Rischmueller, M., Oliveira, F. Reis-de, Trevisani, V. Fernandes Moca, Jurcut, C., Nordmark, Gunnel, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Horvath, I. Fanny, Farris, A. D., Simoncelli, E., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Abacar, K., Ramos-Casals, M., Flores-Chavez, A., Brito-Zeron, P., Ng, W. -f., Szanto, A., Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Oezkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Inanc, N., Bartoloni, E., Rischmueller, M., Oliveira, F. Reis-de, Trevisani, V. Fernandes Moca, Jurcut, C., Nordmark, Gunnel, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Horvath, I. Fanny, Farris, A. D., Simoncelli, E., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Abacar, K., and Ramos-Casals, M.
Abstract: Objective To analyse how the key components at the time of diagnosis of the Sjogren's phenotype (epidemiological profile, sicca symptoms, and systemic disease) can be influenced by the potential exposure to climate-related natural hazards. Methods For the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Climate-related hazards per country were defined according to the OECD and included seven climate-related hazard types: extreme temperature, extreme precipitation, drought, wildfire, wind threats, river flooding, and coastal flooding. Climatic variables were defined as dichotomous variables according to whether each country is ranked among the ten countries with the most significant exposure. Results After applying data-cleaning techniques and excluding people from countries not included in the OECD climate rankings, the database study analysed 16,042 patients from 23 countries. The disease was diagnosed between 1 and 3 years earlier in people living in countries included among the top 10 worst exposed to extreme precipitation, wildfire, wind threats, river flooding, and coastal flooding. A lower frequency of dry eyes was observed in people living in countries exposed to wind threats, river flooding, and coastal flooding, with a level of statistical association being classified as strong (p<0.0001 for the three variables). The frequency of dry mouth was significantly lower in people living in countries exposed to river flooding (p<0.0001) and coastal flooding (p<0.0001). People living in countries included in the worse climate scenarios for extreme temperature (p<0.0001) and river flooding (p<0.0001) showed a higher mean ESSDAI score in comparison with people living in no-risk countries. In contrast, those living in countries exposed to worse climate scenarios for wind threats (p<0.0001) and coastal flooding
Published: 2023
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3. The administration of methotrexate in patients with Still’s disease, “real-life” findings from AIDA Network Still Disease Registry

Author: 362., Ruscitti P, Sota, J, Vitale, A, Lopalco, G, Iannone, F, Morrone, M, Giardini, Ham, D'Agostin, Ma, Antonelli, Ipb, Almaghlouth, I, Asfina, Kn, Khalil, N, Sfikakis, Pp, Laskari, K, Tektonidou, M, Ciccia, F, Iacono, D, Riccio, F, Ragab, G, Hussein, Ma, Govoni, M, Ruffilli, F, Direskeneli, H, Alibaz-Oner, F, Giacomelli, R, Navarini, L, Bartoloni, E, Riccucci, I, Martín-Nares, E, Torres-Ruiz, J, Cipriani, P, Di Cola, I, Hernández-Rodríguez, J, Gómez-Caverzaschi, V, Dagna, L, Tomelleri, A, Makowska, J, Brzezinska, O, Iagnocco, A, Bellis, E, Caggiano, V, Gaggiano, C, Tarsia, M, Mormile, I, Emmi, G, Sfriso, P, Monti, S, Erten, Ş, Del Giudice, E, Lubrano, R, Conti, G, Olivieri, An, Lo Gullo, A, Tharwat, S, Karamanakos, A, Gidaro, A, Maggio, Mc, La Torre, F, Cardinale, F, Ogunjimi, B, Maier, A, Sebastiani, Gd, Opris-Belinski, D, Frassi, M, Viapiana, O, Bizzi, E, Carubbi, F, Fotis, L, Tufan, A, Kardas, Rc, Więsik-Szewczyk, E, Jahnz-Różyk, K, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), 362., Ruscitti P, Sota, J, Vitale, A, Lopalco, G, Iannone, F, Morrone, M, Giardini, Ham, D'Agostin, Ma, Antonelli, Ipb, Almaghlouth, I, Asfina, Kn, Khalil, N, Sfikakis, Pp, Laskari, K, Tektonidou, M, Ciccia, F, Iacono, D, Riccio, F, Ragab, G, Hussein, Ma, Govoni, M, Ruffilli, F, Direskeneli, H, Alibaz-Oner, F, Giacomelli, R, Navarini, L, Bartoloni, E, Riccucci, I, Martín-Nares, E, Torres-Ruiz, J, Cipriani, P, Di Cola, I, Hernández-Rodríguez, J, Gómez-Caverzaschi, V, Dagna, L, Tomelleri, A, Makowska, J, Brzezinska, O, Iagnocco, A, Bellis, E, Caggiano, V, Gaggiano, C, Tarsia, M, Mormile, I, Emmi, G, Sfriso, P, Monti, S, Erten, Ş, Del Giudice, E, Lubrano, R, Conti, G, Olivieri, An, Lo Gullo, A, Tharwat, S, Karamanakos, A, Gidaro, A, Maggio, Mc, La Torre, F, Cardinale, F, Ogunjimi, B, Maier, A, Sebastiani, Gd, Opris-Belinski, D, Frassi, M, Viapiana, O, Bizzi, E, Carubbi, F, Fotis, L, Tufan, A, Kardas, Rc, Więsik-Szewczyk, E, Jahnz-Różyk, K, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: Objectives: To describe clinical characteristics of patients with Still's disease treated with methotrexate (MTX) and to assess drug effectiveness evaluating change in disease activity, reduction of inflammatory markers, and glucocorticoid (GC)-sparing effect. Methods: Patients with Still's disease treated with MTX were assessed among those included in AIDA Network Still Disease Registry. Results: In this registry, 171 patients with Still's disease were treated with MTX (males 43.3%, age 37.1 ± 16.0 years). They were mainly characterised by joint features and fever without a prominent multiorgan involvement. MTX was administered with GCs in 68.4% of patients, with other conventional synthetic DMARDs in 6.4%, and with biologic DMARDs in 25.1%. A significant reduction of the modified systemic score was observed, and 38.6% patients were codified as being in clinical remission at the end of follow-up. The concomitant administration of a biologic DMARD resulted a predictor of the clinical remission. Furthermore, a reduction of inflammatory markers and ferritin levels was observed following the administration of MTX. Additionally, a marked reduction of the dosage of concomitant GCs was identified, while 36.7% discontinued such drugs. Male gender appeared as a predictor of GC discontinuation. MTX was discontinued in 12.3% of patients because of adverse effects, and in 12.3% for lack of efficacy. Conclusions: Clinical characteristics of patients with Still's disease treated with MTX were described, mainly joint features and fever without a prominent multiorgan involvement. The clinical usefulness of MTX was reported in reducing the disease activity, decreasing the inflammatory markers, and as GC-sparing agent.
Published: 2023

4. 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

5. Impact of COVID-19 and vaccination campaign on 1,755 systemic sclerosis patients during first three years of pandemic. Possible risks for individuals with impaired immunoreactivity to vaccine, ongoing immunomodulating treatments, and disease-related lung involvement during the next pandemic phase

Author: Ferri, C., Raimondo, V., Giuggioli, D., Gragnani, L., Lorini, S., Dagna, L., Bosello, Silvia Laura, Foti, R., Riccieri, V., Guiducci, S., Cuomo, G., Tavoni, A., De Angelis, R., Cacciapaglia, F., Zanatta, E., Cozzi, F., Murdaca, G., Cavazzana, I., Romeo, N., Codullo, V., Pellegrini, R., Varcasia, G., De Santis, M., Selmi, C., Abignano, G., Caminiti, M., L'Andolina, M., Olivo, D., Lubrano, E., Spinella, A., Lumetti, F., De Luca, G., Ruscitti, P., Urraro, T., Visentini, M., Bellando-Randone, S., Visalli, E., Testa, D., Sciascia, G., Masini, F., Pellegrino, G., Saccon, F., Balestri, E., Elia, G., Ferrari, S. M., Tonutti, A., Dall'Ara, F., Pagano Mariano, G., Pettiti, G., Zanframundo, G., Brittelli, R., Aiello, V., Dal Bosco, Y., Di Cola, I., Scorpiniti, D., Fusaro, E., Ferrari, T., Gigliotti, P., Campochiaro, C., Francioso, F., Iandoli, C., Caira, V., Zignego, A. L., D'Angelo, S., Franceschini, F., Matucci-Cerinic, M., Giacomelli, R., Doria, A., Santini, Stefano Angelo, Fallahi, P., Iannone, F., Antonelli, A., Bosello S. L. (ORCID:0000-0002-4837-447X), Santini S. A. (ORCID:0000-0003-1956-5899), Ferri, C., Raimondo, V., Giuggioli, D., Gragnani, L., Lorini, S., Dagna, L., Bosello, Silvia Laura, Foti, R., Riccieri, V., Guiducci, S., Cuomo, G., Tavoni, A., De Angelis, R., Cacciapaglia, F., Zanatta, E., Cozzi, F., Murdaca, G., Cavazzana, I., Romeo, N., Codullo, V., Pellegrini, R., Varcasia, G., De Santis, M., Selmi, C., Abignano, G., Caminiti, M., L'Andolina, M., Olivo, D., Lubrano, E., Spinella, A., Lumetti, F., De Luca, G., Ruscitti, P., Urraro, T., Visentini, M., Bellando-Randone, S., Visalli, E., Testa, D., Sciascia, G., Masini, F., Pellegrino, G., Saccon, F., Balestri, E., Elia, G., Ferrari, S. M., Tonutti, A., Dall'Ara, F., Pagano Mariano, G., Pettiti, G., Zanframundo, G., Brittelli, R., Aiello, V., Dal Bosco, Y., Di Cola, I., Scorpiniti, D., Fusaro, E., Ferrari, T., Gigliotti, P., Campochiaro, C., Francioso, F., Iandoli, C., Caira, V., Zignego, A. L., D'Angelo, S., Franceschini, F., Matucci-Cerinic, M., Giacomelli, R., Doria, A., Santini, Stefano Angelo, Fallahi, P., Iannone, F., Antonelli, A., Bosello S. L. (ORCID:0000-0002-4837-447X), and Santini S. A. (ORCID:0000-0003-1956-5899)
Abstract: Introduction: The impact of COVID-19 pandemic represents a serious challenge for ‘frail’ patients' populations with inflammatory autoimmune systemic diseases such as systemic sclerosis (SSc). We investigated the prevalence and severity of COVID-19, as well the effects of COVID-19 vaccination campaign in a large series of SSc patients followed for the entire period (first 38 months) of pandemic. Patients and method: This prospective survey study included 1755 unselected SSc patients (186 M, 1,569F; mean age 58.7 ± 13.4SD years, mean disease duration 8.8 ± 7.3SD years) recruited in part by telephone survey at 37 referral centers from February 2020 to April 2023. The following parameters were carefully evaluated: i. demographic, clinical, serological, and therapeutical features; ii. prevalence and severity of COVID-19; and iii. safety, immunogenicity, and efficacy of COVID-19 vaccines. Results: The prevalence of COVID-19 recorded during the whole pandemic was significantly higher compared to Italian general population (47.3 % vs 43.3 %, p < 0.000), as well the COVID-19-related mortality (1.91 % vs 0.72 %, p < 0.001). As regards the putative prognostic factors of worse outcome, COVID-19 positive patients with SSc-related interstitial lung involvement showed significantly higher percentage of COVID-19-related hospitalization compared to those without (5.85 % vs 1.73 %; p < 0.0001), as well as of mortality rate (2.01 % vs 0.4 %; p = 0.002). Over half of patients (56.3 %) received the first two plus one booster dose of vaccine; while a fourth dose was administered to 35.6 %, and only few of them (1.99 %) had five or more doses of vaccine. Of note, an impaired seroconversion was recorded in 25.6 % of individuals after the first 2 doses of vaccine, and in 8.4 % of patients also after the booster dose. Furthermore, the absence of T-cell immunoreactivity was observed in 3/7 patients tested by QuantiFERON® SARSCoV-2 Starter Set (Qiagen). The efficacy of vaccines, evalu
Published: 2023

6. Canakinumab as first-line biological therapy in Still’s disease and differences between the systemic and the chronic-articular courses: Real-life experience from the international AIDA registry

Author: Vitale, A. Caggiano, V. Maggio, M.C. Lopalco, G. Emmi, G. Sota, J. La Torre, F. Ruscitti, P. Bartoloni, E. Conti, G. Fabiani, C. Mattioli, I. Gaggiano, C. Cardinale, F. Dagna, L. Campochiaro, C. Giacomelli, R. Balistreri, A. Laskari, K. Tufan, A. Ragab, G. Almaghlouth, I.A. Więsik-Szewczyk, E. Pereira, R.M. Frediani, B. Iannone, F. Sfikakis, P.P. Cantarini, L. and Vitale, A. Caggiano, V. Maggio, M.C. Lopalco, G. Emmi, G. Sota, J. La Torre, F. Ruscitti, P. Bartoloni, E. Conti, G. Fabiani, C. Mattioli, I. Gaggiano, C. Cardinale, F. Dagna, L. Campochiaro, C. Giacomelli, R. Balistreri, A. Laskari, K. Tufan, A. Ragab, G. Almaghlouth, I.A. Więsik-Szewczyk, E. Pereira, R.M. Frediani, B. Iannone, F. Sfikakis, P.P. Cantarini, L.
Published: 2022

7. Development and implementation of the AIDA International Registry for patients with Behçet’s disease

Author: Vitale, A. Della Casa, F. Ragab, G. Almaghlouth, I.A. Lopalco, G. Pereira, R.M. Guerriero, S. Govoni, M. Sfikakis, P.P. 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, G.D. Bartoloni, E. Akkoç, N. Gündüz, Ö.S. Cattalini, M. Conti, G. Hatemi, G. Maier, A. Parronchi, P. Del Giudice, E. Erten, S. Insalaco, A. Li Gobbi, F. Maggio, M.C. Shahram, F. Caggiano, V. Hegazy, M.T. Asfina, K.N. Morrone, M. Prado, L.L. Dammacco, R. Ruffilli, F. Arida, A. Navarini, L. Pantano, I. Cavagna, L. Conforti, A. Cauli, A. Marucco, E.M. Kucuk, H. Ionescu, R. Mattioli, I. Espinosa, G. Araújo, O. Karkaş, B. Canofari, C. Sota, J. Laymouna, A.H. Bedaiwi, A.A. Colella, S. Giardini, H.A.M. Albano, V. Lo Monaco, A. Fragoulis, G.E. Kardas, R.C. Berlengiero, V. Hussein, M.A. Ricci, F. La Torre, F. Rigante, D. Więsik-Szewczyk, E. Frassi, M. Gentileschi, S. Tosi, G.M. Dagostin, M.A. Mahmoud, A.A.-M.A. 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. Autoinflammatory Diseases Alliance (AIDA) Network and Vitale, A. Della Casa, F. Ragab, G. Almaghlouth, I.A. Lopalco, G. Pereira, R.M. Guerriero, S. Govoni, M. Sfikakis, P.P. 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, G.D. Bartoloni, E. Akkoç, N. Gündüz, Ö.S. Cattalini, M. Conti, G. Hatemi, G. Maier, A. Parronchi, P. Del Giudice, E. Erten, S. Insalaco, A. Li Gobbi, F. Maggio, M.C. Shahram, F. Caggiano, V. Hegazy, M.T. Asfina, K.N. Morrone, M. Prado, L.L. Dammacco, R. Ruffilli, F. Arida, A. Navarini, L. Pantano, I. Cavagna, L. Conforti, A. Cauli, A. Marucco, E.M. Kucuk, H. Ionescu, R. Mattioli, I. Espinosa, G. Araújo, O. Karkaş, B. Canofari, C. Sota, J. Laymouna, A.H. Bedaiwi, A.A. Colella, S. Giardini, H.A.M. Albano, V. Lo Monaco, A. Fragoulis, G.E. Kardas, R.C. Berlengiero, V. Hussein, M.A. Ricci, F. La Torre, F. Rigante, D. Więsik-Szewczyk, E. Frassi, M. Gentileschi, S. Tosi, G.M. Dagostin, M.A. Mahmoud, A.A.-M.A. 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. Autoinflammatory Diseases Alliance (AIDA) Network
Abstract: 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). The Registry is a clinical physician-driven non-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. Starting from January 31st, 2021, to February 7th, 2022, 110 centres from 23 countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5993 fields organised into 16 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. 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. Trial registration NCT05200715 in 21/01/2022. © 2022, The Author(s).
Published: 2022

8. Efficacy and safety of tocilizumab in adult-onset Still's disease: Real-life experience from the international AIDA registry

Author: Sota, J. Vitale, A. Lopalco, G. Pereira, R.M.R. Giordano, H.F. Antonelli, I.P.B. Makowska, J. Brzezińska, O. Lewandowska-Polak, A. Ruscitti, P. Cipriani, P. Cola, I.D. Govoni, M. Ruffili, F. Sfikakis, P.P. Laskari, K. Ragab, G. Hussein, M.A. Gentileschi, S. Gaggiano, C. La Torre, F. Maier, A. Emmi, G. Marino, A. Ciccia, F. Sfriso, P. Maggio, M.C. Bartoloni, E. Lomater, C. Hegazy, M.T. Tektonidou, M. Dagostin, M.A. Opinc, A. Sebastiani, G.D. Giacomelli, R. Giudice, E.D. Olivieri, A.N. Tufan, A. Kardas, R.K. Nuzzolese, R. Cardinale, F. Więsik-Szewczyk, E. Veronica, P. Tarsia, M. Iannone, F. Della Casa, F. Fabiani, C. Frediani, B. Balistreri, A. Rigante, D. Cantarini, L. and Sota, J. Vitale, A. Lopalco, G. Pereira, R.M.R. Giordano, H.F. Antonelli, I.P.B. Makowska, J. Brzezińska, O. Lewandowska-Polak, A. Ruscitti, P. Cipriani, P. Cola, I.D. Govoni, M. Ruffili, F. Sfikakis, P.P. Laskari, K. Ragab, G. Hussein, M.A. Gentileschi, S. Gaggiano, C. La Torre, F. Maier, A. Emmi, G. Marino, A. Ciccia, F. Sfriso, P. Maggio, M.C. Bartoloni, E. Lomater, C. Hegazy, M.T. Tektonidou, M. Dagostin, M.A. Opinc, A. Sebastiani, G.D. Giacomelli, R. Giudice, E.D. Olivieri, A.N. Tufan, A. Kardas, R.K. Nuzzolese, R. Cardinale, F. Więsik-Szewczyk, E. Veronica, P. Tarsia, M. Iannone, F. Della Casa, F. Fabiani, C. Frediani, B. Balistreri, A. Rigante, D. Cantarini, L.
Abstract: Background/objectives: Long-term efficacy and safety of tocilizumab (TCZ) in adult-onset Still's disease (AOSD) mostly derive from small case series. Herein we report a registry-based study investigating TCZ efficacy and safety in a cohort of patients with AOSD evaluated by clinical and serum inflammatory markers as well as drug retention rate analysis. Methods: This is an international multicentre study analyzing data from patients with AOSD regularly enrolled in the AIDA registry. TCZ efficacy was evaluated between baseline and last follow-up assessment in terms of changes in the Pouchot score and laboratory findings. Drug-retention rate was estimated by the Kaplan-Meier method, while Cox-regression analysis was employed to detect potential predictive factors of treatment withdrawal. Results: Data from 31 patients (15 men, 16 women) refractory to the conventional therapies and treated with TCZ were extracted from the AIDA registry. Mean ± SD time of treatment duration with TCZ was 24.32 ± 20.57 months. Median (IRQ) Pouchot score significantly decreased throughout the study period (p=0.001) with a significant difference between baseline [2.00 (4.00)] and 6 month-follow-up [0.00 (0.00)] (p=0.003) and between baseline and last follow-up assessment [0.00 (0.00)] (p=0.032), while no differences were observed between 6 month-evaluation and last follow-up assessment (p=0.823). Similarly, laboratory parameters significantly decreased from baseline to the last follow-up visit. With regard to drug survival, cumulative TCZ retention rate at 12-, 24-, and 36-month follow-up visit were 83.1%, 71.7% and 63.7%, respectively, without significant differences between biologic naïve patients and those previously treated with other biologics (p=0.329). Likewise, no significant differences were observed between chronic articular course of AOSD and other types of disease course (p=0.938) or between patients co-administered with conventional immunosuppressants and patients receivin
Published: 2022

9. Development and Implementation of the AIDA International Registry for Patients With Still's Disease

Author: Vitale, A. Della Casa, F. Lopalco, G. Pereira, R.M. Ruscitti, P. Giacomelli, R. Ragab, G. La Torre, F. Bartoloni, E. Del Giudice, E. Lomater, C. Emmi, G. Govoni, M. Maggio, M.C. Maier, A. Makowska, J. Ogunjimi, B. Sfikakis, P.P. Sfriso, P. Gaggiano, C. Iannone, F. Dagostin, M.A. Di Cola, I. Navarini, L. Ahmed Mahmoud, A.A. Cardinale, F. Riccucci, I. Paroli, M.P. Marucco, E.M. Mattioli, I. Sota, J. Abbruzzese, A. Antonelli, I.P.B. Cipriani, P. Tufan, A. Fabiani, C. Ramadan, M.M. Cattalini, M. Kardas, R.C. Sebastiani, G.D. Giardini, H.A.M. Hernández-Rodríguez, J. Mastrorilli, V. Więsik-Szewczyk, E. Frassi, M. Caggiano, V. Telesca, S. Giordano, H.F. Guadalupi, E. Giani, T. Renieri, A. Colella, S. Cataldi, G. Gentile, M. Fabbiani, A. Al-Maghlouth, I.A. Frediani, B. Balistreri, A. Rigante, D. Cantarini, L. The Autoinflammatory Diseases Alliance (AIDA) Network and Vitale, A. Della Casa, F. Lopalco, G. Pereira, R.M. Ruscitti, P. Giacomelli, R. Ragab, G. La Torre, F. Bartoloni, E. Del Giudice, E. Lomater, C. Emmi, G. Govoni, M. Maggio, M.C. Maier, A. Makowska, J. Ogunjimi, B. Sfikakis, P.P. Sfriso, P. Gaggiano, C. Iannone, F. Dagostin, M.A. Di Cola, I. Navarini, L. Ahmed Mahmoud, A.A. Cardinale, F. Riccucci, I. Paroli, M.P. Marucco, E.M. Mattioli, I. Sota, J. Abbruzzese, A. Antonelli, I.P.B. Cipriani, P. Tufan, A. Fabiani, C. Ramadan, M.M. Cattalini, M. Kardas, R.C. Sebastiani, G.D. Giardini, H.A.M. Hernández-Rodríguez, J. Mastrorilli, V. Więsik-Szewczyk, E. Frassi, M. Caggiano, V. Telesca, S. Giordano, H.F. Guadalupi, E. Giani, T. Renieri, A. Colella, S. Cataldi, G. Gentile, M. Fabbiani, A. Al-Maghlouth, I.A. Frediani, B. Balistreri, A. Rigante, D. Cantarini, L. The Autoinflammatory Diseases Alliance (AIDA) Network
Abstract: Objective: Aim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder. Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients' management. The collection of standardized data is thought to bring knowledge about real-life clinical research and potentially communicate with other existing and future Registries dedicated to Still's disease. Moreover, it has been conceived to be flexible enough to easily change according to future scientific acquisitions. Results: Starting from June 30th to February 7th, 2022, 110 Centers from 23 Countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 4449 fields organized into 14 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: This international Registry for patients with Still's disease will allow a robust clinical research through collection of standardized data, international consultation, dissemination of knowledge, and implementation of observational studies based on wide cohorts of patients followed-up for very long periods. Solid evidence drawn from “real-life” data represen
Published: 2022

10. Addressing the clinical unmet needs in primary Sjögren's Syndrome through the sharing, harmonization and federated analysis of 21 European cohorts

Author: Pezoulas, V.C. Goules, A. Kalatzis, F. Chatzis, L. Kourou, K.D. Venetsanopoulou, A. Exarchos, T.P. Gandolfo, S. Votis, K. Zampeli, E. Burmeister, J. May, T. Marcelino Pérez, M. Lishchuk, I. Chondrogiannis, T. Andronikou, V. Varvarigou, T. Filipovic, N. Tsiknakis, M. Baldini, C. Bombardieri, M. Bootsma, H. Bowman, S.J. Shahnawaz Soyfoo, M. Parisis, D. Delporte, C. Devauchelle-Pensec, V. Pers, J.-O. Dörner, T. Bartoloni, E. Gerli, R. Giacomelli, R. Jonsson, R. Ng, W.-F. Priori, R. Ramos-Casals, M. Sivils, K. Skopouli, F. Torsten, W. A. G. van Roon, J. Xavier, M. De Vita, S. Tzioufas, A.G. Fotiadis, D.I. and Pezoulas, V.C. Goules, A. Kalatzis, F. Chatzis, L. Kourou, K.D. Venetsanopoulou, A. Exarchos, T.P. Gandolfo, S. Votis, K. Zampeli, E. Burmeister, J. May, T. Marcelino Pérez, M. Lishchuk, I. Chondrogiannis, T. Andronikou, V. Varvarigou, T. Filipovic, N. Tsiknakis, M. Baldini, C. Bombardieri, M. Bootsma, H. Bowman, S.J. Shahnawaz Soyfoo, M. Parisis, D. Delporte, C. Devauchelle-Pensec, V. Pers, J.-O. Dörner, T. Bartoloni, E. Gerli, R. Giacomelli, R. Jonsson, R. Ng, W.-F. Priori, R. Ramos-Casals, M. Sivils, K. Skopouli, F. Torsten, W. A. G. van Roon, J. Xavier, M. De Vita, S. Tzioufas, A.G. Fotiadis, D.I.
Abstract: For many decades, the clinical unmet needs of primary Sjögren's Syndrome (pSS) have been left unresolved due to the rareness of the disease and the complexity of the underlying pathogenic mechanisms, including the pSS-associated lymphomagenesis process. Here, we present the HarmonicSS cloud-computing exemplar which offers beyond the state-of-the-art data analytics services to address the pSS clinical unmet needs, including the development of lymphoma classification models and the identification of biomarkers for lymphomagenesis. The users of the platform have been able to successfully interlink, curate, and harmonize 21 regional, national, and international European cohorts of 7,551 pSS patients with respect to the ethical and legal issues for data sharing. Federated AI algorithms were trained across the harmonized databases, with reduced execution time complexity, yielding robust lymphoma classification models with 85% accuracy, 81.25% sensitivity, 85.4% specificity along with 5 biomarkers for lymphoma development. To our knowledge, this is the first GDPR compliant platform that provides federated AI services to address the pSS clinical unmet needs. © 2022 The Author(s)
Published: 2022

11. Absent or suboptimal response to booster dose of COVID-19 vaccine in patients with autoimmune systemic diseases

Author: Ferri, C., Gragnani, L., Raimondo, V., Visentini, M., Giuggioli, D., Lorini, S., Foti, R., Cacciapaglia, F., Caminiti, M., Olivo, D., Cuomo, G., Pellegrini, R., Pigatto, E., Urraro, T., Naclerio, C., Tavoni, A., Puccetti, L., Cavazzana, I., Ruscitti, P., Vadacca, M., La Gualana, F., Cozzi, F., Spinella, A., Visalli, E., Bosco, Y. D., Amato, G., Masini, F., Mariano, G. P., Brittelli, R., Aiello, V., Scorpiniti, D., Rechichi, G., Varcasia, G., Monti, M., Elia, G., Franceschini, F., Casato, M., Ursini, F., Giacomelli, R., Fallahi, P., Santini, Stefano Angelo, Iannone, F., Salvarani, C., Zignego, A. L., Antonelli, A., Santini S. A. (ORCID:0000-0003-1956-5899), Ferri, C., Gragnani, L., Raimondo, V., Visentini, M., Giuggioli, D., Lorini, S., Foti, R., Cacciapaglia, F., Caminiti, M., Olivo, D., Cuomo, G., Pellegrini, R., Pigatto, E., Urraro, T., Naclerio, C., Tavoni, A., Puccetti, L., Cavazzana, I., Ruscitti, P., Vadacca, M., La Gualana, F., Cozzi, F., Spinella, A., Visalli, E., Bosco, Y. D., Amato, G., Masini, F., Mariano, G. P., Brittelli, R., Aiello, V., Scorpiniti, D., Rechichi, G., Varcasia, G., Monti, M., Elia, G., Franceschini, F., Casato, M., Ursini, F., Giacomelli, R., Fallahi, P., Santini, Stefano Angelo, Iannone, F., Salvarani, C., Zignego, A. L., Antonelli, A., and Santini S. A. (ORCID:0000-0003-1956-5899)
Abstract: Autoimmune systemic diseases (ASD) show impaired immunogenicity to COVID-19 vaccines. Our prospective observational multicenter study aimed at evaluating the seroconversion elicited by COVID-19 vaccine over the entire vaccination cycle including the booster dose. Among 478 unselected ASD patients originally evaluated at the end of the first vaccination cycle (time 1), 344 individuals were re-evaluated after a 6-month period (time 2), and 244 after the booster vaccine dose (time 3). The immunogenicity of mRNA COVID-19 vaccines (BNT162b2 and mRNA-1273) was assessed by measuring serum IgG-neutralizing antibody (NAb) on samples obtained at the three time points in both patients and 502 age-matched controls. In the 244 ASD group that received booster vaccine and monitored over the entire follow-up, the mean serum NAb levels (time 1, 2, and 3: 696.8 ± 52.68, 370.8 ± 41.92, and 1527 ± 74.16SD BAU/mL, respectively; p < 0.0001) were constantly lower compared to controls (p < 0.0001), but they significantly increased after the booster dose compared to the first two measurements (p < 0.0001). The percentage of patients with absent/suboptimal response to vaccine significantly decreased after the booster dose compared to the first and second evaluations (time 1, 2, and 3: from 28.2% to 46.3%, and to 7.8%, respectively; p < 0.0001). Of note, the percentage of patients with absent/suboptimal response after the booster dose was significantly higher compared to controls (19/244, 7.8% vs 1/502, 0.2%; p < 0.0001). Similarly, treatment with immune-modifiers increased the percentage of patients exhibiting absent/suboptimal response (16/122, 13.1% vs 3/122, 2.46%; p = 0.0031). Overall, the above findings indicate the usefulness of booster vaccine administration in ASD patients. Moreover, the persistence of a significantly higher percentage of individuals without effective seroconversion (7.8%), even after the booster dose, warrants for careful monitoring of NAb levels in a
Published: 2022

12. 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

13. 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

14. Efficacy and safety of tocilizumab in adult-onset Still's disease: Real-life experience from the international AIDA registry

Author: Sota, J, Vitale, A, Lopalco, G, Pereira, Rmr, Giordano, Hf, Antonelli, Ipb, Makowska, J, Brzezińska, O, Lewandowska-Polak, A, Ruscitti, P, Cipriani, P, Cola, Id, Govoni, M, Ruffili, F, Sfikakis, Pp, Laskari, K, Ragab, G, Hussein, Ma, Gentileschi, S, Gaggiano, C, La Torre, F, Maier, A, Emmi, G, Marino, A, Ciccia, F, Sfriso, P, Maggio, Mc, Bartoloni, E, Lomater, C, Hegazy, Mt, Tektonidou, M, Dagostin, Ma, Opinc, A, Sebastiani, Gd, Giacomelli, R, Giudice, Ed, Olivieri, An, Tufan, A, Kardas, Rk, Nuzzolese, R, Cardinale, F, Więsik-Szewczyk, E, Veronica, P, Tarsia, M, Iannone, F, Della Casa, F, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Sota, J, Vitale, A, Lopalco, G, Pereira, Rmr, Giordano, Hf, Antonelli, Ipb, Makowska, J, Brzezińska, O, Lewandowska-Polak, A, Ruscitti, P, Cipriani, P, Cola, Id, Govoni, M, Ruffili, F, Sfikakis, Pp, Laskari, K, Ragab, G, Hussein, Ma, Gentileschi, S, Gaggiano, C, La Torre, F, Maier, A, Emmi, G, Marino, A, Ciccia, F, Sfriso, P, Maggio, Mc, Bartoloni, E, Lomater, C, Hegazy, Mt, Tektonidou, M, Dagostin, Ma, Opinc, A, Sebastiani, Gd, Giacomelli, R, Giudice, Ed, Olivieri, An, Tufan, A, Kardas, Rk, Nuzzolese, R, Cardinale, F, Więsik-Szewczyk, E, Veronica, P, Tarsia, M, Iannone, F, Della Casa, F, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: Background/objectives: Long-term efficacy and safety of tocilizumab (TCZ) in adult-onset Still’s disease (AOSD) mostly derive from small case series. Herein we report a registry-based study investigating TCZ efficacy and safety in a cohort of patients with AOSD evaluated by clinical and serum inflammatory markers as well as drug retention rate analysis. Methods: This is an international multicentre study analyzing data from patients with AOSD regularly enrolled in the AIDA registry. TCZ efficacy was evaluated between baseline and last follow-up assessment in terms of changes in the Pouchot score and laboratory findings. Drug-retention rate was estimated by the Kaplan-Meier method, while Cox-regression analysis was employed to detect potential predictive factors of treatment withdrawal. Results: Data from 31 patients (15 men, 16 women) refractory to the conventional therapies and treated with TCZ were extracted from the AIDA registry. Mean ± SD time of treatment duration with TCZ was 24.32 ± 20.57 months. Pouchot score significantly decreased from baseline 2.00 (4.00) to the last follow-up assessment 00.00 (00.00), p=0.003). Similarly, laboratory parameters significantly decreased from baseline to the last follow-up visit. With regard to drug survival, cumulative TCZ retention rate at 12-, 24-, and 36-month follow-up visit were 83.1%, 71.7% and 63.7%, respectively, without significant differences between biologic naïve patients and those previously treated with other biologics (p=0.329). Likewise, no significant differences were observed between chronic articular course of AOSD and other types of disease course (p=0.938) or between patients co-administered with conventional immunosuppressants and patients receiving TCZ as monotherapy (p=0.778). Cox-regression analysis identified no variable associated with a higher hazard of treatment withdrawal. Treatment was discontinued in 9 patients due to long-term remission (n=4), adverse events (n=2), loss of efficacy (n=1)
Published: 2022

15. Development and implementation of the AIDA International Registry for patients with Still’s disease

Author: Vitale, A, Della Casa, F, Lopalco, G, Pereira, Rm, Ruscitti, P, Giacomelli, R, Ragab, G, La Torre, F, Bartoloni, E, Del Giudice, E, Lomater, C, Emmi, G, Govoni, M, Maggio, Mc, Maier, A, Makowska, J, Ogunjimi, B, Sfikakis, Pp, Sfriso, P, Gaggiano, C, Iannone, F, Dagostin, Ma, Di Cola, I, Navarini, L, Ahmed Mahmoud, Aa, Cardinale, F, Riccucci, I, Paroli, Mp, Marucco, Em, Mattioli, I, Sota, J, Abbruzzese, A, Antonelli, Ipb, Cipriani, P, Tufan, A, Fabiani, C, Ramadan, Mm, Cattalini, M, Kardas, Rc, Sebastiani, Gd, Giardini, Ham, Hernández-Rodríguez, J, Mastrorilli, V, Więsik-Szewczyk, E, Frassi, M, Caggiano, V, Telesca, S, Giordano, Hf, Guadalupi, E, Balistreri, A, Rigante, Donato, Cantarini, L., Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Della Casa, F, Lopalco, G, Pereira, Rm, Ruscitti, P, Giacomelli, R, Ragab, G, La Torre, F, Bartoloni, E, Del Giudice, E, Lomater, C, Emmi, G, Govoni, M, Maggio, Mc, Maier, A, Makowska, J, Ogunjimi, B, Sfikakis, Pp, Sfriso, P, Gaggiano, C, Iannone, F, Dagostin, Ma, Di Cola, I, Navarini, L, Ahmed Mahmoud, Aa, Cardinale, F, Riccucci, I, Paroli, Mp, Marucco, Em, Mattioli, I, Sota, J, Abbruzzese, A, Antonelli, Ipb, Cipriani, P, Tufan, A, Fabiani, C, Ramadan, Mm, Cattalini, M, Kardas, Rc, Sebastiani, Gd, Giardini, Ham, Hernández-Rodríguez, J, Mastrorilli, V, Więsik-Szewczyk, E, Frassi, M, Caggiano, V, Telesca, S, Giordano, Hf, Guadalupi, E, Balistreri, A, Rigante, Donato, Cantarini, L., and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: Objective: Aim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder. Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients’ management. The collection of standardized data is thought to bring knowledge about real-life clinical research and potentially communicate with other existing and future Registries dedicated to Still’s disease. Moreover, it has been conceived to be flexible enough to easily change according to future scientific acquisitions. Results: Starting from June 30th to February 7th, 2022, 110 Centers from 23 Countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 4449 fields organized into 14 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: This international Registry for patients with Still’s disease will allow a robust clinical research through collection of standardized data, international consultation, dissemination of knowledge, and implementation of observational studies based on wide cohorts of patients followed-up for very long periods. Solid evidence drawn from “real-life” data represents the ultimate goal of t
Published: 2022

16. Italian recommendations for influenza and pneumococcal vaccination in adult patients with autoimmune rheumatic diseases

Author: Guerrini, G, Franzetti, F, Giacomelli, R, Meroni, L, Riva, A, Scire, C, Scrivo, R, Tavio, M, Agostinone, A, Airo, P, Atzeni, F, Bartalesi, F, Bazzichi, L, Berardicurti, O, Cassola, G, Castagna, A, Castelli, F, Cattelan, A, Citriniti, G, Cristini, F, De Rosa, F, Fracassi, E, Galloway, J, La Paglia, G, Moioli, M, Ripamonti, D, Saracino, A, Tani, C, Tascini, C, Tieghi, T, Tinelli, M, Zabotti, A, Sarzi-Puttini, P, Galli, M, Guerrini G., Franzetti F., Giacomelli R., Meroni L., Riva A., Scire C. A., Scrivo R., Tavio M., Agostinone A., Airo P., Atzeni F., Bartalesi F., Bazzichi L., Berardicurti O., Cassola G., Castagna A., Castelli F., Cattelan A., Citriniti G., Cristini F., De Rosa F., Fracassi E., Galloway J., La Paglia G. M. C., Moioli M. C., Ripamonti D., Saracino A., Tani C., Tascini C., Tieghi T., Tinelli M., Zabotti A., Sarzi-Puttini P., Galli M., Guerrini, G, Franzetti, F, Giacomelli, R, Meroni, L, Riva, A, Scire, C, Scrivo, R, Tavio, M, Agostinone, A, Airo, P, Atzeni, F, Bartalesi, F, Bazzichi, L, Berardicurti, O, Cassola, G, Castagna, A, Castelli, F, Cattelan, A, Citriniti, G, Cristini, F, De Rosa, F, Fracassi, E, Galloway, J, La Paglia, G, Moioli, M, Ripamonti, D, Saracino, A, Tani, C, Tascini, C, Tieghi, T, Tinelli, M, Zabotti, A, Sarzi-Puttini, P, Galli, M, Guerrini G., Franzetti F., Giacomelli R., Meroni L., Riva A., Scire C. A., Scrivo R., Tavio M., Agostinone A., Airo P., Atzeni F., Bartalesi F., Bazzichi L., Berardicurti O., Cassola G., Castagna A., Castelli F., Cattelan A., Citriniti G., Cristini F., De Rosa F., Fracassi E., Galloway J., La Paglia G. M. C., Moioli M. C., Ripamonti D., Saracino A., Tani C., Tascini C., Tieghi T., Tinelli M., Zabotti A., Sarzi-Puttini P., and Galli M.
Abstract: OBJECTIVES: To provide evidence-based recommendations for vaccination against influenza virus and S. pneumoniae in patients with autoimmune rheumatic diseases (ARDs). METHODS: A Consensus Committee including physicians with expertise in rheumatic and infectious diseases was established by two Italian scientific societies, Società Italiana di Reumatologia (SIR) and Società Italiana di Malattie Infettive e Tropicali (SIMIT). The experts were invited to develop evidence-based recommendations concerning vaccinations in ARDs patients, based on their clinical status before and after undergoing immunosuppressive treatments. Key clinical questions were formulated for the systematic literature reviews, based on the clinical pathway. A search was made in Medline (via PubMed) according to the original MeSH strategy from October 2009 and a keyword strategy from January 2016 up to December 2017, updating existing EULAR recommendations. Specific recommendations were separately voted and scored from 0 (no agreement with) to 100 (maximal agreement) and supporting evidence graded. The mean and standard deviation of the scores were calculated to determine the level of agreement among the experts' panel for each recommendation. Total cumulative agreement ≥70 defined consensus for each statement. RESULTS: Nine recommendations, based on 6 key clinical questions addressed by the expert committee, were proposed. The aim of this work is to integrate the 2011 EULAR recommendations on vaccination against influenza and S. pneumoniae in ARDs patients. An implementation plan was proposed to improve the vaccination status of these patients and their safety during immunosuppressive treatments. CONCLUSIONS: Influenza and pneumococcus vaccinations are effective and safe in patients with ARDs. More efforts should be made to translate the accumulated evidence into practice.
Published: 2020

17. Italian recommendations for influenza and pneumococcal vaccination in adult patients with autoimmune rheumatic diseases

Author: Guerrini, G, Franzetti, F, Giacomelli, R, Meroni, L, Riva, A, Scire, C, Scrivo, R, Tavio, M, Agostinone, A, Airo, P, Atzeni, F, Bartalesi, F, Bazzichi, L, Berardicurti, O, Cassola, G, Castagna, A, Castelli, F, Cattelan, A, Citriniti, G, Cristini, F, De Rosa, F, Fracassi, E, Galloway, J, La Paglia, G, Moioli, M, Ripamonti, D, Saracino, A, Tani, C, Tascini, C, Tieghi, T, Tinelli, M, Zabotti, A, Sarzi-Puttini, P, Galli, M, Guerrini G., Franzetti F., Giacomelli R., Meroni L., Riva A., Scire C. A., Scrivo R., Tavio M., Agostinone A., Airo P., Atzeni F., Bartalesi F., Bazzichi L., Berardicurti O., Cassola G., Castagna A., Castelli F., Cattelan A., Citriniti G., Cristini F., De Rosa F., Fracassi E., Galloway J., La Paglia G. M. C., Moioli M. C., Ripamonti D., Saracino A., Tani C., Tascini C., Tieghi T., Tinelli M., Zabotti A., Sarzi-Puttini P., Galli M., Guerrini, G, Franzetti, F, Giacomelli, R, Meroni, L, Riva, A, Scire, C, Scrivo, R, Tavio, M, Agostinone, A, Airo, P, Atzeni, F, Bartalesi, F, Bazzichi, L, Berardicurti, O, Cassola, G, Castagna, A, Castelli, F, Cattelan, A, Citriniti, G, Cristini, F, De Rosa, F, Fracassi, E, Galloway, J, La Paglia, G, Moioli, M, Ripamonti, D, Saracino, A, Tani, C, Tascini, C, Tieghi, T, Tinelli, M, Zabotti, A, Sarzi-Puttini, P, Galli, M, Guerrini G., Franzetti F., Giacomelli R., Meroni L., Riva A., Scire C. A., Scrivo R., Tavio M., Agostinone A., Airo P., Atzeni F., Bartalesi F., Bazzichi L., Berardicurti O., Cassola G., Castagna A., Castelli F., Cattelan A., Citriniti G., Cristini F., De Rosa F., Fracassi E., Galloway J., La Paglia G. M. C., Moioli M. C., Ripamonti D., Saracino A., Tani C., Tascini C., Tieghi T., Tinelli M., Zabotti A., Sarzi-Puttini P., and Galli M.
Abstract: OBJECTIVES: To provide evidence-based recommendations for vaccination against influenza virus and S. pneumoniae in patients with autoimmune rheumatic diseases (ARDs). METHODS: A Consensus Committee including physicians with expertise in rheumatic and infectious diseases was established by two Italian scientific societies, Società Italiana di Reumatologia (SIR) and Società Italiana di Malattie Infettive e Tropicali (SIMIT). The experts were invited to develop evidence-based recommendations concerning vaccinations in ARDs patients, based on their clinical status before and after undergoing immunosuppressive treatments. Key clinical questions were formulated for the systematic literature reviews, based on the clinical pathway. A search was made in Medline (via PubMed) according to the original MeSH strategy from October 2009 and a keyword strategy from January 2016 up to December 2017, updating existing EULAR recommendations. Specific recommendations were separately voted and scored from 0 (no agreement with) to 100 (maximal agreement) and supporting evidence graded. The mean and standard deviation of the scores were calculated to determine the level of agreement among the experts' panel for each recommendation. Total cumulative agreement ≥70 defined consensus for each statement. RESULTS: Nine recommendations, based on 6 key clinical questions addressed by the expert committee, were proposed. The aim of this work is to integrate the 2011 EULAR recommendations on vaccination against influenza and S. pneumoniae in ARDs patients. An implementation plan was proposed to improve the vaccination status of these patients and their safety during immunosuppressive treatments. CONCLUSIONS: Influenza and pneumococcus vaccinations are effective and safe in patients with ARDs. More efforts should be made to translate the accumulated evidence into practice.
Published: 2020

18. The growing role of precision medicine for the treatment of autoimmune diseases; results of a systematic review of literature and Experts’ Consensus

Author: Giacomelli, R. Afeltra, A. Bartoloni, E. Berardicurti, O. Bombardieri, M. Bortoluzzi, A. Carubbi, F. Caso, F. Cervera, R. Ciccia, F. Cipriani, P. Coloma-Bazán, E. Conti, F. Costa, L. D'Angelo, S. Distler, O. Feist, E. Foulquier, N. Gabini, M. Gerber, V. Gerli, R. Grembiale, R.D. Guggino, G. Hoxha, A. Iagnocco, A. Jordan, S. Kahaleh, B. Lauper, K. Liakouli, V. Lubrano, E. Margiotta, D. Naty, S. Navarini, L. Perosa, F. Perricone, C. Perricone, R. Prete, M. Pers, J.-O. Pitzalis, C. Priori, R. Rivellese, F. Ruffatti, A. Ruscitti, P. Scarpa, R. Shoenfeld, Y. Triolo, G. Tzioufas, A. and Giacomelli, R. Afeltra, A. Bartoloni, E. Berardicurti, O. Bombardieri, M. Bortoluzzi, A. Carubbi, F. Caso, F. Cervera, R. Ciccia, F. Cipriani, P. Coloma-Bazán, E. Conti, F. Costa, L. D'Angelo, S. Distler, O. Feist, E. Foulquier, N. Gabini, M. Gerber, V. Gerli, R. Grembiale, R.D. Guggino, G. Hoxha, A. Iagnocco, A. Jordan, S. Kahaleh, B. Lauper, K. Liakouli, V. Lubrano, E. Margiotta, D. Naty, S. Navarini, L. Perosa, F. Perricone, C. Perricone, R. Prete, M. Pers, J.-O. Pitzalis, C. Priori, R. Rivellese, F. Ruffatti, A. Ruscitti, P. Scarpa, R. Shoenfeld, Y. Triolo, G. Tzioufas, A.
Abstract: Autoimmune diseases (AIDs) share similar serological, clinical, and radiological findings, but, behind these common features, there are different pathogenic mechanisms, immune cells dysfunctions, and targeted organs. In this context, multiple lines of evidence suggest the application of precision medicine principles to AIDs to reduce the treatment failure. Precision medicine refers to the tailoring of therapeutic strategies to the individual characteristics of each patient, thus it could be a new approach for management of AIDS which considers individual variability in genes, environmental exposure, and lifestyle. Precision medicine would also assist physicians in choosing the right treatment, the best timing of administration, consequently trying to maximize drug efficacy, and, possibly, reducing adverse events. In this work, the growing body of evidence is summarized regarding the predictive factors for drug response in patients with AIDs, applying the precision medicine principles to provide high-quality evidence for therapeutic opportunities in improving the management of these patients. © 2020
Published: 2021

19. Anakinra and canakinumab for patients with R92Q-associated autoinflammatory syndrome: a multicenter observational study from the AIDA Network

Author: Gaggiano, C, Rigante, Donato, Hernández-Rodríguez, J, Vitale, A, Tarsia, M, Soriano, A, Lopalco, G, Iannone, F, Abdel Jaber, M, Giacomelli, R, Więsik-Szewczyk, E, Cattalini, M, Frassi, M, Piga, M, Ragab, G, Sota, J, Zunica, F, Floris, A, Sabato, V, Hegazy, Mt, Araújo, O, Pelegrin, L, Fabbiani, A, Renieri, A, Grosso, S, Fabiani, C, Frediani, B, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Rigante, Donato, Hernández-Rodríguez, J, Vitale, A, Tarsia, M, Soriano, A, Lopalco, G, Iannone, F, Abdel Jaber, M, Giacomelli, R, Więsik-Szewczyk, E, Cattalini, M, Frassi, M, Piga, M, Ragab, G, Sota, J, Zunica, F, Floris, A, Sabato, V, Hegazy, Mt, Araújo, O, Pelegrin, L, Fabbiani, A, Renieri, A, Grosso, S, Fabiani, C, Frediani, B, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)
Published: 2021

20. Biotechnological agents for patients with tumor necrosis factor receptor associated periodic syndrome - Therapeutic outcome and predictors of response: real-life data from the AIDA Network

Author: Vitale, A, Obici, L, Cattalini, M, Lopalco, G, Merlini, G, Ricco, N, Soriano, A, La Torre, F, Verrecchia, Elena, Insalaco, A, Dagna, L, Jaber, Ma, Montin, D, Emmi, G, Ciarcia, L, Barneschi, S, Parronchi, P, Ruscitti, P, Maggio, Mc, Viapiana, O, Sota, J, Gaggiano, C, Giacomelli, R, Sicignano, Ludovico Luca, Manna, Raffaele, Renieri, A, Lo Rizzo, C, Frediani, B, Rigante, Donato, Cantarini, L, Verrecchia E, Sicignano LL, Manna R (ORCID:0000-0003-1560-3907), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Obici, L, Cattalini, M, Lopalco, G, Merlini, G, Ricco, N, Soriano, A, La Torre, F, Verrecchia, Elena, Insalaco, A, Dagna, L, Jaber, Ma, Montin, D, Emmi, G, Ciarcia, L, Barneschi, S, Parronchi, P, Ruscitti, P, Maggio, Mc, Viapiana, O, Sota, J, Gaggiano, C, Giacomelli, R, Sicignano, Ludovico Luca, Manna, Raffaele, Renieri, A, Lo Rizzo, C, Frediani, B, Rigante, Donato, Cantarini, L, Verrecchia E, Sicignano LL, Manna R (ORCID:0000-0003-1560-3907), and Rigante D (ORCID:0000-0001-7032-7779)
Published: 2021

21. Influence of the age at diagnosis in the disease expression of primary Sjogren's syndrome : Analysis of 12,753 patients from the Sjogren Big Data Consortium

Author: Retamozo, S., Acar-Denizli, N., Horvath, I. F., Ng, W-F, Rasmussen, A., Dong, X., Li, X., Baldini, C., Olsson, P., Priori, R., Seror, R., Gottenberg, J-E, Kruize, A. A., Hernandez-Molina, G., Vissink, A., Sandhya, P., Armagan, B., Quartuccio, L., Sebastian, A., Praprotnik, S., Bartoloni, E., Kwok, S-K, Kvarnstrom, M., Rischmueller, M., Solans-Laque, R., Sene, D., Pasoto, S. G., Suzuki, Y., Isenberg, D. A., Valim, V., Nordmark, Gunnel, Nakamura, H., Fernandes Moca Trevisani, V, Hofauer, B., Siso-Almirall, A., Giacomelli, R., Devauchelle-Pensec, V, Bombardieri, M., Atzeni, F., Hammenfors, D., Maure, B., Carsons, S. E., Gheita, T., Sanchez-Berna, I, Lopez-Dupla, M., Morel, J., Inanc, N., Fonseca-Aizpuru, E., Morcillo, C., Vollenweider, C., Melchor, S., Vazquez, M., Diaz-Cuiza, E., Consani-Fernandez, S., de-Miguel-Campo, B., Szanto, A., Bombardieri, S., Gattamelata, A., Hinrichs, A., Sanchez-Guerrero, J., Danda, D., Kilic, L., de Vita, S., Wiland, P., Gerli, R., Park, S-H, Wahren-Herlenius, M., Bootsma, H., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Retamozo, S., Acar-Denizli, N., Horvath, I. F., Ng, W-F, Rasmussen, A., Dong, X., Li, X., Baldini, C., Olsson, P., Priori, R., Seror, R., Gottenberg, J-E, Kruize, A. A., Hernandez-Molina, G., Vissink, A., Sandhya, P., Armagan, B., Quartuccio, L., Sebastian, A., Praprotnik, S., Bartoloni, E., Kwok, S-K, Kvarnstrom, M., Rischmueller, M., Solans-Laque, R., Sene, D., Pasoto, S. G., Suzuki, Y., Isenberg, D. A., Valim, V., Nordmark, Gunnel, Nakamura, H., Fernandes Moca Trevisani, V, Hofauer, B., Siso-Almirall, A., Giacomelli, R., Devauchelle-Pensec, V, Bombardieri, M., Atzeni, F., Hammenfors, D., Maure, B., Carsons, S. E., Gheita, T., Sanchez-Berna, I, Lopez-Dupla, M., Morel, J., Inanc, N., Fonseca-Aizpuru, E., Morcillo, C., Vollenweider, C., Melchor, S., Vazquez, M., Diaz-Cuiza, E., Consani-Fernandez, S., de-Miguel-Campo, B., Szanto, A., Bombardieri, S., Gattamelata, A., Hinrichs, A., Sanchez-Guerrero, J., Danda, D., Kilic, L., de Vita, S., Wiland, P., Gerli, R., Park, S-H, Wahren-Herlenius, M., Bootsma, H., Mariette, X., Ramos-Casals, M., and Brito-Zeron, P.
Abstract: Objective. To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjogren's syndrome (pSS). Methods. By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results. There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R-2 0.87) and the frequency of abnormal oral tests (adjusted R-2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.18%), and constitutional, cutaneous, and haematological involvements (the frequency decreased by 0.09% for each 1-year increase). In contrast, other domains showed an ascending pattern, especi
Published: 2021

22. Impaired immunogenicity to COVID-19 vaccines in autoimmune systemic diseases. High prevalence of non-response in different patients’ subgroups

Author: Ferri, C., Ursini, F., Gragnani, L., Raimondo, V., Giuggioli, D., Foti, R., Caminiti, M., Olivo, D., Cuomo, G., Visentini, M., Cacciapaglia, F., Pellegrini, R., Pigatto, E., Urraro, T., Naclerio, C., Tavoni, A., Puccetti, L., Varcasia, G., Cavazzana, I., L'Andolina, M., Ruscitti, P., Vadacca, M., Gigliotti, P., La Gualana, F., Cozzi, F., Spinella, A., Visalli, E., Dal Bosco, Y., Amato, G., Masini, F., Pagano Mariano, G., Brittelli, R., Aiello, V., Caminiti, R., Scorpiniti, D., Rechichi, G., Ferrari, T., Monti, M., Elia, G., Franceschini, F., Meliconi, R., Casato, M., Iannone, F., Giacomelli, R., Fallahi, P., Santini, Stefano Angelo, Zignego, A. L., Antonelli, A., Santini S. A. (ORCID:0000-0003-1956-5899), Ferri, C., Ursini, F., Gragnani, L., Raimondo, V., Giuggioli, D., Foti, R., Caminiti, M., Olivo, D., Cuomo, G., Visentini, M., Cacciapaglia, F., Pellegrini, R., Pigatto, E., Urraro, T., Naclerio, C., Tavoni, A., Puccetti, L., Varcasia, G., Cavazzana, I., L'Andolina, M., Ruscitti, P., Vadacca, M., Gigliotti, P., La Gualana, F., Cozzi, F., Spinella, A., Visalli, E., Dal Bosco, Y., Amato, G., Masini, F., Pagano Mariano, G., Brittelli, R., Aiello, V., Caminiti, R., Scorpiniti, D., Rechichi, G., Ferrari, T., Monti, M., Elia, G., Franceschini, F., Meliconi, R., Casato, M., Iannone, F., Giacomelli, R., Fallahi, P., Santini, Stefano Angelo, Zignego, A. L., Antonelli, A., and Santini S. A. (ORCID:0000-0003-1956-5899)
Abstract: Autoimmune systemic diseases (ASD) may show impaired immunogenicity to COVID-19 vaccines. Our prospective observational multicenter study aimed to evaluate the seroconversion after the vaccination cycle and at 6-12-month follow-up, as well the safety and efficacy of vaccines in preventing COVID-19. The study included 478 unselected ASD patients (mean age 59 ± 15 years), namely 101 rheumatoid arthritis (RA), 38 systemic lupus erythematosus (SLE), 265 systemic sclerosis (SSc), 61 cryoglobulinemic vasculitis (CV), and a miscellanea of 13 systemic vasculitis. The control group included 502 individuals from the general population (mean age 59 ± 14SD years). The immunogenicity of mRNA COVID-19 vaccines (BNT162b2 and mRNA-1273) was evaluated by measuring serum IgG-neutralizing antibody (NAb) (SARS-CoV-2 IgG II Quant antibody test kit; Abbott Laboratories, Chicago, IL) on samples obtained within 3 weeks after vaccination cycle. The short-term results of our prospective study revealed significantly lower NAb levels in ASD series compared to controls [286 (53–1203) vs 825 (451–1542) BAU/mL, p < 0.0001], as well as between single ASD subgroups and controls. More interestingly, higher percentage of non-responders to vaccine was recorded in ASD patients compared to controls [13.2% (63/478), vs 2.8% (14/502); p < 0.0001]. Increased prevalence of non-response to vaccine was also observed in different ASD subgroups, in patients with ASD-related interstitial lung disease (p = 0.009), and in those treated with glucocorticoids (p = 0.002), mycophenolate-mofetil (p < 0.0001), or rituximab (p < 0.0001). Comparable percentages of vaccine-related adverse effects were recorded among responder and non-responder ASD patients. Patients with weak/absent seroconversion, believed to be immune to SARS-CoV-2 infection, are at high risk to develop COVID-19. Early determination of serum NAb after vaccination cycle may allow to identify three main groups of ASD patients: responders, subje
Published: 2021

23. Definition of fibromyalgia severity: Findings from a cross-sectional survey of 2339 Italian patients

Author: Salaffi, F., Di Carlo, M., Bazzichi, L., Atzeni, F., Govoni, M., Biasi, G., Di Franco, M., Mozzani, F., Gremese, E., Dagna, L., Batticciotto, A., Fischetti, F., Giacomelli, R., Guiducci, S., Guggino, G., Bentivegna, M., Gerli, R., Salvarani, C., Bajocchi, G., Ghini, M., Iannone, F., Giorgi, V., Farah, S., Cirillo, M., Bonazza, S., Barbagli, S., Gioia, C., Santilli, D., Capacci, A., Cavalli, G., Carubbi, F., Nacci, F., Riccucci, I., Sinigaglia, L., Masullo, M., Polizzi, B. M., Cutolo, M., Sarzi-Puttini, P., Gremese E. (ORCID:0000-0002-2248-1058), Bentivegna M., Santilli D., Cavalli G. (ORCID:0000-0003-1968-8987), Masullo M., Salaffi, F., Di Carlo, M., Bazzichi, L., Atzeni, F., Govoni, M., Biasi, G., Di Franco, M., Mozzani, F., Gremese, E., Dagna, L., Batticciotto, A., Fischetti, F., Giacomelli, R., Guiducci, S., Guggino, G., Bentivegna, M., Gerli, R., Salvarani, C., Bajocchi, G., Ghini, M., Iannone, F., Giorgi, V., Farah, S., Cirillo, M., Bonazza, S., Barbagli, S., Gioia, C., Santilli, D., Capacci, A., Cavalli, G., Carubbi, F., Nacci, F., Riccucci, I., Sinigaglia, L., Masullo, M., Polizzi, B. M., Cutolo, M., Sarzi-Puttini, P., Gremese E. (ORCID:0000-0002-2248-1058), Bentivegna M., Santilli D., Cavalli G. (ORCID:0000-0003-1968-8987), and Masullo M.
Abstract: Objective: To establish optimal cut-off values for the scores of the revised Fibromyalgia Impact Questionnaire (FIQR), the modified Fibromialgia Assessment Scale (FAS 2019mod), and the Polysymptomatic Distress Scale (PDS) in order to distinguish five levels of FM disease severity. Methods: Consecutive FM patients were evaluated with the three clinimetric indices, and each patient was required to answer the anchor question: 'In general, would you say your health is 1 = very good, 2 = good, 3 = fair, 4 = poor, or 5 = very poor?'-which represented the external criterion. Cut-off points were established through the interquartile reconciliation approach. Results: The study sample consisted of 2181 women (93.2%) and 158 men (6.8%), with a mean age of 51.9 (11.5) years, and mean disease duration was 7.3 (6.9) years. The overall median FIQR, FAS 2019 mod and PDS scores (25th-75th percentiles) were respectively 61.16 (41.16-77.00), 27.00 (19.00-32.00) and 19.0 (13.00-24.00). Reconciliation of the mean 75th and 25th percentiles of adjacent categories defined the severity states for FIQR: 0-23 for remission, 24-40 for mild disease, 41-63 for moderate disease, 64-82 for severe disease and >83 for very severe disease; FAS 2019 mod: 0-12 for remission, 13-20 for mild disease, 21-28 for moderate disease, 29-33 for severe disease and >33 for very severe disease; PDS: 0-5 for remission, 6-15 for mild disease, 16-20 for moderate disease, 21-25 for severe disease and >25 for very severe disease. Conclusions: Disease severity cut-offs can represent an important improvement in interpreting FM.
Published: 2021

24. SARS-CoV-2 infection in patients with primary Sjögren syndrome: Characterization and outcomes of 51 patients

Author: Medicina i Cirurgia, Universitat Rovira i Virgili, Brito-Zerón P; Brito-Zerón P; Melchor S; Seror R; Seror R; Priori R; Priori R; Solans R; Solans R; Kostov B; Kostov B; Baldini C; Carubbi F; Carubbi F; Callejas JL; Guisado-Vasco P; Hernández-Molina G; Hernández-Molina G; Pasoto SG; Pasoto SG; Valim V; Valim V; Sisó-Almirall A; Sisó-Almirall A; Mariette X; Carreira P; Ramos-Casals M; Brito-Zerón P; Morcillo C; Flores-Chávez A; Acar-Denizli N; Horvath IF; Szanto A; Tarr T; Mandl T; Olsson P; Li X; Xu B; Baldini C; Bombardieri S; Gottenberg JE; Gandolfo S; De Vita S; Giardina F; Sánchez-Guerrero J; Kruize AA; Hinrichs A; Isenberg D; Rischmueller M; Downie-Doyle S; Kwok SK; Park SH; Nordmark G; Suzuki Y; Kawano M; Giacomelli R; Devauchelle-Pensec V; Saraux A; Hofauer B; Knopf A; Bootsma H; Vissink A; Morel J; Vollenveider C; Atzeni F; Retamozo S; Moça Trevisano V; Armagan B; Kilic L; Kalyoncu U; Consani-Fernández S; Callejas JL; López-Dupla M; Pérez-Alvarez R; Akasbi M; Sánchez I, Medicina i Cirurgia, Universitat Rovira i Virgili, and Brito-Zerón P; Brito-Zerón P; Melchor S; Seror R; Seror R; Priori R; Priori R; Solans R; Solans R; Kostov B; Kostov B; Baldini C; Carubbi F; Carubbi F; Callejas JL; Guisado-Vasco P; Hernández-Molina G; Hernández-Molina G; Pasoto SG; Pasoto SG; Valim V; Valim V; Sisó-Almirall A; Sisó-Almirall A; Mariette X; Carreira P; Ramos-Casals M; Brito-Zerón P; Morcillo C; Flores-Chávez A; Acar-Denizli N; Horvath IF; Szanto A; Tarr T; Mandl T; Olsson P; Li X; Xu B; Baldini C; Bombardieri S; Gottenberg JE; Gandolfo S; De Vita S; Giardina F; Sánchez-Guerrero J; Kruize AA; Hinrichs A; Isenberg D; Rischmueller M; Downie-Doyle S; Kwok SK; Park SH; Nordmark G; Suzuki Y; Kawano M; Giacomelli R; Devauchelle-Pensec V; Saraux A; Hofauer B; Knopf A; Bootsma H; Vissink A; Morel J; Vollenveider C; Atzeni F; Retamozo S; Moça Trevisano V; Armagan B; Kilic L; Kalyoncu U; Consani-Fernández S; Callejas JL; López-Dupla M; Pérez-Alvarez R; Akasbi M; Sánchez I
Abstract: Objective: To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. Methods: We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. Results: A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. Conclusion: Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without co
Published: 2021

25. Influence of the age at diagnosis in the disease expression of primary Sjögren's syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium

Author: Universitat Rovira i Virgili, Retamozo S; Acar-Denizli N; Horváth IF; Ng WF; Rasmussen A; Dong X; Li X; Baldini C; Olsson P; Priori R; Seror R; Gottenberg JE; Kruize AA; Hernandez-Molina G; Vissink A; Sandhya P; Armagan B; Quartuccio L; Sebastian A; Praprotnik S; Bartoloni E; Kwok SK; Kvarnstrom M; Rischmueller M; Soláns-Laqué R; Sene D; Pasoto SG; Suzuki Y; Isenberg DA; Valim V; Nordmark G; Nakamura H; Trevisani VFM; Hofauer B; Sisó-Almirall A; Giacomelli R; Devauchelle-Pensec V; Bombardieri M; Atzeni F; Hammenfors D; Maure B; Carsons SE; Gheita T; Sánchez-Berná I; López-Dupla M; Morel J; Inanç N; Fonseca-Aizpuru E; Morcillo C; Vollenweider C; Melchor S; Vázquez M; Díaz-Cuiza E; Consani-Fernández S; De-Miguel-Campo B; Szántó A; Bombardieri S; Gattamelata A; Hinrichs A; Sánchez-Guerrero J; Danda D; Kilic L; De Vita S; Wiland P; Gerli R; Park SH; Wahren-Herlenius M; Bootsma H; Mariette X; Ramos-Casals M; Brito-Zerón P, Universitat Rovira i Virgili, and Retamozo S; Acar-Denizli N; Horváth IF; Ng WF; Rasmussen A; Dong X; Li X; Baldini C; Olsson P; Priori R; Seror R; Gottenberg JE; Kruize AA; Hernandez-Molina G; Vissink A; Sandhya P; Armagan B; Quartuccio L; Sebastian A; Praprotnik S; Bartoloni E; Kwok SK; Kvarnstrom M; Rischmueller M; Soláns-Laqué R; Sene D; Pasoto SG; Suzuki Y; Isenberg DA; Valim V; Nordmark G; Nakamura H; Trevisani VFM; Hofauer B; Sisó-Almirall A; Giacomelli R; Devauchelle-Pensec V; Bombardieri M; Atzeni F; Hammenfors D; Maure B; Carsons SE; Gheita T; Sánchez-Berná I; López-Dupla M; Morel J; Inanç N; Fonseca-Aizpuru E; Morcillo C; Vollenweider C; Melchor S; Vázquez M; Díaz-Cuiza E; Consani-Fernández S; De-Miguel-Campo B; Szántó A; Bombardieri S; Gattamelata A; Hinrichs A; Sánchez-Guerrero J; Danda D; Kilic L; De Vita S; Wiland P; Gerli R; Park SH; Wahren-Herlenius M; Bootsma H; Mariette X; Ramos-Casals M; Brito-Zerón P
Abstract: Objective. To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren's syndrome (pSS). Methods. By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results. There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R2 0.87) and the frequency of abnormal oral tests (adjusted R2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.
Published: 2021

26. This title is unavailable for guests, please login to see more information.

Author: Gaggiano, C, Vitale, A, Obici, L, Merlini, G, Soriano, A, Viapiana, O, Cattalini, M, Maggio, Mc, Lopalco, G, Montin, D, Jaber, Ma, Dagna, L, Manna, Raffaele, Insalaco, A, Piga, M, La Torre, F, Berlengiero, V, Gelardi, V, Ciarcia, L, Emmi, G, Ruscitti, P, Caso, F, Cimaz, R, Hernàndez-Rodriguez, J, Parronchi, P, Sicignano, Ludovico Luca, Verrecchia, E, Iannone, F, Sota, J, Grosso, S, Salvarani, C, Frediani, B, Giacomelli, R, Mencarelli, Ma, Renieri, A, Rigante, Donato, Cantarini, L, Manna R (ORCID:0000-0003-1560-3907), Sicignano LL, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Vitale, A, Obici, L, Merlini, G, Soriano, A, Viapiana, O, Cattalini, M, Maggio, Mc, Lopalco, G, Montin, D, Jaber, Ma, Dagna, L, Manna, Raffaele, Insalaco, A, Piga, M, La Torre, F, Berlengiero, V, Gelardi, V, Ciarcia, L, Emmi, G, Ruscitti, P, Caso, F, Cimaz, R, Hernàndez-Rodriguez, J, Parronchi, P, Sicignano, Ludovico Luca, Verrecchia, E, Iannone, F, Sota, J, Grosso, S, Salvarani, C, Frediani, B, Giacomelli, R, Mencarelli, Ma, Renieri, A, Rigante, Donato, Cantarini, L, Manna R (ORCID:0000-0003-1560-3907), Sicignano LL, and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: This study explores demographic, clinical, and therapeutic features of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) in a cohort of 80 patients recruited from 19 Italian referral Centers. Patients’ data were collected retrospectively and then analyzed according to age groups (disease onset before or after 16 years) and genotype (high penetrance (HP) and low penetrance (LP) TNFRSF1A gene variants). Pediatric- and adult-onset were reported, respectively, in 44 and 36 patients; HP and LP variants were found, respectively, in 32 and 44 cases. A positive family history for recurrent fever was reported more frequently in the pediatric group than in the adult group (p < 0:05). With reference to clinical features during attacks, pericarditis and myalgia were reported more frequently in the context of adult-onset disease than in the pediatric age (with p < 0:01 and p < 0:05, respectively), while abdominal pain was present in 84% of children and in 25% of adults (p < 0:01). Abdominal pain was significantly associated also to the presence of HP mutations (p < 0:01), while oral aphthosis was more frequently found in the LP variant group (p < 0:05). Systemic amyloidosis occurred in 25% of subjects carrying HP variants. As concerns laboratory features, HP mutations were significantly associated to higher ESR values (p < 0:01) and to the persistence of steadily elevated inflammatory markers during asymptomatic periods (p < 0:05). The presence of mutations involving a cysteine residue, abdominal pain, and lymphadenopathy during flares significantly correlated with the risk of developing amyloidosis and renal impairment. Conversely, the administration of colchicine negatively correlated to the development of pathologic proteinuria (p < 0:05). Both NSAIDs and colchicine were used as monotherapy more frequently in the LP group compared to the HP group (p < 0:01). Biologic agents were prescribed to 49 (61%) patients; R92Q subjects wer
Published: 2020

27. THYMIC STROMAL LYMPHOPOIETIN (TSLP) AS A BIOMARKER OF PRIMARY SJOGREN'S SYNDROME (PSS) AND RELATED LYMPHOMA: VALIDATION IN INDEPENDENT COHORTS

Author: Gandolfo, S. Fabro, C. Colafrancesco, S. Carubbi, F. and Ferro, F. Bocci, E. Bartoloni Kapsogeorgou, E. Goules, A. and Quartuccio, L. Priori, R. Alunno, A. Valesini, G. and Giacomelli, R. Gerli, R. Baldini, C. Tzioufas, A. De Vita, S. and Gandolfo, S. Fabro, C. Colafrancesco, S. Carubbi, F. and Ferro, F. Bocci, E. Bartoloni Kapsogeorgou, E. Goules, A. and Quartuccio, L. Priori, R. Alunno, A. Valesini, G. and Giacomelli, R. Gerli, R. Baldini, C. Tzioufas, A. De Vita, S.
Published: 2020

28. Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies

Author: Acar-Denizli, N., Horvath, I. -F, Mandl, T., Priori, R., Vissink, A., Hernandez-Molina, G., Armagan, B., Praprotnik, S., Sebastian, A., Bartoloni, E., Rischmueller, M., Pasoto, S. G., Nordmark, Gunnel, Nakamura, H., Fernandes Moca Trevisani, V., Retamozo, S., Carsons, S. E., Maure-Noia, B., Sanchez-Berna, I., Lopez-Dupla, M., Fonseca-Aizpuru, E., Melchor Diaz, S., Vazquez, M., Diaz Cuiza, P. E., de Miguel Campo, B., Ng, W. -F, Rasmussen, A., Dong, X., Li, X., Baldini, C., Seror, R., Gottenberg, J. -E, Kruize, A. A., Sandhya, P., Gandolfo, S., Kwok, S. -K, Kvarnstrom, M., Solans, R., Sene, D., Suzuki, Y., Isenberg, D. A., Valim, V., Hofauer, B., Giacomelli, R., Devauchelle-Pensec, V., Atzeni, F., Gheita, T. A., Morel, J., Izzo, R., Kalyoncu, U., Szanto, A., Olsson, P., Bootsma, H., Ramos-Casals, M., Kostov, B., Brito-Zeron, P., Acar-Denizli, N., Horvath, I. -F, Mandl, T., Priori, R., Vissink, A., Hernandez-Molina, G., Armagan, B., Praprotnik, S., Sebastian, A., Bartoloni, E., Rischmueller, M., Pasoto, S. G., Nordmark, Gunnel, Nakamura, H., Fernandes Moca Trevisani, V., Retamozo, S., Carsons, S. E., Maure-Noia, B., Sanchez-Berna, I., Lopez-Dupla, M., Fonseca-Aizpuru, E., Melchor Diaz, S., Vazquez, M., Diaz Cuiza, P. E., de Miguel Campo, B., Ng, W. -F, Rasmussen, A., Dong, X., Li, X., Baldini, C., Seror, R., Gottenberg, J. -E, Kruize, A. A., Sandhya, P., Gandolfo, S., Kwok, S. -K, Kvarnstrom, M., Solans, R., Sene, D., Suzuki, Y., Isenberg, D. A., Valim, V., Hofauer, B., Giacomelli, R., Devauchelle-Pensec, V., Atzeni, F., Gheita, T. A., Morel, J., Izzo, R., Kalyoncu, U., Szanto, A., Olsson, P., Bootsma, H., Ramos-Casals, M., Kostov, B., and Brito-Zeron, P.
Abstract: Objective: To evaluate the systemic phenotype associated with the presence of isolated anti-La/SSB antibodies in a large international registry of patients with primary Sjogren's syndrome (pSS) fulfilling the 2002 classification criteria. Methods: The Big Data Sjogren Project Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centres on clinical research in SS of the 5 continents was collected. Combination patterns of anti-Ro/SSA-La/SSB antibodies at the time of diagnosis defined the following four immunological phenotypes: double positive (combined Ro/SSA and La/SSB,) isolated anti-Ro/SSA, isolated anti-La/SSB, and immunonegative. Results: The cohort included 12,084 patients (11,293 females, mean 52.4 years) with recorded ESSDAI scores available. Among them, 279 (2.3%) had isolated anti-La/SSB antibodies. The mean total ESSDAI score at diagnosis of patients with pSS carrying isolated anti-La/SSB was 6.0, and 80.4% of patients had systemic activity (global ESSDAI score >= 1) at diagnosis. The domains with the highest frequency of active patients were the biological (42.8%), glandular (36.8%) and articular (31.2%) domains. Patients with isolated anti-La/ SSB showed a higher frequency of active patients in all ESSDAI domains but two (articular and peripheral nerve) in comparison with immune-negative patients, and even a higher absolute frequency in six clinical ESSDAI domains in comparison with patients with isolated anti-Ro/SSA. In addition, patients with isolated anti-La/SSB showed a higher frequency of active patients in two ESSDAI domains (pulmonary and glandular) with respect to the most active immunological subset (double-positive antibodies). Meanwhile, systemic activity detected in patients with isolated anti-La/SSB was overwhelmingly low. Even in ESSDAI domains where patients with isolated anti-La/SSB had the highest frequencies of systemic activity (lymphadenopathy and muscular), the percentage
Published: 2020

29. 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

30. Guidelines for biomarkers in autoimmune rheumatic diseases - evidence based analysis

Author: Giacomelli, R. Afeltra, A. Alunno, A. Bartoloni-Bocci, E. Berardicurti, O. Bombardieri, M. Bortoluzzi, A. Caporali, R. Caso, F. Cervera, R. Chimenti, M.S. Cipriani, P. Coloma, E. Conti, F. D'Angelo, S. De Vita, S. Di Bartolomeo, S. Distler, O. Doria, A. Feist, E. Fisher, B.A. Gerosa, M. Gilio, M. Guggino, G. Liakouli, V. Margiotta, D.P.E. Meroni, P. Moroncini, G. Perosa, F. Prete, M. Priori, R. Rebuffi, C. Ruscitti, P. Scarpa, R. Shoenfeld, Y. Todoerti, M. Ursini, F. Valesini, G. Vettori, S. Vitali, C. Tzioufas, A.G. and Giacomelli, R. Afeltra, A. Alunno, A. Bartoloni-Bocci, E. Berardicurti, O. Bombardieri, M. Bortoluzzi, A. Caporali, R. Caso, F. Cervera, R. Chimenti, M.S. Cipriani, P. Coloma, E. Conti, F. D'Angelo, S. De Vita, S. Di Bartolomeo, S. Distler, O. Doria, A. Feist, E. Fisher, B.A. Gerosa, M. Gilio, M. Guggino, G. Liakouli, V. Margiotta, D.P.E. Meroni, P. Moroncini, G. Perosa, F. Prete, M. Priori, R. Rebuffi, C. Ruscitti, P. Scarpa, R. Shoenfeld, Y. Todoerti, M. Ursini, F. Valesini, G. Vettori, S. Vitali, C. Tzioufas, A.G.
Abstract: Autoimmune rheumatic diseases are characterised by an abnormal immune system response, complement activation, cytokines dysregulation and inflammation. In last years, despite many progresses in managing these patients, it has been shown that clinical remission is reached in less than 50% of patients and a personalised and tailored therapeutic approach is still lacking resulting in a significant gap between guidelines and real-world practice. In this context, the need for biomarkers facilitating early diagnosis and profiling those individuals at the highest risk for a poor outcome has become of crucial interest. A biomarker generally refers to a measured characteristic which may be used as an indicator of some biological state or condition. Three different types of medical biomarkers has been suggested: i. mechanistic markers; ii. clinical disease markers; iii. therapeutic markers. A combination of biomarkers from these different groups could be used for an ideal more accurate diagnosis and treatment. However, although a growing body of evidence is focused on improving biomarkers, a significant amount of this information is not integrated on standard clinical care. The overarching aim of this work was to clarify the meaning of specific biomarkers during autoimmune diseases; their possible role in confirming diagnosis, predicting outcome and suggesting specific treatments. © 2018
Published: 2019

31. Guidelines for biomarkers in autoimmune rheumatic diseases - evidence based analysis

Author: Giacomelli, R. Afeltra, A. Alunno, A. Bartoloni-Bocci, E. Berardicurti, O. Bombardieri, M. Bortoluzzi, A. Caporali, R. Caso, F. Cervera, R. Chimenti, M.S. Cipriani, P. Coloma, E. Conti, F. D'Angelo, S. De Vita, S. Di Bartolomeo, S. Distler, O. Doria, A. Feist, E. Fisher, B.A. Gerosa, M. Gilio, M. Guggino, G. Liakouli, V. Margiotta, D.P.E. Meroni, P. Moroncini, G. Perosa, F. Prete, M. Priori, R. Rebuffi, C. Ruscitti, P. Scarpa, R. Shoenfeld, Y. Todoerti, M. Ursini, F. Valesini, G. Vettori, S. Vitali, C. Tzioufas, A.G. and Giacomelli, R. Afeltra, A. Alunno, A. Bartoloni-Bocci, E. Berardicurti, O. Bombardieri, M. Bortoluzzi, A. Caporali, R. Caso, F. Cervera, R. Chimenti, M.S. Cipriani, P. Coloma, E. Conti, F. D'Angelo, S. De Vita, S. Di Bartolomeo, S. Distler, O. Doria, A. Feist, E. Fisher, B.A. Gerosa, M. Gilio, M. Guggino, G. Liakouli, V. Margiotta, D.P.E. Meroni, P. Moroncini, G. Perosa, F. Prete, M. Priori, R. Rebuffi, C. Ruscitti, P. Scarpa, R. Shoenfeld, Y. Todoerti, M. Ursini, F. Valesini, G. Vettori, S. Vitali, C. Tzioufas, A.G.
Abstract: Autoimmune rheumatic diseases are characterised by an abnormal immune system response, complement activation, cytokines dysregulation and inflammation. In last years, despite many progresses in managing these patients, it has been shown that clinical remission is reached in less than 50% of patients and a personalised and tailored therapeutic approach is still lacking resulting in a significant gap between guidelines and real-world practice. In this context, the need for biomarkers facilitating early diagnosis and profiling those individuals at the highest risk for a poor outcome has become of crucial interest. A biomarker generally refers to a measured characteristic which may be used as an indicator of some biological state or condition. Three different types of medical biomarkers has been suggested: i. mechanistic markers; ii. clinical disease markers; iii. therapeutic markers. A combination of biomarkers from these different groups could be used for an ideal more accurate diagnosis and treatment. However, although a growing body of evidence is focused on improving biomarkers, a significant amount of this information is not integrated on standard clinical care. The overarching aim of this work was to clarify the meaning of specific biomarkers during autoimmune diseases; their possible role in confirming diagnosis, predicting outcome and suggesting specific treatments. © 2018
Published: 2019

32. Systemic manifestations of primary Sjogren's syndrome out of the ESSDAI classification : prevalence and clinical relevance in a large international, multi-ethnic cohort of patients

Author: Retamozo, S., Acar-Denizli, N., Rasmussen, A., Horvath, I. F., Baldini, C., Priori, R., Sandhya, P., Hernandez-Molina, G., Armagan, B., Praprotnik, S., Kvarnstrom, M., Gerli, R., Sebastian, A., Solans, R., Rischmueller, M., Pasoto, S. G., Valim, V., Nordmark, Gunnel, Kruize, A. A., Nakamura, H., Hofauer, B., Giacomelli, R., Fernandes Moca Trevisani, V., Devauchelle-Pensec, V., Atzeni, F., Gheita, T. A., Consani-Fernandez, S., Szanto, A., Sivils, K., Gattamelata, A., Danda, D., Kilic, L., Bartoloni, E., Bombardieri, S., Sanchez-Guerrero, J., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Retamozo, S., Acar-Denizli, N., Rasmussen, A., Horvath, I. F., Baldini, C., Priori, R., Sandhya, P., Hernandez-Molina, G., Armagan, B., Praprotnik, S., Kvarnstrom, M., Gerli, R., Sebastian, A., Solans, R., Rischmueller, M., Pasoto, S. G., Valim, V., Nordmark, Gunnel, Kruize, A. A., Nakamura, H., Hofauer, B., Giacomelli, R., Fernandes Moca Trevisani, V., Devauchelle-Pensec, V., Atzeni, F., Gheita, T. A., Consani-Fernandez, S., Szanto, A., Sivils, K., Gattamelata, A., Danda, D., Kilic, L., Bartoloni, E., Bombardieri, S., Sanchez-Guerrero, J., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., and Brito-Zeron, P.
Abstract: Objectives: To analyse the frequency and characterise the systemic presentation of primary Sjogren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. Methods: The Big Data Sjogren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. Results: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). Conclusions: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular
Published: 2019

33. Anakinra drug retention rate and predictive factors of drug survival in systemic juvenile idiopathic arthritis and adult onset Still’s disease

Author: Sota, J, Rigante, Donato, Ruscitti, P, Insalaco, A, Sfriso, P, de Vita, S, Cimaz, R, Lopalco, G, Emmi, G, La Torre, F, Fabiani, C, Olivieri, An, Cattalini, M, Cammelli, D, Gallizzi, R, Alessio, M, Manna, Raffaele, Viapiana, O, Frassi, M, Maier, A, Salvarani, C, Talarico, R, Priori, R, Maggio, Mc, Vitale, A, Giacomelli, R, Cantarini L for the Working Group of Systemic AutoInflammatory Diseases of, S. I. R., Rigante D (ORCID:0000-0001-7032-7779), Manna R (ORCID:0000-0003-1560-3907), Sota, J, Rigante, Donato, Ruscitti, P, Insalaco, A, Sfriso, P, de Vita, S, Cimaz, R, Lopalco, G, Emmi, G, La Torre, F, Fabiani, C, Olivieri, An, Cattalini, M, Cammelli, D, Gallizzi, R, Alessio, M, Manna, Raffaele, Viapiana, O, Frassi, M, Maier, A, Salvarani, C, Talarico, R, Priori, R, Maggio, Mc, Vitale, A, Giacomelli, R, Cantarini L for the Working Group of Systemic AutoInflammatory Diseases of, S. I. R., Rigante D (ORCID:0000-0001-7032-7779), and Manna R (ORCID:0000-0003-1560-3907)
Abstract: Background and Objective: Only a few studies have reported long-term efficacy of interleukin (IL)-1 inhibition in systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still disease (AOSD). Herein we report on the effectiveness of anakinra (ANA), expressed in terms of drug retention rate (DRR), and evaluate the predictive factors of drug survival in a cohort of patients with sJIA and AOSD. Patients and Methods: This is a multicenter study reviewing retrospectively the medical records from 61 patients with sJIA and 76 with AOSD, all treated with ANA in 25 Italian tertiary referral centers. Results: The cumulative retention rate of ANA at 12-, 24-, 48-, and 60-month of follow-up was 74.3%, 62.9%, 49.4%, and 49.4%, respectively, without any significant differences between sJIA and AOSD patients (p = 0.164), and between patients treated in monotherapy compared with the subgroup coadministered with conventional disease-modifying antirheumatic drugs (cDMARDs) (p = 0.473). On the other hand, a significant difference in DRR was found between biologic-naïve patients and those previously treated with biotechnologic drugs (p = 0.009), which persisted even after adjustment for pathology (p = 0.013). In the regression analysis, patients experiencing adverse events (AEs) {hazards ratio (HR) = 3.029 [confidence interval (CI) 1.750-5.242], p < 0.0001} and those previously treated with other biologic agents [HR = 1.818 (CI 1.007-3.282), p = 0.047] were associated with a higher HR of ANA discontinuation. The median treatment delay was significantly higher among patients discontinuing ANA (p < 0.0001). Significant corticosteroid-sparing (p = 0.033) and cDMARD-sparing effects (p < 0.0001) were also recorded. Less than one-third of our cohort developed AEs, and 85% were deemed mild in nature, with 70% of them involving the skin. Conclusions: Our findings display an overall excellent DRR of ANA on the long run for both sJIA and AOSD, that may be further optimized by clo
Published: 2019

34. 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

35. 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

36. A North-South Worldwide Gradient in Systemic Activity of Primary Sjögren Syndrome : Increased Severe Disease in Patients from Southern Countries

Author: Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandi, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., Ramos-Casals, M., Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandi, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., and Ramos-Casals, M.
Published: 2018
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37. Influence of epidemiology and ethnicity on systemic expression of primary Sjögren syndrome in 9974 patients

Author: Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandl, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensee, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., Ramos-Casals, M., Retamozo, S., Acar-Denizli, N., Ng, W. Fai, Zeher, M., Rasmussen, A., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Wahren-Herlenius, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Mandl, T., Suzuki, Y., Isenberg, D., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensee, V., Hofauer, B., Bombardieri, M., Fernandes Moca Trevisani, V., Hammenfors, D., Pasoto, S. G., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Brito-Zeron, P., and Ramos-Casals, M.
Published: 2018
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38. How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis : analysis of 10,500 patients (Sjögren Big Data Project)

Author: Brito-Zeron, P., Acar-Denizli, N., Ng, W. F., Zeher, M., Rasmussen, A., Mandl, T., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Suzuki, Y., Isenberg, D. A., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensec, V., Knopf, A., Bombardieri, M., Trevisani, V. -F, Hammenfors, D., Pasoto, S. G., Retamozo, S., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Horvath, I-F, Sivils, K. L., Olsson, P., De Vita, S., Sanchez-Guerrero, J., Kilic, L., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Ng, W. F., Zeher, M., Rasmussen, A., Mandl, T., Seror, R., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Armagan, B., Kruize, A. A., Kwok, S. -K, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Rischmueller, M., Suzuki, Y., Isenberg, D. A., Valim, V., Wiland, P., Nordmark, Gunnel, Fraile, G., Bootsma, H., Nakamura, T., Giacomelli, R., Devauchelle-Pensec, V., Knopf, A., Bombardieri, M., Trevisani, V. -F, Hammenfors, D., Pasoto, S. G., Retamozo, S., Gheita, T. A., Atzeni, F., Morel, J., Vollenveider, C., Horvath, I-F, Sivils, K. L., Olsson, P., De Vita, S., Sanchez-Guerrero, J., Kilic, L., Wahren-Herlenius, M., Mariette, X., and Ramos-Casals, M.
Abstract: Objective: To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjogren's syndrome (SjS). Methods: The Big Data Sjogren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays. Results: By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti-La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglobulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for ctyoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p<0.001) in the organ-by-organ ESSDAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains). Conclusion: We confirm the strong influence of immunological markers on the phenotype of primary SjS at diagnosis in the largest multi-ethnic international cohort ever analysed, with a greater inf
Published: 2018

39. Patients’, physicians’, nurses’, and pharmacists’ preferences on the characteristics of biologic agents used in the treatment of rheumatic diseases

Author: Scalone, L, Sarzi-Puttini, P, Sinigaglia, L, Montecucco, C, Giacomelli, R, Lapadula, G, Olivieri, I, Giardino, A, Cortesi, P, Mantovani, L, Mecchia, M, Scalone, Luciana, Sarzi-Puttini, Piercarlo, Sinigaglia, Luigi, Montecucco, Carlomaurizio, Giacomelli, Roberto, Lapadula, Giovanni, Olivieri, Ignazio, Giardino, Angela Maria, Cortesi, Paolo Angelo, Mantovani, Lorenzo Giovanni, Mecchia, Monica, Scalone, L, Sarzi-Puttini, P, Sinigaglia, L, Montecucco, C, Giacomelli, R, Lapadula, G, Olivieri, I, Giardino, A, Cortesi, P, Mantovani, L, Mecchia, M, Scalone, Luciana, Sarzi-Puttini, Piercarlo, Sinigaglia, Luigi, Montecucco, Carlomaurizio, Giacomelli, Roberto, Lapadula, Giovanni, Olivieri, Ignazio, Giardino, Angela Maria, Cortesi, Paolo Angelo, Mantovani, Lorenzo Giovanni, and Mecchia, Monica
Abstract: Objective: To estimate preferences in relevant treatment characteristics evaluated by different groups involved in the management of patients with rheumatic diseases. Subjects and methods: We surveyed patients with rheumatic diseases, and rheumatologists, nurses, and pharmacists with experience in treatment with/provision of biologic drugs for these patients. Through a discrete choice experiment, participants evaluated 16 possible scenarios in which pairs of similarly efficacious treatments were described with six characteristics: 1) frequency of administration; 2) mode and place of administration; 3) manner, helpfulness, efficiency, and courtesy of health personnel; 4) frequency of reactions at the site of drug administration; 5) severity of generalized undesired/allergic reactions; and 6) additional cost. The direction and strength of preferences toward each characteristic level and the relative importance of each characteristic were estimated through a random-effects conditional logistic regression model. Results: In total, 513 patients, 110 rheumatologists, 51 nurses, and 46 pharmacists from 30 centers in Italy participated. Characteristics 3, 4, and 6 were the most important for every subgroup; 1 was least important for patients and rheumatologists, 2 was least important for pharmacists, and 2 and 5 were least important for nurses. For characteristic 2, pharmacists preferred subcutaneous self-injection with a syringe; nurses preferred assisted infusion at an infusion center close to the patient’s home; patients and rheumatologists preferred subcutaneous self-injection with a pen. Conclusion: The different preferences for some characteristics shown by the different groups can play an important role, together with purely clinical aspects, in the choice and consequent benefit of treatments, contributing also to a more satisfactory use of resources
Published: 2018

40. Patients’, physicians’, nurses’, and pharmacists’ preferences on the characteristics of biologic agents used in the treatment of rheumatic diseases

Author: Scalone, L, Sarzi-Puttini, P, Sinigaglia, L, Montecucco, C, Giacomelli, R, Lapadula, G, Olivieri, I, Giardino, A, Cortesi, P, Mantovani, L, Mecchia, M, Scalone, Luciana, Sarzi-Puttini, Piercarlo, Sinigaglia, Luigi, Montecucco, Carlomaurizio, Giacomelli, Roberto, Lapadula, Giovanni, Olivieri, Ignazio, Giardino, Angela Maria, Cortesi, Paolo Angelo, Mantovani, Lorenzo Giovanni, Mecchia, Monica, Scalone, L, Sarzi-Puttini, P, Sinigaglia, L, Montecucco, C, Giacomelli, R, Lapadula, G, Olivieri, I, Giardino, A, Cortesi, P, Mantovani, L, Mecchia, M, Scalone, Luciana, Sarzi-Puttini, Piercarlo, Sinigaglia, Luigi, Montecucco, Carlomaurizio, Giacomelli, Roberto, Lapadula, Giovanni, Olivieri, Ignazio, Giardino, Angela Maria, Cortesi, Paolo Angelo, Mantovani, Lorenzo Giovanni, and Mecchia, Monica
Abstract: Objective: To estimate preferences in relevant treatment characteristics evaluated by different groups involved in the management of patients with rheumatic diseases. Subjects and methods: We surveyed patients with rheumatic diseases, and rheumatologists, nurses, and pharmacists with experience in treatment with/provision of biologic drugs for these patients. Through a discrete choice experiment, participants evaluated 16 possible scenarios in which pairs of similarly efficacious treatments were described with six characteristics: 1) frequency of administration; 2) mode and place of administration; 3) manner, helpfulness, efficiency, and courtesy of health personnel; 4) frequency of reactions at the site of drug administration; 5) severity of generalized undesired/allergic reactions; and 6) additional cost. The direction and strength of preferences toward each characteristic level and the relative importance of each characteristic were estimated through a random-effects conditional logistic regression model. Results: In total, 513 patients, 110 rheumatologists, 51 nurses, and 46 pharmacists from 30 centers in Italy participated. Characteristics 3, 4, and 6 were the most important for every subgroup; 1 was least important for patients and rheumatologists, 2 was least important for pharmacists, and 2 and 5 were least important for nurses. For characteristic 2, pharmacists preferred subcutaneous self-injection with a syringe; nurses preferred assisted infusion at an infusion center close to the patient’s home; patients and rheumatologists preferred subcutaneous self-injection with a pen. Conclusion: The different preferences for some characteristics shown by the different groups can play an important role, together with purely clinical aspects, in the choice and consequent benefit of treatments, contributing also to a more satisfactory use of resources
Published: 2018

41. International consensus: What else can we do to improve diagnosis and therapeutic strategies in patients affected by autoimmune rheumatic diseases (rheumatoid arthritis, spondyloarthritides, systemic sclerosis, systemic lupus erythematosus, antiphospholipid syndrome and Sjogren's syndrome)?: The unmet needs and the clinical grey zone in autoimmune disease management

Author: Giacomelli, R. Afeltra, A. Alunno, A. Baldini, C. Bartoloni-Bocci, E. Berardicurti, O. Carubbi, F. Cauli, A. Cervera, R. Ciccia, F. Cipriani, P. Conti, F. De Vita, S. Di Benedetto, P. Doria, A. Drosos, A.A. Favalli, E.G. Gandolfo, S. Gatto, M. Grembiale, R.D. Liakouli, V. Lories, R. Lubrano, E. Lunardi, C. Margiotta, D.P.E. Massaro, L. Meroni, P. Minniti, A. Navarini, L. Pendolino, M. Perosa, F. Pers, J.-O. Prete, M. Priori, R. Puppo, F. Quartuccio, L. Ruffatti, A. Ruscitti, P. Russo, B. Sarzi-Puttini, P. Shoenfeld, Y. Somarakis, G.A. Spinelli, F.R. Tinazzi, E. Triolo, G. Ursini, F. Valentini, G. Valesini, G. Vettori, S. Vitali, C. Tzioufas, A.G. and Giacomelli, R. Afeltra, A. Alunno, A. Baldini, C. Bartoloni-Bocci, E. Berardicurti, O. Carubbi, F. Cauli, A. Cervera, R. Ciccia, F. Cipriani, P. Conti, F. De Vita, S. Di Benedetto, P. Doria, A. Drosos, A.A. Favalli, E.G. Gandolfo, S. Gatto, M. Grembiale, R.D. Liakouli, V. Lories, R. Lubrano, E. Lunardi, C. Margiotta, D.P.E. Massaro, L. Meroni, P. Minniti, A. Navarini, L. Pendolino, M. Perosa, F. Pers, J.-O. Prete, M. Priori, R. Puppo, F. Quartuccio, L. Ruffatti, A. Ruscitti, P. Russo, B. Sarzi-Puttini, P. Shoenfeld, Y. Somarakis, G.A. Spinelli, F.R. Tinazzi, E. Triolo, G. Ursini, F. Valentini, G. Valesini, G. Vettori, S. Vitali, C. Tzioufas, A.G.
Abstract: Autoimmune diseases are a complex set of diseases characterized by immune system activation and, although many progresses have been done in the last 15 years, several unmet needs in the management of these patients may be still identified. Recently, a panel of international Experts, divided in different working groups according to their clinical and scientific expertise, were asked to identify, debate and formulate a list of key unmet needs within the field of rheumatology, serving as a roadmap for research as well as support for clinicians. After a systematic review of the literature, the results and the discussions from each working group were summarised in different statements. Due to the differences among the diseases and their heterogeneity, a large number of statements was produced and voted by the Experts to reach a consensus in a plenary session. At all the steps of this process, including the initial discussions by the steering committee, the identification of the unmet needs, the expansion of the working group and finally the development of statements, a large agreement was attained. This work confirmed that several unmet needs may be identified and despite the development of new therapeutic strategies as well as a better understanding of the effects of existing therapies, many open questions still remain in this field, suggesting a research agenda for the future and specific clinical suggestions which may allow physicians to better manage those clinical conditions still lacking of scientific clarity. © 2017
Published: 2017

42. Analysis Of 9302 Patients From The Big Data International Primary Sjogren Syndrome Cohort : Clinical Presentation At Diagnosis Of European Vs Non-European Patients

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Priori, R., Quartuccio, L., Hernandez-Molina, G., Kruize, A., Park, S. -H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Suzuki, Y., Isenberg, D., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Vissink, A., Nakamura, T., Valim, V., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Carsons, S. E., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Priori, R., Quartuccio, L., Hernandez-Molina, G., Kruize, A., Park, S. -H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Suzuki, Y., Isenberg, D., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Vissink, A., Nakamura, T., Valim, V., Giacomelli, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Carsons, S. E., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., and Ramos-Casals, M.
Published: 2017
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43. Baseline Essdai/ Das Scores In 8061 Patients With Primary Sjögren Syndrome : Characterization Of Systemic Disease

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Li, X., Baldini, C., Gottenberg, J-E, Danda, D., Quartuccio, L., Hernandez-Molina, G., Kruize, A. A., Park, S-H, Kvarnstrom, M., Praprotnik, S., Sene, D., Alunno, A., Solans, R., Mandl, T., Suzuki, Y., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Wiland, P., Bootsma, H., Nakamura, T., Valim, V., Giacomelli, R., Seror, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Minniti, A., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Li, X., Baldini, C., Gottenberg, J-E, Danda, D., Quartuccio, L., Hernandez-Molina, G., Kruize, A. A., Park, S-H, Kvarnstrom, M., Praprotnik, S., Sene, D., Alunno, A., Solans, R., Mandl, T., Suzuki, Y., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Wiland, P., Bootsma, H., Nakamura, T., Valim, V., Giacomelli, R., Seror, R., Devauchelle-Pensec, V., Hofauer, B., Bombardieri, M., Trevisani, V., Hammenfors, D., Minniti, A., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., and Ramos-Casals, M.
Published: 2017
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44. Predicting Survival In 6240 Patients With Primary Sjögren' Syndrome (Big Data Sjögren Project)

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Li, X., Baldini, C., Gottenberg, J-E, Danda, D., Quartuccio, L., Hernandez-Molina, G., Kruize, A. A., Park, S-H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Mandl, T., Suzuki, Y., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Bootsma, H., Nakamura, T., Valim, V., Giacomelli, R., Seror, R., Devauchelle-Pensec, V., Hofauer, B., Bombaidieri, M., Trevisani, V., Hammenfors, D., Priori, R., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Li, X., Baldini, C., Gottenberg, J-E, Danda, D., Quartuccio, L., Hernandez-Molina, G., Kruize, A. A., Park, S-H, Kvarnstrom, M., Praprotnik, S., Sene, D., Bartoloni, E., Solans, R., Mandl, T., Suzuki, Y., Rischmueller, M., Nordmark, Gunnel, Fraile, G., Sebastian, A., Bootsma, H., Nakamura, T., Valim, V., Giacomelli, R., Seror, R., Devauchelle-Pensec, V., Hofauer, B., Bombaidieri, M., Trevisani, V., Hammenfors, D., Priori, R., Pasoto, S. G., Morel, J., Retamozo, S., Gheita, T. A., Atzeni, F., Vollenveider, C., Mariette, X., and Ramos-Casals, M.
Published: 2017
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45. Ethnic Differences Strongly Influence The Phenotypic Expression of Primary Sjögren : Study of 7887 Patients from 20 Countries on 5 Continents (EULAR-SS Task Force Big Data Sjögren Project)

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Kruize, A., Valim, V., Kvarnstrom, M., Sene, D., Gerli, R., Praprotnik, S., Isenberg, D., Solans, R., Rischmueller, M., Park, S. -H, Nordmark, Gunnel, Suzuki, Y., Giacomelli, R., Saraux, A., Bombardieri, M., Hofauer, B., Bootsma, H., Hammenfors, D., Fraile, G., Carsons, S., Gheita, T., Morel, J., Vollenveider, C., Atzeni, F., Retamozo, S., Horvath, I. -F, Sivils, K., Theander, E., Sandhya, P., De Vita, S., Sanchez-Guerrero, J., van der Heijden, E., Moca-Trevisano, V., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Kruize, A., Valim, V., Kvarnstrom, M., Sene, D., Gerli, R., Praprotnik, S., Isenberg, D., Solans, R., Rischmueller, M., Park, S. -H, Nordmark, Gunnel, Suzuki, Y., Giacomelli, R., Saraux, A., Bombardieri, M., Hofauer, B., Bootsma, H., Hammenfors, D., Fraile, G., Carsons, S., Gheita, T., Morel, J., Vollenveider, C., Atzeni, F., Retamozo, S., Horvath, I. -F, Sivils, K., Theander, E., Sandhya, P., De Vita, S., Sanchez-Guerrero, J., van der Heijden, E., Moca-Trevisano, V., Wahren-Herlenius, M., Mariette, X., and Ramos-Casals, M.
Published: 2016
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46. Worldwide Heterogeneous Diagnostic Approach To Primary Sjögren Syndrome in 8315 Patients (EULAR-SS Task Force Big Data Sjögren Project)

Author: Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Kruize, A., Valim, V., Kvarnstrom, M., Sene, D., Bartoloni, E., Praprotnik, S., Isenberg, D., Solans, R., Rischmueller, M., Kwok, S. -K, Nordmark, Gunnel, Suzuki, Y., Giacomelli, R., Devauchelle-Pensec, V., Bombardieri, M., Hofauer, B., Bootsma, H., Hammenfors, D., Fraile, G., Carsons, S., Gheita, T., Morel, J., Vollenveider, C., Atzeni, F., Retamozo, S., Horvath, I. -F, Sivils, K., Theander, E., Sandhya, P., De Vita, S., Sanchez-Guerrero, J., van der Heijden, E., Moca-Trevisano, V., Wahren-Herlenius, M., Mariette, X., Ramos-Casals, M., Brito-Zeron, P., Acar-Denizli, N., Zeher, M., Rasmussen, A., Seror, R., Mandl, T., Li, X., Baldini, C., Gottenberg, J. -E, Danda, D., Quartuccio, L., Priori, R., Hernandez-Molina, G., Kruize, A., Valim, V., Kvarnstrom, M., Sene, D., Bartoloni, E., Praprotnik, S., Isenberg, D., Solans, R., Rischmueller, M., Kwok, S. -K, Nordmark, Gunnel, Suzuki, Y., Giacomelli, R., Devauchelle-Pensec, V., Bombardieri, M., Hofauer, B., Bootsma, H., Hammenfors, D., Fraile, G., Carsons, S., Gheita, T., Morel, J., Vollenveider, C., Atzeni, F., Retamozo, S., Horvath, I. -F, Sivils, K., Theander, E., Sandhya, P., De Vita, S., Sanchez-Guerrero, J., van der Heijden, E., Moca-Trevisano, V., Wahren-Herlenius, M., Mariette, X., and Ramos-Casals, M.
Published: 2016
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47. Association of psoriasis and/or psoriatic arthritis with autoimmune diseases: the experience of two Italian integrated Dermatology/Rheumatology outpatient clinics

Author: Carubbi, F, Chimenti, M, Blasetti, G, Cipriani, P, Musto, A, Fargnoli, Mc, Perricone, R, Giacomelli, R, Peris, Ketty, Peris, Ketty (ORCID:0000-0002-5237-0463), Carubbi, F, Chimenti, M, Blasetti, G, Cipriani, P, Musto, A, Fargnoli, Mc, Perricone, R, Giacomelli, R, Peris, Ketty, and Peris, Ketty (ORCID:0000-0002-5237-0463)
Abstract: The systemic nature of psoriasis and its association with arthropathy, metabolic syndrome and cardiovascular disease is well established. In contrast, the association between psoriatic disease and other autoimmune disorders is still a matter of debate and data available in the literature are scarce.
Published: 2015

48. Quality of life and unmet needs in patients with inflammatory arthropathies: results from the multicentre, observational RAPSODIA study

Author: Giacomelli, R, Gorla, R, Trotta, F, Tirri, R, Grassi, W, Bazzichi, L, Galeazzi, M, Matucci Cerinic, M, Scarpa, R, Cantini, F, Gerli, R, Lapadula, G, Sinigaglia, L, Ferraccioli, Gianfranco, Olivieri, I, Ruscitti, P, Sarzi Puttini, P., Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Giacomelli, R, Gorla, R, Trotta, F, Tirri, R, Grassi, W, Bazzichi, L, Galeazzi, M, Matucci Cerinic, M, Scarpa, R, Cantini, F, Gerli, R, Lapadula, G, Sinigaglia, L, Ferraccioli, Gianfranco, Olivieri, I, Ruscitti, P, Sarzi Puttini, P., and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)
Abstract: The observational RAPSODIA (RA, PsA and spondylitis including AS) study was planned to assess, in patients with RA, AS and PsA, their involvement in medical decisions, quality of life and unmet needs 15 years after the introduction of biologic therapies in Italy.
Published: 2015

49. Patient evaluations of subcutaneous golimumab delivery by autoinjector (SmartJect®) for treatment of rheumatoid arthritis

Author: Schulze-Koops, H, Herold, M, Giacomelli, R, Samborski, W, Rednic, S, Govoni, M, Vastesaeger, N, Weng, H, Yao, R, Schulze-Koops, H, Herold, M, Giacomelli, R, Samborski, W, Rednic, S, Govoni, M, Vastesaeger, N, Weng, H, and Yao, R
Published: 2014

50. Patient evaluations of subcutaneous golimumab delivery by autoinjector (SmartJect®) for treatment of rheumatoid arthritis

Author: Schulze-Koops, H, Herold, M, Giacomelli, R, Samborski, W, Rednic, S, Govoni, M, Vastesaeger, N, Weng, H, Yao, R, Schulze-Koops, H, Herold, M, Giacomelli, R, Samborski, W, Rednic, S, Govoni, M, Vastesaeger, N, Weng, H, and Yao, R
Published: 2014

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