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1. ACE inhibitors in SSc patients display a risk factor for scleroderma renal crisis—a EUSTAR analysis

Author

Bütikofer L, Varisco PA, Distler O, Kowal-Bielecka O, Allanore Y, Riemekasten G, Villiger PM, Adler S, Avouac J, Walker UA, Guiducci S, Airò P, Hachulla E, Valentini G, Carreira PE, Cozzi F, Gurman AB, Braun-Moscovici Y, Damjanov N, Ananieva LP, Scorza R, Jimenez S, Busquets J, Li M, Müller-Ladner U, Maurer B, Tyndall A, Lapadula G, Iannone F, Becvar R, Sierakowsky S, Bielecka OK, Cutolo M, Sulli A, Cuomo G, Vettori S, Rednic S, Nicoara I, Vlachoyiannopoulos P, Montecucco C, Caporali R, Novak S, Czirják L, Varju C, Chizzolini C, Kucharz EJ, Kotulska A, Kopec-Medrek M, Widuchowska M, Rozman B, Mallia C, Coleiro B, Gabrielli A, Farge D, Hij A, Hesselstrand R, Scheja A, Wollheim F, Martinovic D, Govoni M, Monaco AL, Hunzelmann N, Pellerito R, Bambara LM, Caramaschi P, Black C, Denton C, Henes J, Santamaria VO, Heitmann S, Krasowska D, Seidel M, Oleszowsky M, Burkhardt H, Himsel A, Salvador MJ, Stamenkovic B, Stankovic A, Tikly M, Starovoytova MN, Engelhart M, Strauss G, Nielsen H, Damgaard K, Szücs G, Mendoza AZ, de la Puente Buijdos C, Sifuentes Giraldo WA, Midtvedt Ø, Garen T, Launay D, Valesini G, Riccieri V, Ionescu RM, Opris D, Groseanu L, Wigley FM, Mihai CM, Cornateanu RS, Ionitescu R, Gherghe AM, Gorga M, Dobrota R, Bojinca M, Schett G, Distler JHW, Meroni P, Zeni S, Mouthon L, De Keyser F, Smith V, Cantatore FP, Corrado A, Ullman S, Iversen L, Pozzi MR, Eyerich K, Hein R, Knott E, Szechinski J, Wiland P, Szmyrka-Kaczmarek M, Sokolik R, Morgiel E, Krummel-Lorenz B, Saar P, Aringer M, Günther C, Anic B, Baresic M, Mayer M, Radominski SC, de Souza Müller C, Azevedo VF, Agachi S, Groppa L, Chiaburu L, Russu E, Zenone T, Stebbings S, Highton J, Stamp L, Chapman P, Baron M, O'Donnell J, Solanki K, Doube A, Veale D, O'Rourke M, Loyo E, Rosato E, Pisarri S, Tanaseanu CM, Popescu M, Dumitrascu A, Tiglea I, Chirieac R, Ancuta C, Furst DE, Kafaja S, de la Peña Lefebvre PG, Rubio SR, Exposito MV, Sibilia J, Chatelus E, Gottenberg JE, Chifflot H, Litinsky I, Venalis A, Butrimiene I, Venalis P, Rugiene R, Karpec D, Kerzberg E, Montoya F, Cosentino V, Castellvi I., Publica, Bütikofer, L, Varisco, Pa, Distler, O, Kowal-Bielecka, O, Allanore, Y, Riemekasten, G, Villiger, Pm, Adler, S, Avouac, J, Walker, Ua, Guiducci, S, Airò, P, Hachulla, E, Valentini, G, Carreira, Pe, Cozzi, F, Gurman, Ab, Braun-Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller-Ladner, U, Maurer, B, Tyndall, A, Lapadula, G, Iannone, F, Becvar, R, Sierakowsky, S, Bielecka, Ok, Cutolo, M, Sulli, A, Cuomo, G, Vettori, S, Rednic, S, Nicoara, I, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Novak, S, Czirják, L, Varju, C, Chizzolini, C, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Rozman, B, Mallia, C, Coleiro, B, Gabrielli, A, Farge, D, Hij, A, Hesselstrand, R, Scheja, A, Wollheim, F, Martinovic, D, Govoni, M, Monaco, Al, Hunzelmann, N, Pellerito, R, Bambara, Lm, Caramaschi, P, Black, C, Denton, C, Henes, J, Santamaria, Vo, Heitmann, S, Krasowska, D, Seidel, M, Oleszowsky, M, Burkhardt, H, Himsel, A, Salvador, Mj, Stamenkovic, B, Stankovic, A, Tikly, M, Starovoytova, Mn, Engelhart, M, Strauss, G, Nielsen, H, Damgaard, K, Szücs, G, Mendoza, Az, de la Puente Buijdos, C, Sifuentes Giraldo, Wa, Midtvedt, Ø, Garen, T, Launay, D, Valesini, G, Riccieri, V, Ionescu, Rm, Opris, D, Groseanu, L, Wigley, Fm, Mihai, Cm, Cornateanu, R, Ionitescu, R, Gherghe, Am, Gorga, M, Dobrota, R, Bojinca, M, Schett, G, Distler, Jhw, Meroni, P, Zeni, S, Mouthon, L, De Keyser, F, Smith, V, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, Pozzi, Mr, Eyerich, K, Hein, R, Knott, E, Szechinski, J, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Krummel-Lorenz, B, Saar, P, Aringer, M, Günther, C, Anic, B, Baresic, M, Mayer, M, Radominski, Sc, de Souza Müller, C, Azevedo, Vf, Agachi, S, Groppa, L, Chiaburu, L, Russu, E, Zenone, T, Stebbings, S, Highton, J, Stamp, L, Chapman, P, Baron, M, O'Donnell, J, Solanki, K, Doube, A, Veale, D, O'Rourke, M, Loyo, E, Rosato, E, Pisarri, S, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Chirieac, R, Ancuta, C, Furst, De, Kafaja, S, de la Peña Lefebvre, Pg, Rubio, Sr, Exposito, Mv, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Venalis, A, Butrimiene, I, Venalis, P, Rugiene, R, Karpec, D, Kerzberg, E, Montoya, F, Cosentino, V, and Castellvi, I.

Subjects
INVOLVEMENT, Male, Hypertension, Renal, ACE inhibitors, lcsh:Diseases of the musculoskeletal system, Scleroderma Renal Crisis, MULTICENTER, Angiotensin-Converting Enzyme Inhibitors, Scleroderma, Scleroderma renal crisis, 0302 clinical medicine, Risk Factors, Medicine and Health Sciences, Medicine, 030212 general & internal medicine, Prospective Studies, 610 Medicine & health, Renal, Antihypertensive drugs, Outcome, antihypertensive drugs, arterial hypertension, outcome, scleroderma renal crisis, Incidence (epidemiology), Incidence, Hazard ratio, Acute Kidney Injury, Middle Aged, Europe, Treatment Outcome, Population Surveillance, Cohort, Hypertension, Female, 360 Social problems & social services, Proto-oncogene tyrosine-protein kinase Src, Research Article, Arterial hypertension, medicine.medical_specialty, 03 medical and health sciences, ENDOTHELIN-1, Internal medicine, Humans, Risk factor, Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, SYSTEMIC-SCLEROSIS, Systemic, medicine.disease, Concomitant, lcsh:RC925-935, business
Abstract

Objectives To investigate the effect of ACE inhibitors (ACEi) on the incidence of scleroderma renal crisis (SRC) when given prior to SRC in the prospectively collected cohort from the European Scleroderma Trial and Research Group (EUSTAR). Methods SSc patients without prior SRC and at least one follow-up visit were included and analyzed regarding SRC, arterial hypertension, and medication focusing on antihypertensive medication and glucocorticoids (GC). Results Out of 14,524 patients in the database, we identified 7648 patients with at least one follow-up. In 27,450 person-years (py), 102 patients developed SRC representing an incidence of 3.72 (3.06–4.51) per 1000 py. In a multivariable time-to-event analysis adjusted for age, sex, disease severity, and onset, 88 of 6521 patients developed SRC. The use of ACEi displayed an increased risk for the development of SRC with a hazard ratio (HR) of 2.55 (95% confidence interval (CI) 1.65–3.95). Adjusting for arterial hypertension resulted in a HR of 2.04 (95%CI 1.29–3.24). There was no evidence for an interaction of ACEi and arterial hypertension (HR 0.83, 95%CI 0.32–2.13, p = 0.69). Calcium channel blockers (CCB), angiotensin receptor blockers (ARB), endothelin receptor antagonists, and GC—mostly in daily dosages below 15 mg of prednisolone—did not influence the hazard for SRC. Conclusions ACEi in SSc patients with concomitant arterial hypertension display an independent risk factor for the development of SRC but are still first choice in SRC treatment. ARBs might be a safe alternative, yet the overall safety of alternative antihypertensive drugs in SSc patients needs to be further studied.

Published
2020

2. Clinical characteristics of juvenile onset systemic sclerosis patients from the juvenile scleroderma inception cohort compared to adult age juvenile-onset patients from EUSTAR. Are these differences suggesting risk for mortality?

Author

Foeldvari, I, Klotsche, J, Carreira, PE, Kasapcopur, O, Adrovic, A, Torok, K, Airò, P, Iannone, F, Allanore, Y, Balbir-Gurman, A, Schmeiser, T, Sztajnbok, F, Terreri, MT, Stanevicha, V, Anton, J, Feldman, B, Khubchandani, R, Alexeeva, E, Johnson, S, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Campochiaro, C, De Vries-Bouwstra, J, Kostik, M, Lehman, T, Marrani, E, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Malcova, H, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Seskute, G, Truchetet, ME, Veale, D, Hoffmann-Vold, AM, Gabrielli, A, Distler, O, Foeldvari, I, Klotsche, J, Carreira, PE, Kasapcopur, O, Adrovic, A, Torok, K, Airò, P, Iannone, F, Allanore, Y, Balbir-Gurman, A, Schmeiser, T, Sztajnbok, F, Terreri, MT, Stanevicha, V, Anton, J, Feldman, B, Khubchandani, R, Alexeeva, E, Johnson, S, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Campochiaro, C, De Vries-Bouwstra, J, Kostik, M, Lehman, T, Marrani, E, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Malcova, H, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Seskute, G, Truchetet, ME, Veale, D, Hoffmann-Vold, AM, Gabrielli, A, and Distler, O

Published
2022

3. Inmune-mediated inflammatory rheumatic diseases in transgender people: A scoping review

Author

Salgado E, Romera-Baurés M, Beltran-Catalan E, Naredo E, Carreira PE, Garcia-Vivar M, Moreno-Muelas JV, Boteanu A, Calvo-Penades I, Sellas-Fernandez A, Valero M, and Gomez-Reino JJ

Subjects
Muskuloeskeletal disease, Hormone replacement therapy, Immune mediated rheumatic disease, Transgender, Inflammatory arthropathy
Abstract

BACKGROUND: In immune-mediated inflammatory rheumatic diseases (IMIRD), there are differences between cis-men and cis-women in epidemiology, clinical feature, therapeutic approach, treatment response, and prognosis. In transgender individuals, information concerning IMIRD is not substantial. The assessment of information concerning rheumatic diseases in transgenders is crucial because transgenders may undergo treatments with potential impacts on IMIRD. We aim to collect and discuss current knowledge on IMIRD in transgender individuals, determine the coverage of the literature, identify the knowledge gaps, and highlight opportunities for future research. METHODS: We did a scoping review of publications collected through a systematic search of transgender patients with any IMIRD. Data sources were Medline, Embase, and Web of Knowledge. Synthesis of results and qualitative review of data information was collected in tables. A semi-quantification of the quality of the articles reporting clinical studies was performed. RESULTS: There were 11 transwoman, and 3 transmen cases of systemic lupus erythematosus (5 cases), skin lupus erythematosus (2), systemic sclerosis (4), anti-synthetase syndrome (1), rheumatoid arthritis (1) and ankylosing spondylitis (1). Eleven were de novo cases and three had prior history of IMIRD and developed a comorbidity after starting hormone replacement therapy. The clinical expression of the disease was variable. Two transwomen and one transman developed thrombotic events. The lupus skin lesions in one transman improved following testosterone treatment. No clinical studies were identified. Quality of publications was disparate. CONCLUSION: Although the number of cases is small, most cases of IMIRD occur in transwomen. The absence of solid data warrants caution in establishing recommendations regarding hormone replacement therapy in transgenders with IMIRD. There is an essential need for the consideration of cisgender and transgender particularities in future research on IMIRD.

Published
2022

4. Estimated glomerular filtration rate is a marker of mortality in the European Scleroderma Trials and Research Group (EUSTAR) database

Author

Gigante A, Hoffmann-Vold AM, Fegatelli DA, Gabrielli A, Leodori G, Coleiro B, De Santis M, Dagna L, Alegre-Sancho JJ, Montecucco C, Carreira PE, Balbir-Gurman A, Doria A, Riemekasten G, Airò P, Distler J, Distler O, Rosato E, and EUSTAR collaborators

Subjects
EUSTAR, glomerular filtration rate, integumentary system, systemic sclerosis, scleroderma renal crisis
Abstract

Objectives The study aim was to evaluate the estimated glomerular filtration rate (eGFR), its association with clinical disease and its predictive ability with respect to mortality in SSc patients from the European Scleroderma Trials and Research Group (EUSTAR) database. Methods SSc patients from the EUSTAR database who had items required for the calculation of eGFR at a baseline visit and a second follow-up visit available were included. A cut-off eGFR value of 60 ml/min was chosen for all SSc patients, and 30 ml/min for those with scleroderma renal crisis (SRC). Cox regression and competing risk analysis were performed to evaluate the use of eGFR as a predictive factor of mortality. Results A total of 3650 SSc patients were included in this study. The median serum level of creatinine and the mean of eGFR were 0.8 mg/dl (interquartile range = 0.6-0.9) and 86.6 +/- 23.7 ml/min, respectively. The eGFR was significantly lower in patients with pulmonary hypertension. Overall survival (OS) was significantly reduced in SSc patients with eGFR < 60 ml/min compared with patients with eGFR >= 60 ml/min [OS at 5 years 0.763 (95% CI: 0.700, 0.814) vs 0.903 (95% CI: 0.883, 0.919; P < 0.001)]. In multivariable analysis, OS was associated with male gender (P < 0.01), systolic pulmonary arterial pressure (sPAP) (P < 0.001) and eGFR (P < 0.001). The cumulative incidence of deaths due to SSc was associated with increased sPAP (P < 0.001) and reduced eGFR (P < 0.05). The OS at 5 years of 53 SRC patients was not significantly different between SSc patients with eGFR > 30 ml/min and those with eGFR Conclusion eGFR represents a predictive risk factor for overall survival in SSc. The eGFR, however, does not represent a risk factor for death in SRC.

Published
2021

5. LAUDES Study: impact of digital ulcers on hand functional limitation, work productivity and daily activities, in systemic sclerosis patients

Author

Castellví I, Eguiluz S, Escudero-Contreras A, Ríos JJ, Calvo-Alén J, Callejas-Rubio JL, De la Puente C, Simeón CP, Narváez FJ, Espinosa G, Carreira PE, Rubio-Rivas M, Alegre JJ, Guillén-Del-Castillo A, Román-Ivorra JA, and Fonollosa V

Subjects
Quality of life, Scleroderma and related disorders, Disability evaluation, Human activities, Disability evaluation, Hand, Human activities, Quality of life, Scleroderma and related disorders, Hand
Abstract

The objective of this study was to evaluate the impact of digital ulcers (DUs) in daily life of systemic sclerosis (SSc) Spanish patients. We developed a multicenter observational study to compare functional disability in SSc patients with active DUs vs. those without DUs. An additional correlation between perception of patients and physicians on disability due to DUs was performed. A total of 199 patients were enrolled, 70 (35%) with DUs. Patients with DUs were younger (48 vs. 58 years; p < 0.001) and had more frequently the diffuse subtype of SSc (45 vs. 24%; p = 0.004) than patients without DUs. Patients with DUs showed significantly higher scores in the Cochin Hand Function Scale overall (p < 0.002) and for each of its five dimensions. They also showed higher scores in the Systemic Sclerosis Health Assessment Questionnaire items related to hand function such as, dress and self-care (p < 0.013), eat (p < 0.013) and grip (p < 0.03), and higher Visual Analogic Scale scores for pain (p < 0.013), trouble related with Raynaud's Phenomenon (p < 0.001) and sense of severity (p < 0.004). Impact on daily activities was significantly higher in patients with DUs (p = 0.002), with a non-significant trend to experience higher impact on work productivity (p = 0.07). A high correlation was found between DUs patients and physicians opinion on the impact of DUs (daily life: Pearson R = 0.86; work productivity: Pearson R = 0.87). Study findings show an impaired hand function and increased disability for daily life activities and work productivity in SSc patients with DUs compared with patients without DUs in Spanish population.

Published
2019

6. IDENTIFICATION OF FOUR NEW SHARED SUSCEPTIBILITY GENES IN SYSTEMIC SCLEROSIS AND CROHN¿S DISEASE THROUGH A CROSS-DISEASE META-GWAS

Author

González-Serna, David, Ochoa, Eguzkine, López-Isac, Elena, Julià, A, Degenhardt, Frauke, van Beelen Granlund, A, Carreira, PE, Simeon, CP, Ortego-Centeno, N., González-Gay, M. A., Vicente, Esther, Beltran, E, Alegre-Sancho, Juan-José, Spanish Scleroderma Group, Beretta, L., Santaniello, A, Lunardi, C., Hunzelmann, Nicolas, Riemekasten, G, Witte, T, Distler, J.H.W., Kreuter, A., Airó, P, Koeleman, B. P., Voskuyl, AE, de Vries-Bouwstra, Jeska, Radstake, TRDJ, Wijmenga, C, Assassi, S., Franke, Andre, Marsal, Sara, Mayes, Maureen D., Martín, J., and Márquez A

Published
2019

7. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Author

Elhai, M, Boubaya, M, Distler, O, Smith, V, Matucci-Cerinic, M, Sancho, JJ, Truchetet, ME, Braun-Moscovici, Y, Iannone, F, Novikov, PI, Lescoat, A, Siegert, E, Castellvi, I, Airo, P, Vettori, S, Langhe, E, Hachulla, E, Erler, A, Ananieva, L, Krusche, M, Lopez-Longo, FJ, Distler, JHW, Hunzelmann, N, Hoffmann-Vold, AM, Riccieri, V, Hsu, VM, Pozzi, MR, Ancuta, C, Rosato, E, Mihai, C, Kuwana, M, Saketkoo, LA, Chizzolini, C, Hesselstrand, R, Ullman, S, Yavuz, S, Rednic, S, Caimmi, C, Bloch-Queyrat, C, Allanore, Y, Guiducci, S, Walker, UA, Kyburz, D, Lapadula, G, Maurer, B, Jordan, S, Dobrota, R, Becvar, R, Sierakowsky, S, Bielecka, OK, Sulli, A, Cutolo, M, Cuomo, G, Nicoara, I, Kahan, A, Vlachoyiannopoulos, PG, Montecucco, CM, Caporali, R, Stork, J, Inanc, M, Carreira, PE, Novak, S, Czirjak, L, Varju, C, Kucharz, EJ, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Cozzi, F, Rozman, B, Mallia, C, Coleiro, B, Gabrielli, A, Farge, D, Wu, C, Marjanovic, Z, Faivre, H, Hij, D, Dhamadi, R, Wollheim, F, Scheja, A, Wuttge, DM, Andreasson, K, Martinovic, D, Balbir-Gurman, A, Trotta, F, Lo Monaco, A, Pellerito, R, Mauriziano, O, Caramaschi, P, Morovic-Vergles, J, Black, C, Denton, C, Damjanov, N, Henes, J, Santamaria, VO, Heitmann, S, Krasowska, D, Matthias, Hasler, P, Burkhardt, H, Himsel, A, Bajocchi, G, Da Silva, JAP, Salvador, MJ, Stamenkovic, B, Stankovic, A, Selmi, CF, De Santis, M, Tikly, M, Denisov, LN, Herrick, A, Muller-Ladner, U, Frerix, M, Tarner, I, Scorza, R, Puppo, F, Engelhart, M, Strauss, G, Nielsen, H, Damgaard, K, Szucs, G, Mendoza, AZ, de la Puente, C, Giraldo, WAS, Midtvedt, O, Reiseter, S, Garen, T, Launay, D, Valesini, G, Ionescu, RM, Groseanu, L, Opris, D, Cornateanu, RS, Ionitescu, R, Gherghe, AM, Soare, A, Gorga, M, Bojinca, M, Milicescu, M, Sunderkotter, C, Kuhn, A, Sandorfi, N, Schett, G, Beyer, C, Meroni, P, Ingegnoli, F, Mouthon, L, De Keyser, F, Melsens, K, Cantatore, FP, Corrado, A, Iversen, L, von Muhlen, CA, Bohn, JM, Lonzetti, LS, Eyerich, K, Hein, R, Knott, E, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Houssiau, FA, Krummel-Lorenz, B, Saar, P, Aringer, M, Gunther, C, Westhovens, R, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Uprus, M, Otsa, K, Granel, B, Muller, CD, Radominski, SC, Azevedo, VF, Jimenez, S, Busquets, J, Agachi, S, Groppa, L, Chiaburu, L, Russu, E, Popa, S, Zenone, T, Pileckyte, M, Mathieu, A, Vacca, A, Sampaio-Barros, PD, Yoshinari, NH, Marangoni, RG, Martin, P, Fuocco, L, Stebbings, S, Highton, J, Chapman, P, O'Donnell, J, Stamp, L, Doube, A, Solanki, K, Veale, D, O'Rourke, M, Loyo, E, Li, MT, Mohamed, WAAA, Amoroso, A, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, CM, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Chirieac, R, Furst, D, Villiger, P, Adler, S, van Laar, J, Kayser, C, Fathi, N, Hassanien, M, Lefebvre, PGD, Rubio, SR, Exposito, MV, Chatelus, E, Sibilia, J, Gottenberg, JE, Chifflot, H, Litinsky, I, Emery, P, Buch, M, Del Galdo, F, Venalis, A, Butrimiene, I, Venalis, P, Rugiene, R, Karpec, D, Lasky, JA, Cosentino, V, Kerzberg, E, Montoya, F, Bianchi, W, Carneiro, S, Maretti, GB, Bianchi, DV, Limonta, M, Lupi, ALBE, Lupi, E, Rosner, I, Rozenbaum, M, Slobodin, G, Boulman, N, Rimar, D, Couto, M, Kahl, S, Chen, F, McCloskey, D, Malveaux, H, Spertini, F, Ribi, C, Buss, G, Martin, T, Guffroy, A, Poindron, V, Chotchaeva, F, Mukhin, NA, Moiseev, S, EUSTAR Network, Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan José, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I, Lescoat, Alain, Siegert, Elise, Castellví, Ivan, Airó, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, López-Longo, F. J., Distler, Jörg H W, Hunzelmann, Nicola, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M, Pozzi, Maria R, Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, Kuwana, Masataka, Saketkoo, Lesley Ann, Chizzolini, Carlo, Hesselstrand, Roger, Ullman, Susanne, Yavuz, Sule, Rednic, Simona, Caimmi, Cristian, Bloch-Queyrat, Coralie, Allanore, Yannick, and Cuomo, Giovanna

Subjects
Male, Vital capacity, systemic sclerosis, Pulmonary Fibrosis, Vital Capacity, Scleroderma, lung fibrosis, rituximab, skin fibrosis, immune system diseases, DLCO, hemic and lymphatic diseases, Immunology and Allergy, Medicine, Prospective Studies, Registries, skin and connective tissue diseases, Prospective cohort study, Lung, skin fibrosi, Skin, ddc:616, integumentary system, Orvostudományok, Middle Aged, Respiratory Function Tests, lung fibrosis, rituximab, skin fibrosis, systemic sclerosis, Treatment Outcome, lung fibrosi, Antirheumatic Agents, Systemic sclerosis, Rituximab, Female, systemic sclerosi, medicine.drug, Adult, medicine.medical_specialty, Immunology, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, FEV1/FVC ratio, Rheumatology, Internal medicine, Humans, Adverse effect, Propensity Score, Aged, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Systemic, Skin fibrosis, business.industry, medicine.disease, Fibrosis, Lung fibrosis, business
Abstract

ObjectiveTo assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice.MethodsWe performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab.Results254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47–5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55–1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56–3.53], pConclusionRituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.

Published
2019

8. Smoking in Systemic Sclerosis: A Longitudinal European Scleroderma Trials and Research Group Study

Author

Jaeger, VK, Valentini, G, Hachulla, E, Cozzi, F, Distler, O, Airó, P, Czirják, L, Allanore, Y, Siegert, E, Rosato, E, Matucci-Cerinic, M, Caimmi, C, Henes, J, Carreira, PE, Smith, V, del Galdo, F, Denton, CP, Ullman, S, Langhe, ED, Riccieri, V, Alegre-Sancho, JJ, Rednic, S, Müller-Ladner, U, Walker, UA, and EUSTAR coauthors

Subjects
smoking, systemic sclerosis, scleroderma, respiratory tract diseases
Abstract

OBJECTIVE: Data on the role of tobacco exposure in systemic sclerosis (SSc ; scleroderma) severity and progression are scarce. We aimed to assess the effects of smoking on the evolution of pulmonary and skin manifestations, based on the European Scleroderma Trials and Research group database. METHODS: Adult SSc patients with data on smoking history and a 12-24-month follow-up visit were included. Associations of severity and progression of organ involvement with smoking history and the Comprehensive Smoking Index were assessed using multivariable regression analyses. RESULTS: A total of 3, 319 patients were included (mean age 57 years, 85% female) ; 66% were never smokers, 23% were ex-smokers, and 11% were current smokers. Current smokers had a lower percentage of antitopoisomerase autoantibodies than previous or never smokers (31% versus 40% and 45%, respectively). Never smokers had a higher baseline forced expiratory volume in 1 second/forced vital capacity (FEV1 /FVC) ratio than previous and current smokers (P < 0.001). The FEV1 /FVC ratio declined faster in current smokers than in never smokers (P = 0.05) or ex-smokers (P = 0.01). The baseline modified Rodnan skin thickness score (MRSS) and the MRSS decline were comparable across smoking groups. Although heavy smoking (>25 pack- years) increased the odds of digital ulcers by almost 50%, there was no robust adverse association of smoking with digital ulcer development. CONCLUSION: The known adverse effect of smoking on bronchial airways and alveoli is also observed in SSc patients ; however, robust adverse effects of smoking on the progression of SSc-specific pulmonary or cutaneous manifestations were not observed.

Published
2018

9. Abatacept in patients with rheumatoid arthritis and interstitial lung disease: A national multicenter study of 63 patients

Author

Fernández-Díaz C, Loricera J, Castañeda S, López-Mejías R, Ojeda-García C, Olivé A, Rodríguez-Muguruza S, Carreira PE, Pérez-Sandoval T, Retuerto M, Cervantes-Pérez EC, Flores-Robles BJ, Hernández-Cruz B, Urruticoechea A, Maíz-Alonso O, Arboleya L, Bonilla G, Hernández-Rodríguez Í, Palma D, Delgado C, Expósito-Molinero R, Ruibal-Escribano A, Álvarez-Rodríguez B, Blanco-Madrigal J, Bernal J, Vela C, Rodríguez-Gómez M, Fito C, Ortiz-Sanjuán F, Narváez J, Moreno M, López-Corbeto M, Mena-Vázquez N, Aguilera-Cros C, Romero-Yuste S, Ordóñez S, Villa-Blanco I, Gonzélez-Vela MC, Mora-Cuesta V, Palmou-Fontana N, Hernández JL, González-Gay MA, and Blanco R

Subjects
Abatacept, Interstitial lung disease, respiratory system, Rheumatoid arthritis, Biologics, respiratory tract diseases
Abstract

Objective: Interstitial lung disease (ILD) is one of the most serious complications of rheumatoid arthritis (RA). In the present study, we aimed to assess the efficacy of abatacept (ABA) in patients with ILD associated to RA. Methods: National multicenter, non-controlled, open-label registry study of RA patients with ILD treated with ABA. Results: 63 patients (36 women) with RA-associated ILD undergoing ABA therapy were studied. The mean +/- standard deviation age at the time of the study was 63.2 +/- 9.8 years. The median duration of RA and ILD from diagnosis were 6.8 and 1 year, respectively. RA was seropositive in 55 patients (87.3%). In 15 (23.8%) of 63 patients the development of ILD was closely related to the administration of synthetic or biologic disease modifying anti-rheumatic drugs. After a follow-up of 9.4 +/- 3.2 months, two-thirds of patients remained stable whereas one-quarter experienced improvement in the Modified Medical Research Council scale. At that time forced vital capacity remained stable in almost two-thirds of patents and improved in one out of five patients assessed. Also, diffusing capacity of the lung for carbon monoxide remained stable in almost two-thirds and showed improvement in a quarter of the patients assessed. At 12 months, 50% of the 22 patients in whom chest HRCT scan was performed due persistence of respiratory symptoms showed stabilization, 8 (36.4%) improvement and 3 worsening of the HRCT scan pattern. Eleven of 63 patients had to discontinue ABA, mainly due to adverse events. Conclusion: ABA appears to be an effective in RA-associated ILD. (C) 2018 Elsevier Inc. All rights reserved.

Published
2018

10. Gender differences in early systemic sclerosis patients: a report from the EULAR scleroderma trials and research group (EUSTAR) database

Author

Carreira, PE, Carmona, L, Joven, BE, Loza, E, Andreu, JL, Riemekasten, G, Vettori, S, Balbir-Gurman, A, Airò, P, Walker, UA, Damjanov, N, Matucci-Cerinic, M, Ananieva, LP, Rednic, S, Czirják, L, Distler, O, Farge, D, Hesselstrand, R, Corrado, A, Caramaschi, P, Tikly, M, Allanore, Y, Valentini, G, Hanes, J, Gabrielli, A, Lapadula, G, Heitmann, S, Valesini, G, von Mühlen, CA, Kucharz, EJ, Cozzi, F, Rozman, B, Pellerito, R, Müller-Ladner, U, Montecucco, C, Smith, V, Jimenez, S, Martinovic, D, Novak, S, Burkhardt, H, Mihai, CM, Pozzi, MR, Vacca, A, Radominski, SC, Chizzolini, C, Krasowska, D, Mouthon, L, Westhovens, R, Mallia, C, Wiland, P, Kummel-Lorenz, B, Vlachoyiannopoulos, P, Hachulla, E, Üprus, M, Sulli, A, Stork, J, Denton, CP, Ortiz, V, Stamenkovic, B, de la Puente, C, Meroni, P, Popa, S, Solanki, K, Becvar, R, Seidel, M, Pereira da Silva, JA, Selmi, CF, Nielsen, H, Aringer, M, Anic, B, and Yavuz, S

Subjects
integumentary system, systemic sclerosis, gender, cohort, skin and connective tissue diseases
Abstract

OBJECTIVES: To describe differences in clinical presentation between men and women in a large group of patients with early (

Published
2018

11. Digital ulcers predict a worse disease course in patients with systemic sclerosis

Author

Mihai C, Landewé R, van der Heijde D, Walker UA, Constantin PI, Gherghe AM, Ionescu R, Rednic S, Allanore Y, Avouac J, Czirják L, Hachulla E, Riemekasten G, Cozzi F, Airò P, Cutolo M, Mueller-Ladner U1 Matucci-Cerinic M, Launay D, Dobrotă R, Sfrenţ-Cornăţeanu R, Zingarelli S, Pigatto E, Cuomo G, Caramaschi P, Ananieva L, Ullman S, Iversen L, Gurman AB, Braun-Moscovici Y, Carreira PE, Joven BE, Minier T, Guiducci S, Bellando-Randone S, Pellerito R, Hunzelmann N, Tarner IH, Radominski SC, de Souza Müller C, Iannone F, Henes J, Bancel DF, Damjanov N, Ostojić P, Pozzi MR, Hesselstrand R, Denton C, Krasowska D, Tikly M, Riccieri V, Cantatore FP, Corrado A, Da Silva JA, Salvador MJ, Tyndall A, Gabrielli A, Distler O, Jordan S, Heitmann S, Burkhardt H, Himsel A, Rozman B, Smith V, De Keyser F, Kalitena DM, Radic M, Filipescu I, Petcu A, Vlachoyiannopoulos P, Kucharz EJ, Widuchowska M, Kopec-Medrek M, Kotulska A, Szücs G, Stankovic A, Stamenkovic B, Selmi CF, De Santis M, Marasini B, Coleiro B, Santamaria VO, Westhovens R, Bečvář R, Novak S, Engelhart M, Meroni P, Ingegnoli F, Zeni S, Sulli A, Distler J, Yavuz S, Montecucco C, Eyerich K, Krummel-Lorenz B, Zenone T, Midtvedt Ø, Chizzolini C, Seidel M, Oleszowsky M, Üprus M, Opriş D, Groşeanu L, Bielecka OK, Antonio ZM, Szechinski J, Morović-Vergles J, Scorza R, Puppo F, Mathieu A, Anic B, Stork J, Stebbings S, Inanc M, Hasler P, von Mühlen CA, Aringer M, Popa S, Li M, Rosato E., Mihai, C, Landewé, R, van der Heijde, D, Walker, Ua, Constantin, Pi, Gherghe, Am, Ionescu, R, Rednic, S, Allanore, Y, Avouac, J, Czirják, L, Hachulla, E, Riemekasten, G, Cozzi, F, Airò, P, Cutolo, M, Mueller-Ladner U1 Matucci-Cerinic, M, Launay, D, Dobrotă, R, Sfrenţ-Cornăţeanu, R, Zingarelli, S, Pigatto, E, Cuomo, G, Caramaschi, P, Ananieva, L, Ullman, S, Iversen, L, Gurman, Ab, Braun-Moscovici, Y, Carreira, Pe, Joven, Be, Minier, T, Guiducci, S, Bellando-Randone, S, Pellerito, R, Hunzelmann, N, Tarner, Ih, Radominski, Sc, de Souza Müller, C, Iannone, F, Henes, J, Bancel, Df, Damjanov, N, Ostojić, P, Pozzi, Mr, Hesselstrand, R, Denton, C, Krasowska, D, Tikly, M, Riccieri, V, Cantatore, Fp, Corrado, A, Da Silva, Ja, Salvador, Mj, Tyndall, A, Gabrielli, A, Distler, O, Jordan, S, Heitmann, S, Burkhardt, H, Himsel, A, Rozman, B, Smith, V, De Keyser, F, Kalitena, Dm, Radic, M, Filipescu, I, Petcu, A, Vlachoyiannopoulos, P, Kucharz, Ej, Widuchowska, M, Kopec-Medrek, M, Kotulska, A, Szücs, G, Stankovic, A, Stamenkovic, B, Selmi, Cf, De Santis, M, Marasini, B, Coleiro, B, Santamaria, Vo, Westhovens, R, Bečvář, R, Novak, S, Engelhart, M, Meroni, P, Ingegnoli, F, Zeni, S, Sulli, A, Distler, J, Yavuz, S, Montecucco, C, Eyerich, K, Krummel-Lorenz, B, Zenone, T, Midtvedt, Ø, Chizzolini, C, Seidel, M, Oleszowsky, M, Üprus, M, Opriş, D, Groşeanu, L, Bielecka, Ok, Antonio, Zm, Szechinski, J, Morović-Vergles, J, Scorza, R, Puppo, F, Mathieu, A, Anic, B, Stork, J, Stebbings, S, Inanc, M, Hasler, P, von Mühlen, Ca, Aringer, M, Popa, S, Li, M, and Rosato, E.

Subjects
Epidemiology, Cardiovascular Disease, Systemic Sclerosis
Published
2016

12. Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study.

Author

Herrick, AL, Peytrignet, S, Lunt, M, Pan, X, Hesselstrand, R, Mouthon, L, Silman, AJ, Dinsdale, G, Brown, E, Czirják, L, Distler, JHW, Distler, O, Fligelstone, K, Gregory, WJ, Ochiel, R, Vonk, MC, Ancuţa, C, Ong, VH, Farge, D, Hudson, M, Matucci-Cerinic, M, Balbir-Gurman, A, Midtvedt, Ø, Jobanputra, P, Jordan, AC, Stevens, W, Moinzadeh, P, Hall, FC, Agard, C, Anderson, ME, Diot, E, Madhok, R, Akil, M, Buch, MH, Chung, L, Damjanov, NS, Gunawardena, H, Lanyon, P, Ahmad, Y, Chakravarty, K, Jacobsen, S, MacGregor, AJ, McHugh, N, Müller-Ladner, U, Riemekasten, G, Becker, M, Roddy, J, Carreira, PE, Fauchais, AL, Hachulla, E, Hamilton, J, İnanç, M, McLaren, JS, van Laar, JM, Pathare, S, Proudman, SM, Rudin, A, Sahhar, J, Coppere, B, Serratrice, C, Sheeran, T, Veale, DJ, Grange, C, Trad, G-S, Denton, CP, Herrick, AL, Peytrignet, S, Lunt, M, Pan, X, Hesselstrand, R, Mouthon, L, Silman, AJ, Dinsdale, G, Brown, E, Czirják, L, Distler, JHW, Distler, O, Fligelstone, K, Gregory, WJ, Ochiel, R, Vonk, MC, Ancuţa, C, Ong, VH, Farge, D, Hudson, M, Matucci-Cerinic, M, Balbir-Gurman, A, Midtvedt, Ø, Jobanputra, P, Jordan, AC, Stevens, W, Moinzadeh, P, Hall, FC, Agard, C, Anderson, ME, Diot, E, Madhok, R, Akil, M, Buch, MH, Chung, L, Damjanov, NS, Gunawardena, H, Lanyon, P, Ahmad, Y, Chakravarty, K, Jacobsen, S, MacGregor, AJ, McHugh, N, Müller-Ladner, U, Riemekasten, G, Becker, M, Roddy, J, Carreira, PE, Fauchais, AL, Hachulla, E, Hamilton, J, İnanç, M, McLaren, JS, van Laar, JM, Pathare, S, Proudman, SM, Rudin, A, Sahhar, J, Coppere, B, Serratrice, C, Sheeran, T, Veale, DJ, Grange, C, Trad, G-S, and Denton, CP

Abstract

OBJECTIVES: Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs). METHODS: The modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. 'Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (±3 months). Logistic models were fitted to predict progression and, using receiver operating characteristic (ROC) curves, were compared on the basis of the area under curve (AUC), accuracy and positive predictive value (PPV). RESULTS: 66 patients (22.5%) progressed, 227 (77.5%) did not (33 could not have their status assessed due to insufficient data). Progressors had shorter disease duration (median 8.1 vs 12.6 months, P=0.001) and lower mRSS (median 19 vs 21 units, P=0.030) than non-progressors. Skin score was highest, and peaked earliest, in the anti-RNA polymerase III (Pol3+) subgroup (n=50). A first predictive model (including mRSS, duration of skin thickening and their interaction) had an accuracy of 60.9%, AUC of 0.666 and PPV of 33.8%. By adding a variable for Pol3 positivity, the model reached an accuracy of 71%, AUC of 0.711 and PPV of 41%. CONCLUSIONS: Two prediction models for progressive skin thickening were derived, for use both in clinical practice and for cohort enrichment in RCTs. These models will inform recruitment into the many clinical trials of dcSSc projected for the coming years. TRIAL REGISTRATION NUMBER: NCT02339441.

Published
2018

13. Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database

Author

Carreira, PE, primary, Carmona, L, additional, Joven, BE, additional, Loza, E, additional, Andréu, JL, additional, Riemekasten, G, additional, Vettori, S, additional, Balbir-Gurman, A, additional, Airò, P, additional, Walker, U, additional, Damjanov, N, additional, Matucci-Cerinic, M, additional, Ananieva, LP, additional, Rednic, S, additional, Czirják, L, additional, Distler, O, additional, Farge, D, additional, Hesselstrand, R, additional, Corrado, A, additional, Caramaschi, P, additional, Tikly, M, additional, and Allanore, Y, additional

Published
2018
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14. Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS).

Author

Herrick, AL, Pan, X, Peytrignet, S, Lunt, M, Hesselstrand, R, Mouthon, L, Silman, A, Brown, E, Czirják, L, Distler, JHW, Distler, O, Fligelstone, K, Gregory, WJ, Ochiel, R, Vonk, M, Ancuţa, C, Ong, VH, Farge, D, Hudson, M, Matucci-Cerinic, M, Balbir-Gurman, A, Midtvedt, Ø, Jordan, AC, Jobanputra, P, Stevens, W, Moinzadeh, P, Hall, FC, Agard, C, Anderson, ME, Diot, E, Madhok, R, Akil, M, Buch, MH, Chung, L, Damjanov, N, Gunawardena, H, Lanyon, P, Ahmad, Y, Chakravarty, K, Jacobsen, S, MacGregor, AJ, McHugh, N, Müller-Ladner, U, Riemekasten, G, Becker, M, Roddy, J, Carreira, PE, Fauchais, AL, Hachulla, E, Hamilton, J, İnanç, M, McLaren, JS, van Laar, JM, Pathare, S, Proudman, S, Rudin, A, Sahhar, J, Coppere, B, Serratrice, C, Sheeran, T, Veale, DJ, Grange, C, Trad, G-S, Denton, CP, Herrick, AL, Pan, X, Peytrignet, S, Lunt, M, Hesselstrand, R, Mouthon, L, Silman, A, Brown, E, Czirják, L, Distler, JHW, Distler, O, Fligelstone, K, Gregory, WJ, Ochiel, R, Vonk, M, Ancuţa, C, Ong, VH, Farge, D, Hudson, M, Matucci-Cerinic, M, Balbir-Gurman, A, Midtvedt, Ø, Jordan, AC, Jobanputra, P, Stevens, W, Moinzadeh, P, Hall, FC, Agard, C, Anderson, ME, Diot, E, Madhok, R, Akil, M, Buch, MH, Chung, L, Damjanov, N, Gunawardena, H, Lanyon, P, Ahmad, Y, Chakravarty, K, Jacobsen, S, MacGregor, AJ, McHugh, N, Müller-Ladner, U, Riemekasten, G, Becker, M, Roddy, J, Carreira, PE, Fauchais, AL, Hachulla, E, Hamilton, J, İnanç, M, McLaren, JS, van Laar, JM, Pathare, S, Proudman, S, Rudin, A, Sahhar, J, Coppere, B, Serratrice, C, Sheeran, T, Veale, DJ, Grange, C, Trad, G-S, and Denton, CP

Abstract

OBJECTIVES: The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches. METHODS: This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Clinicians selected one of four protocols for each patient: methotrexate, mycophenolate mofetil (MMF), cyclophosphamide or 'no immunosuppressant'. Patients were assessed three-monthly for up to 24 months. The primary outcome was the change in modified Rodnan skin score (mRSS). Confounding by indication at baseline was accounted for using inverse probability of treatment (IPT) weights. As a secondary outcome, an IPT-weighted Cox model was used to test for differences in survival. RESULTS: Of 326 patients recruited from 50 centres, 65 were prescribed methotrexate, 118 MMF, 87 cyclophosphamide and 56 no immunosuppressant. 276 (84.7%) patients completed 12 and 234 (71.7%) 24 months follow-up (or reached last visit date). There were statistically significant reductions in mRSS at 12 months in all groups: -4.0 (-5.2 to -2.7) units for methotrexate, -4.1 (-5.3 to -2.9) for MMF, -3.3 (-4.9 to -1.7) for cyclophosphamide and -2.2 (-4.0 to -0.3) for no immunosuppressant (p value for between-group differences=0.346). There were no statistically significant differences in survival between protocols before (p=0.389) or after weighting (p=0.440), but survival was poorest in the no immunosuppressant group (84.0%) at 24 months. CONCLUSIONS: These findings may support using immunosuppressants for early dcSSc but suggest that overall benefit is modest over 12 months and that better treatments are needed. TRIAL REGISTRATION NUMBER: NCT02339441.

Published
2017

15. Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study

Author

Jaeger, VK, Wirz, EG, Allanore, Y, Rossbach, P, Riemekasten, G, Hachulla, E, Distler, O, Airò, P, Carreira, PE, Balbir Gurman, A, Tikly, M, Vettori, S, Damjanov, N, Müller-Ladner, U, Distler, JHW, Li, M, Walker, UA, EUSTAR co-authors, Ananieva, L, Heitmann, S, Rednic, S, Riccieri, V, Szmyrka-Kaczmarek, M, Farge, D, Lapadula, G, Matucci-Cerinic, M, Guiducci, S, Hunzelmann, N, Ricci, M, Mihai, C, Veale, D, Hesselstrand, R, Mariok, E, Smith, V, Tarner, IH, Kucharz, EJ, Czirjak, L, Martinovic, D, Solanki, K, Ancuta, CM, Sibilia, J, Paola, C, Hassanien, M, Kahl, S, Wigley, F, Vanthuyne, M, Opris, D, Radominski, SC, Lo Monaco, A, Corrado, A, Koehm, M, Codullo, V, Radim, B, Loyo, E, Uprus, M, Pellerito, R, Zenone, T, Gabrielli, A, Kowal-Bielecka, O, Rozman, B, Scorza, R, Saketkoo, LA, Midtvedt, O, von Muhlen, A, Henes, J, Branimir, A, Hasler, P, Yavuz, S, Adler, S, Krummel-Lorenz, B, Posa, M, Engelhart, M, Denton, C, Krasowska, D, Garcia de la Pena Lefebvre, P, Cozzi, F, Mouthon, L, Rosato, E, Selmi, C, Sancho, JJA, Mallia, C, Limonta, M, Seidel, M, Foti, R, Stamp, L, Ullman, S, Stebbings, S, Santamaria, VO, Del Galdo, F, De Langhe, E, Mathieu, A, Sunderkotter, C, Eyerich, K, Stamenkovic, B, Novak, S, Sampaio-Barros, PD, Kayser, C, Litinsky, I, Couto, M, and Assassi, S

Subjects
integumentary system
Abstract

Objective Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc. Methods In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis. Results Of the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis. Conclusion In SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to ‘widen' the still very narrow ‘window of opportunity'. They may also enable physicians to counsel and manage patients presenting early in the course of SSc more accurately.

Published
2016

16. There is a need for new systemic sclerosis subset criteria. A content analytic approach

Author

Johnson, SR, primary, Soowamber, ML, additional, Fransen, J, additional, Khanna, D, additional, Van Den Hoogen, F, additional, Baron, M, additional, Matucci-Cerinic, M, additional, Denton, CP, additional, Medsger, TA, additional, Carreira, PE, additional, Riemekasten, G, additional, Distler, J, additional, Gabrielli, A, additional, Steen, V, additional, Chung, L, additional, Silver, R, additional, Varga, J, additional, Müller-Ladner, U, additional, Vonk, MC, additional, Walker, UA, additional, Wollheim, FA, additional, Herrick, A, additional, Furst, DE, additional, Czirjak, L, additional, Kowal-Bielecka, O, additional, Del Galdo, F, additional, Cutolo, M, additional, Hunzelmann, N, additional, Murray, CD, additional, Foeldvari, I, additional, Mouthon, L, additional, Damjanov, N, additional, Kahaleh, B, additional, Frech, T, additional, Assassi, S, additional, Saketkoo, LA, additional, and Pope, JE, additional

Published
2017
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17. Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database.

Author

Carreira, PE, Carmona, L, Joven, BE, Loza, E, Andréu, JL, Riemekasten, G, Vettori, S, Balbir-Gurman, A, Airò, P, Walker, U, Damjanov, N, Matucci-Cerinic, M, Ananieva, LP, Rednic, S, Czirják, L, Distler, O, Farge, D, Hesselstrand, R, Corrado, A, and Caramaschi, P

Subjects
*SYSTEMIC scleroderma, *RAYNAUD'S disease, *BIVARIATE analysis, *SYSTOLIC blood pressure, *IMMUNOGLOBULINS, *AGE distribution, *AGE factors in disease, *COMPARATIVE studies, *DATABASES, *DEMOGRAPHY, *RESEARCH methodology, *MEDICAL cooperation, *RESEARCH, *EVALUATION research, *DISEASE incidence, *ACQUISITION of data, *DISEASE prevalence, *CROSS-sectional method, *RETROSPECTIVE studies, *DIAGNOSIS
Abstract

Objective: The aim of this study was to analyse differences in clinical presentation in patients with early (< 3 years' duration) systemic sclerosis (SSc), comparing three age groups according to disease subsets.Method: Cross-sectional analysis of the prospective EULAR Scleroderma Trials and Research database (EUSTAR) was performed. Patients fulfilling preliminary American College of Rheumatology 1980 classification criteria for SSc, with < 3 years from the first non-Raynaud's SSc symptom at first entry, were selected. Patients with < 3 years from the first SSc symptom, including Raynaud's phenomenon, were also analysed. SSc-related variables, including antibodies, SSc subsets, and organ involvement, were examined. Age was categorized into ≤ 30, 31-59, and ≥ 60 years. We performed descriptive and bivariate analyses.Results: The study included 1027 patients: 90% Caucasian, 80% women, and 40% with diffuse disease. In early stages of SSc, younger patients had significantly more anti-Scl-70 antibodies and diffuse disease. With increasing age, we observed more elevation of estimated pulmonary systolic pressure on echocardiography (5%, 13%, and 30%, respectively, in the three age groups), cardiac conduction blocks (6%, 6%, and 15%), and left ventricular diastolic dysfunction (4%, 12%, and 27%). The results were similar for 650 patients with < 3 years from first SSc symptom, including Raynaud's.Conclusion: In early stages of SSc, older patients showed data indicating more severe disease with greater cardiac involvement. The diffuse subset was more frequent in the younger subgroup. The identification of such differences may help in selecting appropriate management for individual patients in clinical practice. [ABSTRACT FROM AUTHOR]

Published
2019
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18. Reconciling Healthcare Professional and Patient Perspectives in the Development of Disease Activity and Response Criteria in Connective Tissue Disease Related Interstitial Lung Diseases

Author

Saketkoo, La, Mittoo, S, Frankel, S, Lesage, D, Sarver, C, Phillips, K, Strand, V, Matteson, El, OMERACT Baughman RP, Brown, Kk, Christmann, Rb, Dellaripa, P, Denton, Cp, Distler, O, Fischer, A, Flaherty, K, Huscher, D, Khanna, D, Kowal Bielecka, O, Merkel, Pa, Oddis, Cv, Pittrow, D, Sandorfi, N, Seibold, Jr, Swigris, J, Wells, A, Antoniou, K, Castelino, Fv, Christopher Stine, L, Collard, Hr, Cottin, V, Danoff, S, Hedlund, R, Highland, Kb, Hummers, L, Lynch, Da, Kim, Ds, Ryu, Jh, Miller, Fw, Nichols, K, Proudman, Sm, Richeldi, L, Shah, Aa, van den Assum, P, Aggarwal, R, Ainslie, G, Alkassab, F, Allanore, Y, Anderson, Me, Andonopoulos, Ap, Antin Ozerkis, D, Arrobas, A, Ascherman, Dp, Assassi, S, Baron, M, Bathon, Jm, Baughman, Rp, Behr, J, Beretta, L, Bingham, Co, Binnie, M, Birring, Ss, Boin, F, Bongartz, T, Bourdin, A, Bouros, D, Brasington, R, Bresser, P, Buch, Mh, Burge, Ps, Carmona, L, Carreira, Pe, Carvalho, Cr, Catoggio, Lj, Chan, Km, Chapman, J, Chatterjee, S, Chua, F, Chung, L, Conron, M, Corte, T, Cosgrove, G, Costabel, U, Cox, G, Crestani, B, Crofford, Lj, Csuka, Me, Curbelo, P, Czirják, L, Daniil, Z, D'Arsigny, Cl, Davis, Gs, de Andrade JA, Dellaripa, Pf, De Vuyst, P, Dempsey, Oj, Derk, Ct, Distler, J, Dixon, Wg, Downey, G, Doyle, Mk, Drent, M, Durairaj, L, Emery, P, Espinoza, Lr, Farge, D, Fathi, M, Fell, Cd, Fessler, Bj, Fitzgerald, Je, Flaherty, Kr, Foeldvari, I, Fox, Ga, Frech, Tm, Freitas, S, Furst, De, Gabrielli, A, García Vicuña, R, Georgiev, Ob, Gerbino, A, Gillisen, A, Gladman, Dd, Glassberg, M, Gochuico, Br, Gogali, A, Goh, Ns, Goldberg, A, Goldberg, Hj, Gourley, Mf, Griffing, L, Grutters, Jc, Gunnarsson, R, Hachulla, E, Hall, Fc, Harari, S, Herrick, Al, Herzog, El, Hesselstrand, R, Highland, K, Hirani, N, Hodgson, U, Hollingsworth, Hm, Homer, Rj, Hoyles, Rk, Hsu, Vm, Hubbard, Rb, Hunzelmann, N, Isasi, Me, Isasi, Es, Jacobsen, S, Jimenez, Sa, Johnson, Sr, Jones, Ch, Kahaleh, B, Kairalla, Ra, Kalluri, M, Kalra, S, Kaner, Rj, Kinder, Bw, Kiter, G, Klingsberg, Rc, Kokosi, M, Kolb, Mr, Kowal Bielecka OM, Kur Zalewska, J, Kuwana, M, Lake, Fr, Lally, Ev, Lasky, Ja, Laurindo, Im, Able, L, Lee, P, Leonard, Ct, Lien, Dc, Limper, Ah, Liossis, Sn, Lohr, Km, Loyd, Je, Lundberg, Ie, Mageto, Yn, Maher, Tm, Mahmud, Th, Manganas, H, Marie, I, Marras, Tk, Martinez, Ja, Martinez, Fj, Mathieu, A, Matucci Cerinic, M, Mayes, Md, Mckown, Km, Medsger, Ta, Meehan, Rt, Mendes, Ac, Meyer, Kc, Millar, Ab, Moğulkoc, N, Molitor, Ja, Morais, A, Mouthon, L, Müller, V, Müller Quernheim, J, Nadashkevich, O, Nador, R, Nash, P, Nathan, Sd, Navarro, C, Neves, S, Noth, I, Nunes, H, Olson, Al, Opitz, Cf, Padilla, M, Pappas, D, Parfrey, H, Pego Reigosa JM, Pereira, Ca, Perez, R, Pope, Je, Porter, Jc, Renzoni, Ea, Riemekasten, G, Riley, Dj, Rischmueller, M, Rodriguez Reyna TS, Rojas Serrano, J, Roman, J, Rosen, Gd, Rossman, M, Rothfield, N, Sahn, Sa, Sanduzzi, A, Scholand, Mb, Selman, M, Senécal, Jl, Seo, P, Shah, A, Silver, Rm, Solomon, Jj, Steen, V, Stevens, W, Strange, C, Sussman, R, Sutton, Ed, Sweiss, Nj, Tornling, G, Tzelepis, Ge, Undurraga, A, Vacca, A, Vancheri, Carlo, Varga, J, Veale, Dj, Volkov, S, Walker, Ua, Wells, Au, Wencel, M, Wesselius, Lj, Wickremasinghe, M, Wilcox, P, Wilsher, Ml, Wollheim, Fa, Wuyts, Wa, Yung, G, Zanon, P, Zappala, Cj, Groshong, Sd, Leslie, Ko, Myers, Jl, Padera, Rf, Desai, Sr, Goldin, J, Kazerooni, Ea, Klein, Js, and Keen, Kj

Subjects
Male, medicine.medical_specialty, Delphi Technique, Consensus Development Conferences as Topic, Health Personnel, Immunology, Context (language use), Disease, Severity of Illness Index, Article, Idiopathic pulmonary fibrosis, Rheumatology, medicine, Immunology and Allergy, Humans, Disease management (health), Intensive care medicine, Connective Tissue Diseases, Randomized Controlled Trials as Topic, business.industry, Interstitial lung disease, Disease Management, respiratory system, Focus Groups, medicine.disease, Comorbidity, Connective tissue disease, respiratory tract diseases, Clinical trial, Treatment Outcome, Patient Satisfaction, Physical therapy, Quality of Life, ÍNDICE DE GRAVIDADE DA DOENÇA, Interdisciplinary Communication, business, Lung Diseases, Interstitial
Abstract

Interstitial lung diseases (ILD), including those related to connective tissue disease (CTD), and idiopathic pulmonary fibrosis (IPF) carry high morbidity and mortality. Great efforts are under way to develop and investigate meaningful treatments in the context of clinical trials. However, efforts have been challenged by a lack of validated outcome measures and by inconsistent use of measures in clinical trials. Lack of consensus has fragmented effective use of strategies in CTD-ILD and IPF, with a history of resultant difficulties in obtaining agency approval of treatment interventions. Until recently, the patient perspective to determine domains and outcome measures in CTD-ILD and IPF had never been applied. Efforts described here demonstrate unequivocally the value and influence of patient involvement on core set development. Regarding CTD-ILD, this is the first OMERACT working group to directly address a manifestation/comorbidity of a rheumatic disease (ILD) as well as a disease not considered rheumatic (IPF). The OMERACT 11 proceedings of the CTD-ILD Working Group describe the forward and lateral process to include both the medical and patient perspectives in the urgently needed identification of a core set of preliminary domains and outcome measures in CTD-ILD and IPF.

Published
2014

19. Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: Provisional core sets of domains and instruments for use in clinical trials

Author

Saketkoo, La, Mittoo, S, Huscher, D, Khanna, D, Dellaripa, Pf, Distler, O, Flaherty, Kr, Frankel, S, Oddis, Cv, Denton, Cp, Fischer, A, Kowal Bielecka OM, Lesage, D, Merkel, Pa, Phillips, K, Pittrow, D, Swigris, J, Antoniou, K, Baughman, Rp, Castelino, Fv, Christmann, Rb, Christopher Stine, L, Collard, Hr, Cottin, V, Danoff, S, Highland, Kb, Hummers, L, Shah, Aa, Kim, Ds, Lynch, Da, Miller, Fw, Proudman, Sm, Richeldi, L, Ryu, Jh, Sandorfi, N, Sarver, C, Wells, Au, Strand, V, Matteson, El, Brown, Kk, Seibold, Jr, Aggarwal, R, Ainslie, G, Alkassab, F, Allanore, Y, Descartes, P, Anderson, Me, Andonopoulos, Ap, Antin Ozerkis, D, Arrobas, A, Ascherman, Dp, Assassi, S, Baron, M, Bathon, Jm, Behr, J, Beretta, L, Bingham, Co, Binnie, M, Birring, Ss, Boin, F, Bongartz, T, Bourdin, A, Bouros, D, Brasington, R, Bresser, P, Buch, Mh, Burge, Ps, Carmona, L, Carreira, Pe, Carvalho, Cr, Catoggio, Lj, Chan, Km, Chapman, J, Chatterjee, S, Chua, F, Chung, L, Conron, M, Corte, T, Cosgrove, G, Costabel, U, Cox, G, Crestani, B, Crofford, Lj, Csuka, Me, Curbelo, P, László, C, Daniil, Z, D'Arsigny, Cl, Davis, Gs, de Andrade JA, De Vuyst, P, Dempsey, Oj, Derk, Ct, Distler, J, Dixon, Wg, Downey, G, Doyle, Mk, Drent, M, Durairaj, L, Emery, P, Espinoza, Lr, Farge, D, Fathi, M, Fell, Cd, Fessler, Bj, Fitzgerald, Je, Fox, Ga, Foeldvari, I, Frech, Tm, Freitas, S, Furst, De, Gabrielli, A, García Vicuña, R, Georgiev, Ob, Gerbino, A, Gillisen, A, Gladman, Dd, Glassberg, M, Gochuico, Br, Gogali, A, Goh, Ns, Goldberg, A, Goldberg, Hj, Gourley, Mf, Griffing, L, Grutters, Jc, Gunnarsson, R, Hachulla, E, Hall, Fc, Harari, S, Herrick, Al, Herzog, El, Hesselstrand, R, Hirani, N, Hodgson, U, Hollingsworth, Hm, Homer, Rj, Hoyles, Rk, Hsu, Vm, Hubbard, Rb, Hunzelmann, N, Isasi, Me, Isasi, Es, Jacobsen, S, Jimenez, Sa, Johnson, Sr, Jones, Ch, Kahaleh, B, Kairalla, Ra, Kalluri, M, Kalra, S, Kaner, Rj, Kinder, Bw, Klingsberg, Rc, Kokosi, M, Kolb, Mr, Kur Zalewska, J, Kuwana, M, Lake, Fr, Lally, Ev, Lasky, Ja, Laurindo, Im, Able, L, Lee, P, Leonard, Ct, Lien, Dc, Limper, Ah, Liossis, Sn, Lohr, Km, Loyd, Je, Lundberg, Ie, Mageto, Yn, Maher, Tm, Mahmud, Th, Manganas, H, Marie, I, Marras, Tk, Antônio Baddini Martinez, J, Martinez, Fj, Mathieu, A, Matucci Cerinic, M, Mayes, Md, Mckown, Km, Medsger, Ta, Meehan, Rt, Cristina, Ma, Meyer, Kc, Millar, Ab, Moğulkoc, N, Molitor, Ja, Morais, A, Luc Mouthon, P, Müller, V, Müller Quernheim, J, Nadashkevich, O, Nador, R, Nash, P, Nathan, Sd, Navarro, C, Neves, S, Noth, I, Nunes, H, Olson, Al, Opitz, Cf, Padilla, M, Pappas, D, Parfrey, H, Pego Reigosa JM, Pereira, Ca, Perez, R, Pope, Je, Porter, Jc, Renzoni, Ea, Riemekasten, G, Riley, Dj, Rischmueller, M, Rodriguez Reyna TS, Rojas, Serrano, Roman, J, Rosen, Gd, Rossman, M, Rothfield, N, Sahn, Sa, Sanduzzi, A, Scholand, Mb, Selman, M, Senécal, Jl, Seo, P, Silver, Rm, Solomon, Jj, Steen, V, Stevens, W, Strange, C, Sussman, R, Sutton, Ed, Sweiss, Nj, Tornling, G, Tzelepis, Ge, Undurraga, A, Vacca, A, Vancheri, Carlo, Varga, J, Veale, Dj, Volkov, S, Walker, Ua, Wencel, M, Wesselius, Lj, Wickremasinghe, M, Wilcox, P, Wilsher, Ml, Wollheim, Fa, Wuyts, Wa, Yung, G, Zanon, P, Zappala, Cj, Groshong, Sd, Leslie, Ko, Myers, Jl, Padera, Rf, Desai, Sr, Goldin, J, Kazerooni, Ea, Klein, Js, Cenac, Sl, Grewal, Hk, Christensen, Am, Ferguson, S, Tran, M, Keen, K. J., Costabel, Ulrich (Beitragende*r), Raynauds & Scleroderma Association, Arthritis Research UK, The Scleroderma Society, and British Lung Foundation

Subjects
Lung Diseases, Connective tissue disease associated lung disease, CTD-ILD Special Interest Group, International Cooperation, Respiratory System, Medizin, Rheumatoid lung disease, Idiopathic pulmonary fibrosis, Quality of life, QUALITY-OF-LIFE, CYCLOPHOSPHAMIDE, SCLERODERMA LUNG, Registries, Connective Tissue Diseases, Societies, Medical, Randomized Controlled Trials as Topic, Interstitial lung disease, respiratory system, Connective tissue disease, Interstitial Fibrosis, medicine.anatomical_structure, Life Sciences & Biomedicine, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Consensus, Clinical Sciences, END-POINT, Interstitial Lung Disease, Systemic disease and lungs, Medical, medicine, Humans, ENSAIO CLÍNICO CONTROLADO RANDOMIZADO, VALIDITY, Intensive care medicine, Lung, Science & Technology, COUGH, business.industry, Clinical study design, MORTALITY, SYSTEMIC-SCLEROSIS, 1103 Clinical Sciences, Congresses as Topic, medicine.disease, GEORGES RESPIRATORY QUESTIONNAIRE, respiratory tract diseases, Clinical trial, IPF, Physical therapy, Interstitial, Societies, business, Lung Diseases, Interstitial
Abstract

Rationale: Clinical trial design in interstitial lung diseases (ILDs) has been hampered by lack of consensus on appropriate outcome measures for reliably assessing treatment response. In the setting of connective tissue diseases (CTDs), some measures of ILD disease activity and severity may be confounded by non-pulmonary comorbidities. Methods: The Connective Tissue Disease associated Interstitial Lung Disease (CTD-ILD) working group of Outcome Measures in Rheumatology-a non-profit international organisation dedicated to consensus methodology in identification of outcome measures-conducted a series of investigations which included a Delphi process including >248 ILD medical experts as well as patient focus groups culminating in a nominal group panel of ILD experts and patients. The goal was to define and develop a consensus on the status of outcome measure candidates for use in randomised controlled trials in CTD-ILD and idiopathic pulmonary fibrosis (IPF). Results: A core set comprising specific measures in the domains of lung physiology, lung imaging, survival, dyspnoea, cough and health-related quality of life is proposed as appropriate for consideration for use in a hypothetical 1-year multicentre clinical trial for either CTD-ILD or IPF. As many widely used instruments were found to lack full validation, an agenda for future research is proposed. Conclusion: Identification of consensus preliminary domains and instruments to measure them was attained and is a major advance anticipated to facilitate multicentre RCTs in the field.

Published
2014

20. There is a need for new systemic sclerosis subset criteria. A content analytic approach.

Author

Johnson, SR, Soowamber, ML, Fransen, J, Khanna, D, Van Den Hoogen, F, Baron, M, Matucci-Cerinic, M, Denton, CP, Medsger, TA, Carreira, PE, Riemekasten, G, Distler, J, Gabrielli, A, Steen, V, Chung, L, Silver, R, Varga, J, Müller-Ladner, U, Vonk, MC, and Walker, UA

Subjects
NOSOLOGY, SYSTEMIC scleroderma, AUTOANTIBODIES, MEDICAL communication, CLUSTER analysis (Statistics), PROGNOSIS, THERAPEUTICS, RISK assessment, CROSS-sectional method, DISEASE progression, DIAGNOSIS
Abstract

Objectives: Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets.Methods: We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed.Results: Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5 years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly).Conclusions: We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies). [ABSTRACT FROM AUTHOR]

Published
2018
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21. Causes and risk factors for death in systemic sclerosis – A study from the EULAR Scleroderma Trials And Research (EUSTAR) data base

Author

Tyndall, Aj, Bannert, B, Vonk, M., Airò, P, Cozzi, F, Carreira, Pe, Farge Bancel, D, Allanore, Y, Müller Ladner, U, Distler, O, Iannone, F, Pellerito, F, Pileckyte, M, Miniati, I, Ananieva, L, Balbir Gurman, A, Damjanov, N, Mueller, A, Valentini, G, Riemekasten, G, Tikly, M, Hummers, L, Henriques, Mjs, Caramaschi, Paola, Scheja, A, Rozman, B, Ton, E, Kumánovics, G, Coleiro, B, Feierl, E, Szucs, G, Von Mühlen CA, Riccieri, V, Novak, S, Chizzolini, C, Kotulska, A, Denton, C, Coelho, Pc, Kötter, I, Simsek, I, De la Pena Lefebvre PC, Hachulla, E, Seibold, Jr, Rednic, S, Štork, J, Morovic Vergles, J, and Walker, Ua

Subjects
pulmonary fibrosis, Systemic sclerosis, pulmonary artery hypertension
Published
2010

23. Multicenter longitudinal study of B-lymphocyte depletion in refractory systemic lupus erythematosus: the LESIMAB study

Author

Fernández-Nebro, A, primary, de la Fuente, JL Marenco, additional, Carreño, L, additional, Izquierdo, M Galindo, additional, Tomero, E, additional, Rúa-Figueroa, I, additional, Hernández-Cruz, BE, additional, Narváez, J, additional, Úcar, E, additional, Olivé, A, additional, Zea, A, additional, Fernández-Castro, M, additional, Raya-Álvarez, E, additional, Pego-Reigosa, JM, additional, Freire, M, additional, Martínez-Taboada, VM, additional, Pérez-Venegas, J, additional, Sánchez-Atrio, AI, additional, Villa-Blanco, I, additional, Manrique-Arija, S, additional, López-Longo, FJ, additional, Carreira, PE, additional, Martínez-Pérez, R, additional, and García-Vicuña, R, additional

Published
2012
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25. Polymorphism of the interleukin-1 receptor antagonist gene: a factor in susceptibility to rheumatoid arthritis in a Spanish population.

Author

Carreira PE, Gonzalez-Crespo MR, Ciruelo E, Pablos JL, Santiago B, Gomez-Camara A, and Gomez-Reino JJ

Abstract

OBJECTIVE: To assess whether an interleukin-1 receptor antagonist gene (IL1RN) polymorphism is associated with disease susceptibility and/or severity in a Spanish population of patients with rheumatoid arthritis (RA). METHODS: An 86-bp variable-number tandem repeat polymorphism within IL1RN intron 2 was analyzed by polymerase chain reaction in genomic DNA obtained from 247 unrelated patients with RA (group A) and 287 healthy control subjects. The polymorphism analysis was repeated in a second group of 194 patients with RA (group B). Clinical information from patients in group A was used to compare activity and severity data in patients stratified according to the different alleles or genotypes. Odds ratios (ORs) with 95% confidence intervals (95% CIs) were used to determine the strength of the association of the different alleles or genotypes with RA activity or severity. RESULTS: In the control group, the allelic frequencies were 76% for IL1RN*1 (4 repeats), 21% for IL1RN*2 (2 repeats), 3% for IL1RN*3 (5 repeats), and 0.3% for IL1RN*4 (3 repeats). In group A patients with RA, both the frequency (OR 1.47, 95% CI 1.1-1.96, P = 0.007) and carriage rate (OR 1.6, 95% CI 1.1-2.2, P = 0.01) of allele IL1RN*2 were significantly increased. The increased frequency of IL1RN*2 was confirmed in group B patients with RA (OR 1.44, 95% CI 1.1-1.97, P = 0.01). In patients with RA, homozygosity for IL1RN*2 was associated with an increased number of affected articular areas during the first year of followup but not with other parameters of disease activity or severity. CONCLUSION: Our results suggest that IL1RN has a role in determining susceptibility to RA in the Spanish population. [ABSTRACT FROM AUTHOR]

Published
2005
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27. Overexpression of the Cytokine BAFF and Autoimmunity Risk

Author

Steri, M, Orrù, V, Idda, ML, Pitzalis, M, Pala, M, Zara, I, Sidore, C, Faà, V, Floris, M, Deiana, M, Asunis, I, Porcu, E, Mulas, A, Piras, MG, Lobina, M, Lai, S, Marongiu, M, Serra, V, Sole, G, Busonero, F, Maschio, A, Cusano, R, Cuccuru, G, Deidda, F, Poddie, F, Farina, G, Dei, M, Virdis, F, Olla, S, Satta, MA, Pani, M, Delitala, A, Cocco, E, Frau, J, Coghe, G, Lorefice, L, Fenu, G, Ferrigno, P, Ban, M, Barizzone, N, Leone, M, Guerini, FR, Piga, M, Firinu, D, Kockum, I, Lima Bomfim, I, Olsson, T, Alfredsson, L, Suarez, A, Carreira, PE, Castillo-Palma, MJ, Marcus, JH, Congia, M, Angius, A, Melis, M, Gonzalez, A, Alarcón Riquelme, ME, Da Silva, BM, Marchini, M, Danieli, MG, Del Giacco, S, Mathieu, A, Pani, A, Montgomery, SB, Rosati, G, Hillert, J, Sawcer, S, D'Alfonso, S, Todd, JA, Novembre, J, Abecasis, GR, Whalen, MB, Marrosu, MG, Meloni, A, Sanna, S, Gorospe, M, Schlessinger, D, Fiorillo, E, Zoledziewska, M, and Cucca, F

Subjects
transcription, genetic, phenotype, case-control studies, gene Expression, autoimmunity, polymorphism, single nucleotide, multiple sclerosis, sequence analysis, RNA, 3. Good health, MicroRNAs, INDEL Mutation, Italy, B-Cell Activating Factor, Lupus Erythematosus, Systemic, humans, Genome-Wide Association Study, risk
Abstract

$\textbf{BACKGROUND}$: Genomewide association studies of autoimmune diseases have mapped hundreds of susceptibility regions in the genome. However, only for a few association signals has the causal gene been identified, and for even fewer have the causal variant and underlying mechanism been defined. Coincident associations of DNA variants affecting both the risk of autoimmune disease and quantitative immune variables provide an informative route to explore disease mechanisms and drug-targetable pathways. $\textbf{METHODS}$: Using case-control samples from Sardinia, Italy, we performed a genomewide association study in multiple sclerosis followed by TNFSF13B locus-specific association testing in systemic lupus erythematosus (SLE). Extensive phenotyping of quantitative immune variables, sequence-based fine mapping, cross-population and cross-phenotype analyses, and gene-expression studies were used to identify the causal variant and elucidate its mechanism of action. Signatures of positive selection were also investigated. $\textbf{RESULTS}$: A variant in TNFSF13B, encoding the cytokine and drug target B-cell activating factor (BAFF), was associated with multiple sclerosis as well as SLE. The disease-risk allele was also associated with up-regulated humoral immunity through increased levels of soluble BAFF, B lymphocytes, and immunoglobulins. The causal variant was identified: an insertion-deletion variant, GCTGT→A (in which A is the risk allele), yielded a shorter transcript that escaped microRNA inhibition and increased production of soluble BAFF, which in turn up-regulated humoral immunity. Population genetic signatures indicated that this autoimmunity variant has been evolutionarily advantageous, most likely by augmenting resistance to malaria. $\textbf{CONCLUSIONS}$: A TNFSF13B variant was associated with multiple sclerosis and SLE, and its effects were clarified at the population, cellular, and molecular levels. (Funded by the Italian Foundation for Multiple Sclerosis and others.).

28. Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database

Author

José Luis Andreu, Alexandra Balbir-Gurman, Roger Hesselstrand, Simona Rednic, Mohammed Tikly, G. Riemekasten, Paola Caramaschi, Serena Vettori, Marco Matucci-Cerinic, Dominique Farge, Yannick Allanore, Nemanja Damjanov, L. Czirják, Ada Corrado, Beatriz Joven, Loreto Carmona, Estíbaliz Loza, Paolo Airò, Ulrich A. Walker, Oliver Distler, Patricia Carreira, Lidiya P. Ananieva, Carreira, Pe, Carmona, L, Joven, Be, Loza, E, Andréu, Jl, Riemekasten, G, Vettori, S, Balbir-Gurman, A, Airò, P, Walker, U, Damjanov, N, Matucci-Cerinic, M, Ananieva, Lp, Rednic, S, Czirják, L, Distler, O, Farge, D, Hesselstrand, R, Corrado, A, Caramaschi, P, Tikly, M, and Allanore, Y

Subjects
Adult, Male, medicine.medical_specialty, Databases, Factual, Cross-sectional study, Immunology, Disease, Scleroderma, 03 medical and health sciences, Age Distribution, 0302 clinical medicine, Rheumatology, Internal medicine, Cardiac conduction, Prevalence, medicine, Humans, Immunology and Allergy, Registries, 030212 general & internal medicine, Age of Onset, Sex Distribution, skin and connective tissue diseases, Retrospective Studies, 030203 arthritis & rheumatology, Scleroderma, Systemic, integumentary system, business.industry, Incidence, Incidence (epidemiology), Age Factors, Retrospective cohort study, General Medicine, Middle Aged, medicine.disease, 3. Good health, Europe, Cross-Sectional Studies, Female, Age of onset, business
Abstract

Objective: The aim of this study was to analyse differences in clinical presentation in patients with early (< 3 years’ duration) systemic sclerosis (SSc), comparing three age groups according to disease subsets. Method: Cross-sectional analysis of the prospective EULAR Scleroderma Trials and Research database (EUSTAR) was performed. Patients fulfilling preliminary American College of Rheumatology 1980 classification criteria for SSc, with < 3 years from the first non-Raynaud’s SSc symptom at first entry, were selected. Patients with < 3 years from the first SSc symptom, including Raynaud’s phenomenon, were also analysed. SSc-related variables, including antibodies, SSc subsets, and organ involvement, were examined. Age was categorized into ≤ 30, 31–59, and ≥ 60 years. We performed descriptive and bivariate analyses. Results: The study included 1027 patients: 90% Caucasian, 80% women, and 40% with diffuse disease. In early stages of SSc, younger patients had significantly more anti-Scl-70 antibodies and diffuse disease. With increasing age, we observed more elevation of estimated pulmonary systolic pressure on echocardiography (5%, 13%, and 30%, respectively, in the three age groups), cardiac conduction blocks (6%, 6%, and 15%), and left ventricular diastolic dysfunction (4%, 12%, and 27%). The results were similar for 650 patients with < 3 years from first SSc symptom, including Raynaud’s. Conclusion: In early stages of SSc, older patients showed data indicating more severe disease with greater cardiac involvement. The diffuse subset was more frequent in the younger subgroup. The identification of such differences may help in selecting appropriate management for individual patients in clinical practice. (Less)

Published
2018

29. Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population

Author

Hachulla, Eric, Clerson, Pierre, Airò, Paolo, Cuomo, Giovanna, Allanore, Yannick, Caramaschi, Paola, Rosato, Edoardo, Carreira, Patricia E., Riccieri, Valeria, Sarraco, Marta, Denton, Christopher P., Riemekasten, Gabriela, Pozzi, Maria Rosa, Zeni, Silvana, Mihai, Carmen Marina, Ullman, Susanne, Distler, Oliver, Rednic, Simona, Smith, Vanessa, Walker, Ulrich A., Matucci-Cerinic, Marco, Müller-Ladner, Ulf, Launay, David, Bellando Randone, S, Guiducci, S, University of Zurich, Hachulla, Eric, Hachulla, E, Clerson, P, Airò, P, Cuomo, Giovanna, Allanore, Y, Caramaschi, P, Rosato, E, Carreira, Pe, Riccieri, V, Sarraco, M, Denton, Cp, Riemekasten, G, Pozzi, Mr, Zeni, S, Mihai, Cm, Ullman, S, Distler, O2, Rednic, S2, Smith, V2, Walker, Ua, Matucci Cerinic, M, Müller Ladner, U, Launay, D1, and on behalf of the EUSTAR co, Workers

Subjects
Male, European League Against Rheumatism Scleroderma Trial and Research, systemic sclerosis, 2745 Rheumatology, MULTICENTER, systolic pulmonary artery pressure, Blood Pressure, Systemic scleroderma, Scleroderma, Cohort Studies, Systemic sclerosi, DLCO, Risk Factors, Diffusing capacity, pulmonary hypertension, survival, systolic pulmonary arterial pressure, tricuspid regurgitant jet velocity, Medicine and Health Sciences, Medicine, 2736 Pharmacology (medical), Pharmacology (medical), education.field_of_study, 10051 Rheumatology Clinic and Institute of Physical Medicine, Pulmonary, Orvostudományok, Clinical Science, Middle Aged, Prognosis, Europe, Survival Rate, Echocardiography, Hypertension, Cardiology, SURVIVAL, Female, Adult, medicine.medical_specialty, DATABASE, Systole, Hypertension, Pulmonary, Population, Pulmonary hypertension, Survival, Systemic sclerosis, Systolic pulmonary arterial pressure, Tricuspid regurgitant jet velocity, Aged, Follow-Up Studies, Humans, Multivariate Analysis, Pulmonary Artery, Retrospective Studies, Scleroderma, Systemic, Rheumatology, 610 Medicine & health, Klinikai orvostudományok, DIAGNOSIS, Internal medicine, Risk factor, education, Survival rate, HYPERTENSION, business.industry, Systemic, medicine.disease, stomatognathic diseases, Blood pressure, EULAR SCLERODERMA TRIALS, business
Abstract

Objective. The aim of this study was to assess the prognostic value of systolic pulmonary artery pressure (sPAP) estimated by echocardiography in the multinational European League Against Rheumatism Scleroderma Trial and Research (EUSTAR) cohort. Methods. Data for patients with echocardiography documented between 1 January 2005 and 31 December 2011 were extracted from the EUSTAR database. Stepwise forward multivariable statistical Cox pulmonary hypertension analysis was used to examine the independent effect on survival of selected variables. Results. Based on our selection criteria, 1476 patients were included in the analysis; 87% of patients were female, with a mean age of 56.3 years (S.D. 13.5) and 31% had diffuse SSc. The mean duration of follow-up was 2.0 years (S.D. 1.2, median 1.9). Taking index sPAP of 50 mmHg. In a multivariable Cox model, sPAP and the diffusing capacity for carbon monoxide (DLCO) were independently associated with the risk of death [HR 1.833 (95% CI 1.035, 3.247) and HR 0.973 (95% CI 0.955, 0.991), respectively]. sPAP was an independent risk factor for death with a HR of 3.02 (95% CI 1.91, 4.78) for sPAP >= 36 mmHg. Conclusion. An estimated sPAP >36mmHg at baseline echocardiography was significantly and independently associated with reduced survival, regardless of the presence of pulmonary hypertension based on right heart catheterization.

Published
2015

30. Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis. a 10-year longitudinal study from the EUSTAR database

Author

Wirz, E. G., Jaeger, V. K., Allanore, Y., Riemekasten, G., Hachulla, E., Distler, O., Airo, P., Carreira, P. E., Tikly, M., Vettori, S., Gurman, A. B., Damjanov, N., Muller-Ladner, U., Distler, J., Li, M., Hausermann, P., Walker, U. A., Ananieva, L., Heitmann, S., Rednic, S., Jimenez, S., Riccieri, V., Szmyrka-Kaczmarek, M., Farge, D., Lapadula, G., Matucci-Cerinic, M., Guiducci, S., Hunzelmann, N., Rosa Pozzi, M., Mihai, C., Veale, D., Hesselstrand, R., Mariok, E., Smith, V., Kucharz, E. J., Czirjak, L., Martinovic, D., Solanki, K., Mihaela Ancuta, C., Sibilia, J., Paola, C., Hassanien, M., Kahl, S., Woods, A., Vanthuyne, M., Ruxandra, I., Radominski, S. C., Lo Monaco, A., Corrado, A., Koehm, M., Maurizio, M., Radim, B., Loyo, E., Uprus, M., Pellerito, R., Zenone, T., Gabrielli, A., Kowal-Bielecka, O., Rozman, B., Scorza, R., Ann Saketkoo, L., Midtvedt, O., von Muhlen, C. A., Henes, J., Branimir, A., Hasler, P., Yavuz, S., Villiger, P., Krummel-Lorenz, B., Posa, M., Engelhart, M., Denton, C., Krasowska, D., de la Pena Lefebvre, P. G., Cozzi, F., Mouthon, L., Rosato, E., Carlo, S., Alegre Sancho, J. J., Mallia, C., Limonta, M., Seidel, M., Foti, R., Stamp, L., Ullman, S., Stebbings, S., Ortiz Santamaria, V., Del Galdo, F., De Langhe, E., Mathieu, A., Sunderkotter, C., Eyerich, K., Stamenkovic, B., Novak, S., Sampaio-Barros, P. D., Kayser, C., Litinsky, I., Couto, M., University of Zurich, Walker, U A, Wirz, Eg, Jaeger, Vk, Allanore, Y, Riemekasten, G, Hachulla, E, Distler, O, Airò, P, Carreira, Pe, Tikly, M, Vettori, Serena, Balbir Gurman, A, Damjanov, N, Müller Ladner, U, Distler, J, Li, M, Häusermann, P, Walker, Ua, and Eustar, Coauthors

Subjects
Male, Genetics and Molecular Biology (all), Pathology, Longitudinal study, Time Factors, Databases, Factual, Epidemiology, systemic sclerosis, 2745 Rheumatology, Kaplan-Meier Estimate, Severity of Illness Index, Biochemistry, Scleroderma, Risk Factors, Medizinische Fakultät, Immunology and Allergy, Longitudinal Studies, Prospective Studies, 610 Medicine & health, integumentary system, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Connective tissue disease, 3. Good health, Autoantibodies, Systemic Sclerosis, Cohort, 2723 Immunology and Allergy, Female, Adult, medicine.medical_specialty, Immunology, General Biochemistry, Genetics and Molecular Biology, Sex Factors, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Skin Ulcer, medicine, Humans, ddc:610, Proportional Hazards Models, 2403 Immunology, Scleroderma, Systemic, business.industry, medicine.disease, Clinical trial, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Diffuse, business
Abstract

Objectives To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud9s phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan–Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.

Published
2016

31. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study

Author

F. Lauffer, Kati Otsa, Oliver Distler, S. Zeni, Marco Matucci Cerinic, Maria Rosa Pozzi, Margarita Pileckyte, John Highton, Paola Caramaschi, Jacek Szechiński, Maria João Salvador, Diana Karpec, Maurizio Cutolo, Codrina Ancuta, Patrizia Boracchi, Simonetta Pisarri, Fabiana Montoya, Vanessa Smith, Mengtao Li, Carolina de Souza Müller, Patricia Carreira, C. Mihai, Henrik Nielsen, Luc Mouthon, L. Denisov, Marc Frerix, Pier Luigi Meroni, Øyvind Midtvedt, Francesco Paolo Cantatore, Ada Corrado, Sebastião Cezar Radominski, Serena Guiducci, Francesco Puppo, Simon Stebbings, Armando Gabrielli, Giovanna Cuomo, Irena Butrimiene, Piotr Wiland, Ira Litinsky, Maria Uprus, Merete Engelhart, Roger Hesselstrand, Ulrich A Walker, Rodica Chirieac, Ulf Müller-Ladner, David Launay, Kirsten Damgaard, Kamal Solanki, Cristina Mihaela Tanaseanu, Torhild Garen, Isabela Tiglea, Aleksandra Stanković, L. Ananieva, Francesca Ingegnoli, Magdalena Szmyrka-Kaczmarek, Jörg Henes, Alan Tyndall, Roberta Gualtierotti, Rüdiger Hein, Ewa Morgiel, Edoardo Rosato, Ivan Foeldvari, Valderílio Feijó Azevedo, Gitte Strauss, Valeria Riccieri, Anna Kotulska, Marta Valero Exposito, R. Becvar, José António Pereira da Silva, Blaz Rozman, Vera Ortiz-Santamaria, Paloma García de la Peña Lefebvre, Szilvia Szamosi, Małgorzata Widuchowska, Gabriella Szücs, Martin Aringer, Paulius Venalis, Roberto Caporali, Kilian Eyerich, Florenzo Iannone, Alina Dumitrascu, Eugene J. Kucharz, Laura Groseanu, Alessandra Vacca, Monica Popescu, Cristiane Kayser, Yannick Allanore, Brigitte Krummel-Lorenz, P. Saar, Mihai Bojinca, Magdalena Kopec-Medrek, Eduardo Kerzberg, Cecília Varjú, Nemanja Damjanov, Luis Eduardo Coelho Andrade, Rita Rugiene, Paolo Airò, Filip De Keyser, Nicola Ughi, Bojana Stamenkovic, Claudia Günther, Ruxandra Ionescu, László Czirják, Matthias Seidel, Silvia Rodriguez Rubio, Paola Gottschalk, Dirk M. Wuttge, Alan Doube, Vanesa Cosentino, Thierry Zenone, Dominique Farge-Bancel, Esthela Loyo, Algirdas Venalis, Renata Sokolik, Alberto Sulli, Rosario Foti, Stefan Heitmann, Eric Hachulla, Juan José Alegre-Sancho, Carlomaurizio Montecucco, Daniela Opris, Ingegnoli, F, Boracchi, P, Gualtierotti, R, Smith, V, Cutolo, M, Foeldvari, I, Airò, P, Alegre-Sancho, Jj, Allanore, Y, Ananieva, Lp, Ancuta, C, Andrade, Le, Aringer, M, Becvar, R, Bojinca, M, Butrimiene, I, Cantatore, Fp, Caporali, R, Caramaschi, P, Carreira, Pe, Chirieac, R, Corrado, A, Cosentino, V, Cuomo, G, Czirjak, L, Da Silva, Ja, la Peña Lefebvre, Pg, De Keyser, F, de Souza Müller, C, Damgaard, K, Damjanov, N, Denisov, Ln, Distler, O, Doube, A, Dumitrascu, A, Engelhart, M, Exposito, Mv, Eyerich, K, Farge-Bancel, D, Azevedo, Vf, Foti, R, Frerix, M, Gabrielli, A, Garen, T, Gottschalk, P, Groseanu, L, Guiducci, S, Günther, C, Hachulla, Hein, R, Heitmann, S, Henes, J, Hesselstrand, R, Highton, J, Iannone, F, Ionescu, Rm, Kayser, C, Karpec, D, Kerzberg, E, Kotulska, A, Kopec-Medrek, M, Kucharz, E, Krummel-Lorenz, B, Lauffer, F, Launay, D, Li, M, Litinsky, I, Loyo, E, Cerinic, Mm, Meroni, P, Midtvedt, Ø, Mihai, Cm, Montecucco, C, Montoya, F, Morgiel, E, Mouthon, L, Müller-Ladner, U, Nielsen, H, Opris, D, Ortiz-Santamaria, V, Otsa, K, Pileckyte, M, Pisarri, S, Popescu, M, Pozzi, Mr, Puppo, F, Radominski, Sc, Riccieri, V, Rosato, E, Rozman, B, Rubio, Sr, Rugiene, R, Saar, P, Salvador, Mj, Seidel, M, Sokolik, R, Solanki, K, Stamenkovic, B, Stankovic, A, Stebbings, S, Strauss, G, Sulli, A, Szamosi, S, Szechinski, J, Szmyrka-Kaczmarek, M, Szücs, G, Tanaseanu, Cm, Tiglea, I, Tyndall, A, Ughi, N, Uprus, M, Vacca, A, Varju, C, Venalis, A, Venalis, P, Walker, Ua, Widuchowska, M, Wiland, P, Wuttge, Dm, Zeni, S, and Zenone, T.

Subjects
Adult, Male, medicine.medical_specialty, Pathology, Adolescent, Klinikai orvostudományok, Biochemistry, Juvenile systemic sclerosi, Scleroderma, Microscopic Angioscopy, Systemic sclerosi, Scleroderma, Localized, Young Adult, Medicine, Juvenile, Humans, Young adult, Age of Onset, skin and connective tissue diseases, Child, Nailfold Capillaroscopy, Videocapillaroscopy, Aged, Retrospective Studies, EUSTAR, Scleroderma, Systemic, integumentary system, Capillaroscopy, business.industry, Similar distribution, Microcirculation, Autoantibody, Retrospective cohort study, Orvostudományok, Cell Biology, Middle Aged, medicine.disease, Dermatology, Capillaries, Nailfold capillaroscopy, Female, Age of onset, Cardiology and Cardiovascular Medicine, business, Juvenile systemic sclerosis, Systemic sclerosis
Abstract

Objective: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. Methods: Data collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. Results: 30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% Cl 0.57-10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% Cl 0.28-2.93. The late scleroderma pattern was present in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% Cl 0.34-3.56. Conclusion: This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of sderoderma pattern, but a similar distribution of the three patterns was suggested. Further studies are needed to define this issue. (C) 2015 Elsevier Inc. All rights reserved.

Published
2015

32. A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study

Author

Antonio C. Zea Mendoza, Jean Sibilia, Kamal Solanki, Cristina Mihaela Tanaseanu, Fredrick M. Wigley, Guido Valesini, M. Govoni, Lisa K. Stamp, Christopher P. Denton, Yolanda Braun-Moscovici, Ruxandra Ionescu, Øyvind Midtvedt, Ileana Nicoara, Aleksandra Stanković, Rüdiger Hein, Alina Dumitrascu, Susanne Ullman, Alan Tyndall, Sergio A. Jimenez, Irena Butrimiene, Alan Doube, Eugene J. Kucharz, Mohammed Tikly, Pier Luigi Meroni, Simon Stebbings, Renata Sokolik, Alexandra Balbir Gurman, Roger Hesselstrand, Kirsten Damgaard, Francesco Paolo Cantatore, Razvan Ionitescu, Silvana Zeni, Marco Matucci-Cerinic, Maria Rosa Pozzi, Jacques-Eric Gottenberg, Srdan Novak, Ana Maria Gherghe, David Launay, Liliana Groppa, Carlomaurizio Montecucco, Roxana Sfrent Cornateanu, Stefan Heitmann, Paloma García de la Peña Lefebvre, Daniela Opris, Peter T. Chapman, Line V. Iversen, Bernard Coleiro, Ulf Müller-Ladner, John Highton, Mara Oleszowsky, Gabriella Szücs, Magdalena Kopec-Medrek, Carlos De La Puente Buijdos, Paola Caramaschi, Magdalena Szmyrka-Kaczmarek, Rucsandra Dobrota, Gabriele Valentini, Fabiana Montoya, Blaz Rozman, Alberto Sulli, Hélène Chifflot, Raffaella Scorza, Patricia Carreira, Paulius Venalis, Lisa Maria Bambara, Torhild Garen, Isabela Tiglea, Agneta Scheja, Duska Martinovic, Jörg H W Distler, Jörg Henes, Giovanni Lapadula, Luc Mouthon, Diana Karpec, Douglas J. Veale, Valeria Riccieri, Nicolas Hunzelmann, Muriel Elhai, Serena Guiducci, Codrina Ancuta, Simonetta Pisarri, Thierry Zenone, Esthela Loyo, Branimir Anić, Claudia Günther, R. Becvar, Eugen Russu, Serena Vettori, Carlo Chizzolini, Vanessa Smith, Mengtao Li, Stanislaw Sierakowsky, Carmel Mallia, Małgorzata Widuchowska, Carolina de Souza Müller, László Czirják, Algirdas Venalis, Adrian Hij, Marta Valero Exposito, Simona Rednic, Miroslav Mayer, Laura Groseanu, Walter Alberto Sifuentes Giraldo, Murray Baron, Dominique Farge, Anna Kotulska, Marko Baresic, Svetlana Agachi, Martin Aringer, Merete Engelhart, John L. O'Donnell, Jérôme Avouac, Filip De Keyser, Ulrich A. Walker, Roberto Caporali, Harald Burkhardt, P. G. Vlachoyiannopoulos, Maurizio Cutolo, Frank A. Wollheim, Edoardo Rosato, Suzanne Kafaja, Valderílio Feijó Azevedo, Kilian Eyerich, Paolo Airò, Emmanuel Chatelus, L. Ananieva, Ira Litinsky, Andrea Lo Monaco, Vanesa Cosentino, Rita Rugiene, Eric Hachulla, P. Saar, Bojana Stamenkovic, Brigitte Krummel-Lorenz, Yannick Allanore, Elisabeth Knott, Oliver Distler, Matthias Seidel, Silvia Rodriguez Rubio, Franco Cozzi, Mihai Bojinca, Nemanja Damjanov, Maria João Salvador, Joanna Busquets, Otylia Kowal Bielecka, André Kahan, Jacek Szechiński, Daniel E. Furst, C. Mihai, Rodica Chirieac, Ewa Morgiel, Georg Schett, Armando Gabrielli, Giovanna Cuomo, Piotr Wiland, Maya N. Starovoytova, Sebastião Cezar Radominski, Gitte Strauss, Lealea Chiaburu, Florenzo Iannone, Carol M. Black, Andrea Himsel, Eduardo Kerzberg, Cecília Varjú, Vera Ortiz Santamaria, Gabriela Riemekasten, Dorota Krasowska, Marilena Gorga, Monica Popescu, Marie O'Rourke, Henrik Nielsen, Raffaele Pellerito, Ada Corrado, Elhai, M, Avouac, J, Walker, Ua, Matucci Cerinic, M, Riemekasten, G, Airò, P, Hachulla, E, Valentini, Gabriele, Carreira, Pe, Cozzi, F, Balbir Gurman, A, Braun Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller Ladner, U, Kahan, A, Distler, O, Allanore, Y, EUSTAR co, Author, EUSTAR co, Authors, Matucci-Cerinic, M, Airo, P, Valentini, G, Gurman, Ab, Braun-Moscovici, Y, Mt, Li, Muller-Ladner, U, Allanore, Y EUSTAR co-authors: Serena Guiducci, Alan, Tyndall, Giovanni, Lapadula, Florenzo, Iannone, Radim, Becvar, Stanislaw, Sierakowsky, Otylia Kowal Bielecka, Maurizio, Cutolo, Alberto, Sulli, Cuomo, Giovanna, Vettori, Serena, Simona, Rednic, Ileana, Nicoara, Vlachoyiannopoulos, P, Montecucco, C, Roberto, Caporali, Srdan, Novak, László, Czirják, Cecilia, Varju, Carlo, Chizzolini, Eugene, J Kucharz, Anna, Kotulska, Magdalena, Kopec-Medrek, Malgorzata, Widuchowska, Blaz, Rozman, Carmel, Mallia, Bernard, Coleiro, Armando, Gabrielli, Dominique, Farge, Adrian, Hij, Roger, Hesselstrand, Agneta, Scheja, Frank, Wollheim, Duska, Martinovic, Govoni, M, Andrea Lo Monaco, Nicolas, Hunzelmann, Raffaele, Pellerito, Lisa Maria Bambara, Paola, Caramaschi, Carol, Black, Christopher, Denton, Jörg, Hene, Vera Ortiz Santamaria, Stefan, Heitmann, Dorota, Krasowska, Matthias, Seidel, Mara, Oleszowsky, Harald, Burkhardt, Andrea, Himsel, Maria, J Salvador, Bojana, Stamenkovic, Aleksandra, Stankovic, Mohammed, Tikly, Maya, N Starovoytova, Merete, Engelhart, Gitte, Strau, Henrik, Nielsen, Kirsten, Damgaard, Gabriella, Szüc, Antonio Zea Mendoza, Carlos de la Puente Buijdos, Walter, A Sifuentes Giraldo, Øyvind, Midtvedt, Torhild, Garen, David, Launay, Guido, Valesini, Valeria, Riccieri, Ruxandra Maria Ionescu, Daniela, Opri, Laura, Groseanu, Fredrick, M Wigley, Carmen, M Mihai, Roxana Sfrent Cornateanu, Razvan, Ionitescu, Ana Maria Gherghe, Marilena, Gorga, Rucsandra, Dobrota, Mihai, Bojinca, Georg, Schett, Jörg Hw Distler, Pierluigi, Meroni, Silvana, Zeni, Luc, Mouthon, Filip De Keyser, Vanessa, Smith, Francesco, P Cantatore, Ada, Corrado, Susanne, Ullman, Line, Iversen, Maria, R Pozzi, Kilian, Eyerich, Rüdiger, Hein, Elisabeth, Knott, Jacek, Szechinski, Piotr, Wiland, Magdalena, Szmyrka-Kaczmarek, Renata, Sokolik, Ewa, Morgiel, Brigitte, Krummel-Lorenz, Petra, Saar, Martin, Aringer, Claudia, Günther, Branimir, Anic, Marko, Baresic, Miroslav, Mayer, Sebastião, C Radominski, Carolina de Souza Müller, Valderílio, F Azevedo, Svetlana, Agachi, Liliana, Groppa, Lealea, Chiaburu, Eugen, Russu, Thierry, Zenone, Simon, Stebbing, John, Highton, Lisa, Stamp, Peter, Chapman, Murray, Baron, John, O'Donnell, Kamal, Solanki, Alan, Doube, Douglas, Veale, Marie, O'Rourke, Esthela, Loyo, Edoardo, Rosato, Simonetta, Pisarri, Cristina-Mihaela, Tanaseanu, Monica, Popescu, Alina, Dumitrascu, Isabela, Tiglea, Rodica, Chirieac, Codrina, Ancuta, Daniel, E Furst, Suzanne, Kafaja, Paloma García de la Peña Lefebvre, Silvia Rodriguez Rubio, Marta Valero Exposito, Jean, Sibilia, Emmanuel, Chatelu, Jacques Eric Gottenberg, Hélène, Chifflot, Ira, Litinsky, Algirdas, Venali, Irena, Butrimiene, Paulius, Venali, Rita, Rugiene, Diana, Karpec, Eduardo, Kerzberg, Fabiana, Montoya, Vanesa, Cosentino, and Chizzolini, Carlo

Subjects
0301 basic medicine, Male, heart dysfunction, Databases, Factual, Epidemiology, autoimmune diseases, epidemiology, systemic sclerosis, Kaplan-Meier Estimate, 0302 clinical medicine, Cardiovascular Disease, Immunology and Allergy, Prospective Studies, Age of Onset, skin and connective tissue diseases, Prospective cohort study, ddc:616, Orvostudományok, Middle Aged, Prognosis, Connective tissue disease, 3. Good health, Europe, Cardiovascular Diseases, Cohort, Disease Progression, Female, Autoimmune Diseases, Systemic Sclerosis, Human, Adult, medicine.medical_specialty, Prognosi, Immunology, Socio-culturale, Klinikai orvostudományok, Autoimmune Disease, General Biochemistry, Genetics and Molecular Biology, Follow-Up Studie, 03 medical and health sciences, Sex Factors, Rheumatology, Internal medicine, medicine, Humans, Sex Distribution, Systemic Sclerosi, Aged, 030203 arthritis & rheumatology, Lupus erythematosus, Scleroderma, Systemic, business.industry, medicine.disease, Pulmonary hypertension, Prospective Studie, 030104 developmental biology, Heart failure, Age of onset, business, Follow-Up Studies
Abstract

OBJECTIVES: In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes. METHOD: We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. RESULTS: 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p

Published
2014

33. Items for developing revised classification criteria in systemic sclerosis: Results of a consensus exercise with the ACR/EULAR working committee for classification criteria in systemic sclerosis

Author

Fransen, J, Johnson, S R, van den Hoogen, F, Baron, M, Allanore, Y, Carreira, P E, Czirják, L, Denton, C P, Distler, O, Furst, D E, Gabrielli, A, Herrick, A, Inanc, M, Kahaleh, B, Kowal-Bielecka, O, Medsger, T A, Mueller-Ladner, U, Riemekasten, G, Sierakowski, S, Valentini, G, Veale, D, Vonk, M C, Walker, U, Chung, L, Clements, P J, Collier, D H, Csuka, M E, Jimenez, S, Merkel, P A, Seibold, J R, Silver, R, Steen, V, Tyndall, A, Matucci-Cerinic, M, Pope, J E, Khanna, D, Fransen, J, Johnson, Sr, van den Hoogen, F, Baron, M, Allanore, Y, Carreira, Pe, Czirják, L, Denton, Cp, Distler, O, Furst, De, Gabrielli, A, Herrick, A, Inanc, M, Kahaleh, B, Kowal Bielecka, O, Medsger TA, Jr, Mueller Ladner, U, Riemekasten, G, Sierakowski, S, Valentini, Gabriele, Veale, D, Vonk, Mc, Walker, U, Chung, L, Clements, Pj, Collier, Dh, Csuka, Me, Jimenez, S, Merkel, Pa, Seibold, Jr, Silver, R, Steen, V, Tyndall, A, Matucci Cerinic, M, Pope, Je, Khanna, D., University of Zurich, and Khanna, D

Subjects
2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health, Delphi technique, nominal group technique, systemic sclerosis, scleroderma, classification, classification criteria
Abstract

Background Classification criteria for systemic sclerosis (SSc) are being updated. Objective To select a set of items potentially useful for the classification of SSc using consensus procedures including the Delphi and nominal group techniques (NGT). Methods Items were identified through two independent consensus exercises performed by the Scleroderma Clinical Trials Consortium (SCTC) and the EULAR Scleroderma Trials and Research Group (EUSTAR). The first-round items from both exercises were collated and redundancies were removed leaving 168 items. A 3-round Delphi exercise was performed using a 1–9 scale (1=completely inappropriate and 9=completely appropriate) and a consensus meeting using NGT. During the last Delphi, the items were ranked on a 1–10 scale. Results Round 1: 106 experts rated the 168 items. Those with a median score

Published
2012

34. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

Author

Tyndall, A. J., Bannert, B., Vonk, M., Airo, P., Cozzi, F., Carreira, P. E., Bancel, D. F., Allanore, Y., Muller Ladner, U., Distler, O., Iannone, F., Pellerito, R., Pileckyte, M., Miniati, I., Ananieva, L., Gurman, A. B., Damjanov, N., Mueller, A., Valentini, G., Riemekasten, G., Tikly, M., Hummers, L., Henriques, M. J. S., Caramaschi, P., Scheja, A., Rozman, B., Ton, E., Kumanovics, G., Coleiro, B., Feierl, E., Szucs, G., Von Muhlen, C. A., Riccieri, Valeria, Novak, S., Chizzolini, C., Kotulska, A., Denton, C., Coelho, P. C., Kotter, I., Simsek, I., La Pena, D. E., Lefebvre, P. G. D. L. P., Hachulla, E., Seibold, J. R., Rednic, S., Stork, J., Morovic Vergles, J., Walker, U. A., Tyndall, Aj, Bannert, B, Vonk, M, Airò, P, Cozzi, F, Carreira, Pe, Bancel, Df, Allanore, Y, MÜLLER LADNER, U, Distler, O, Iannone, F, Pellerito, R, Pileckyte, M, Miniati, I, Ananieva, L, Gurman, Ab, Damjanov, N, Mueller, A, Valentini, Gabriele, Riemekasten, G, Tikly, M, Hummers, L, Henriques, Mj, Caramaschi, P, Scheja, A, Rozman, B, Ton, E, Kumánovics, G, Coleiro, B, Feierl, E, Szucs, G, VON MÜHLEN, Ca, Riccieri, V, Novak, S, Chizzolini, C, Kotulska, A, Denton, C, Coelho, Pc, Kötter, I, Simsek, I, DE LA PENA LEFEBVRE, Pg, Hachulla, E, Seibold, Jr, Rednic, S, Stork, J, MOROVIC VERGLES, J, and Walker, Ua

Subjects
Lung Diseases, systemic sclreosis, risk factors, Male, Lung Diseases/mortality, Comorbidity, 030204 cardiovascular system & hematology, 0302 clinical medicine, Neoplasms, Epidemiology, Pulmonary fibrosis, Immunology and Allergy, skin and connective tissue diseases, Cause of death, ddc:616, integumentary system, Interstitial lung disease, Sepsis/mortality, Middle Aged, Prognosis, 3. Good health, Pneumonia/mortality, Cohort, Female, Neoplasms/mortality, Gastrointestinal Hemorrhage, Scleroderma, Systemic/*mortality, Adult, medicine.medical_specialty, Heart Diseases, Immunology, Auto-immunity, transplantation and immunotherapy [N4i 4], General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, Rheumatology, Sepsis, Internal medicine, medicine, Humans, Gastrointestinal Hemorrhage/mortality, Risk factor, Health care ethics [NCEBP 5], Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, Proportional hazards model, Heart Diseases/mortality, Pneumonia, medicine.disease, Surgery, Evaluation of complex medical interventions [NCEBP 2], Heart failure, business, Epidemiologic Methods
Abstract

Item does not contain fulltext OBJECTIVES: To determine the causes and predictors of mortality in systemic sclerosis (SSc). METHODS: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. RESULTS: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). CONCLUSION: Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc. 01 oktober 2010

Published
2010

35. Gender differences in early systemic sclerosis patients: A report from the EULAR scleroderma trials and research group (EUSTAR) database

Author

Carreira, Patricia E, Carmona, Loreto, Joven, Beatriz E, Loza, Estíbaliz, Andreu, Jose Luis, Riemekasten, Gabriela, Vettori, Serena, Balbir-Gurman, Alexandra, Airò, Paolo, Walker, Ulrich A, Damjanov, Nemanja, Matucci-Cerinic, Marco, Ananieva, Lidia P, Rednic, Simona, Czirják, László, Distler, Oliver, Farge, Dominique, Hesselstrand, Roger, Corrado, Ada, Caramaschi, Paola, Tikly, Mohammed, Allanore, Yannick, Eustar, Coauthors, Sulli, Alberto, Carreira, Pe, Carmona, L, Joven, Be, Loza, E, Andreu, Jl, Riemekasten, G, Vettori, S, Balbir-Gurman, A, Airò, P, Walker, Ua, Damjanov, N, Matucci-Cerinic, M, Ananieva, Lp, Rednic, S, Czirják, L, Distler, O, Farge, D, Hesselstrand, R, Corrado, A, Caramaschi, P, Tikly, M, Allanore, Y, University of Zurich, Carreira, Patricia E, and Chizzolini, Carlo

Subjects
Lung Diseases, Male, 0301 basic medicine, Databases, Factual, 2745 Rheumatology, Acute-Phase Proteins/analysis, 610 Medicine & health, Severity of Illness Index, Scleroderma, Databases, 03 medical and health sciences, Sex Factors, 0302 clinical medicine, Scleroderma, Limited, Risk Factors, Humans, Limited, skin and connective tissue diseases, Lung Diseases/diagnosis/etiology, Factual, Diffuse/blood/complications/diagnosis/immunology, Autoantibodies, ddc:616, 030203 arthritis & rheumatology, 2403 Immunology, integumentary system, Raynaud Disease/diagnosis/etiology, 10051 Rheumatology Clinic and Institute of Physical Medicine, Nuclear Proteins, Raynaud Disease, Health Status Disparities, Acute-Phase Proteins, Biomarkers, Cross-Sectional Studies, Disease Progression, Female, Prognosis, Scleroderma, Diffuse, Diffuse, 030104 developmental biology, DNA Topoisomerases, Type I, 2723 Immunology and Allergy, Nuclear Proteins/immunology, Autoantibodies/blood, Limited/blood/complications/diagnosis/immunology, Biomarkers/blood
Abstract

OBJECTIVES: To describe differences in clinical presentation between men and women in a large group of patients with early (

36. Subcutaneous vs intravenous abatacept in rheumatoid arthritis-interstitial lung disease. National multicentre study of 397 patients.

Author

López-Maraver M, Serrano-Combarro A, Atienza-Mateo B, Del Val N, Casafont-Solé I, Melero-Gonzalez RB, Pérez-Linaza A, Calvo Gutiérrez J, Mena-Vázquez N, Vegas-Revenga N, Domínguez-Casas L, Loarce Martos J, Peralta Ginés CA, Diez Morrondo C, Pérez Albaladejo L, López Sánchez R, Manzano Canabal MG, Brandy-García AM, López Viejo P, Bonilla G, Maiz-Alonso O, Carrasco-Cubero C, Garijo Bufort M, Moreno M, Urruticoechea-Arana A, Ordóñez-Palau S, González-Montagut C, Giner Serret E, De Dios Jiménez De Aberasturi JR, Lozano Morillo F, Vázquez Rodríguez T, Carreira PE, Blanco Madrigal JM, Miguel Ibáñez B, Rodríguez López M, Fernández-Díaz C, Loricera J, Ferraz-Amaro I, Ferrer-Pargada D, Castañeda S, and Blanco R

Subjects
Humans, Female, Male, Middle Aged, Retrospective Studies, Aged, Injections, Subcutaneous, Treatment Outcome, Administration, Intravenous, Adult, Arthritis, Rheumatoid drug therapy, Arthritis, Rheumatoid physiopathology, Abatacept administration & dosage, Abatacept therapeutic use, Lung Diseases, Interstitial drug therapy, Lung Diseases, Interstitial physiopathology, Antirheumatic Agents administration & dosage, Antirheumatic Agents therapeutic use
Abstract

Background: Evidence on abatacept (ABA) utility for rheumatoid arthritis (RA) - associated interstitial lung disease (ILD) is growing. Clinical trials have shown equivalence in subcutaneous (SC) and intravenous (IV) administration of ABA for articular manifestations. However, this has not been studied in respiratory outcomes., Objective: To compare the effectiveness of ABA in RA-ILD patients according to the route of administration., Methods: National retrospective multicentre study of RA-ILD patients on treatment with ABA. They were divided into 2 groups: a) IV, and b) SC. The following outcomes were analysed from baseline to final follow-up using linear mixed models: a) forced vital capacity (FVC), b) diffusing capacity of the lungs for carbon monoxide (DLCO), c) chest high resolution computed tomography (HRCT), d) dyspnoea, e) RA activity, and f) sparing corticosteroids effect., Results: A total of 397 patients were included (94 IV-ABA and 303 SC-ABA), median follow-up of 24 [10-48] months. After adjustment for possible confounders, FVC and DLCO remained stable during the first 24 months without differences between IV-ABA and SC-ABA (p = 0.6304 and 0.5337). Improvement/ stability of lung lesions in HRCT was observed in 67 % of patients (75 % IV-ABA, 64 % SC-ABA; p = 0.07). Dyspnoea stabilized/ improved in 84 % of patients (90 % IV-ABA, 82 % SC-ABA; p = 0.09). RA - disease activity improved in both groups. No statistically significant differences regarding any of the variables studied between the two groups were found. ABA was withdrawn in 87 patients (21.9 %), 45 % IV-ABA and 37 % SC-ABA (p = 0.29). ILD worsening and articular inefficacy were the most common reasons for ABA discontinuation., Conclusion: In patients with RA-ILD, ABA seems to be equally effective regardless of the route of administration., Competing Interests: Declaration of competing interest Disclosures that may be interpreted as constituting of possible conflict(s) of interest for the study: B. A.-M. received grants/research supports from Abbvie and Roche and had consultation fees/participation in company-sponsored speaker´s bureau from Pfizer, Celgene, Novartis, Sanofi, Janssen, UCB and Lilly, outside the submitted work; N.V.-R. has received fees in company-sponsored speaker's bureau and research supports from AbbVie, Lilly, Celgene, Grünenthal, UCB Pharma, Nordic Pharma, MSD and Rubió; L.C.D.-C has received research supports and participation in company-sponsored speaker's bureau from Abbvie, Janssen, Lilly and Celgene; J.L.M has received fees in company-sponsored speaker's bureau from Boehringer Ingelheim, Bristol-Myers Squibb (BMS) and Galapagos and had consultation fees from Boehringer Ingelheim; G.B has received fees in company-sponsored speaker's bureau from Bristol-Myers Squibb (BMS); A.U.-A. has received grants/research supports from Abbvie, Galápagos, Novartis, GSK, Astrazeneca, Janssen, Pfizer and Amgen; C.F.-D. has received fees in company-sponsored speaker's bureau from: Janssen, Galápagos, Amgen, Bristol Myers Squibb (BMS); I. F.-A. has received grants/research supports from Abbott, MSD, Janssen, and Roche, as well as consultation fees from company sponsored speakers bureaus associated with Abbott, Pfizer, Roche, Sanofi, Celgene, and MSD; S.C. has received research supports from MSD and Pfizer and had consultation fees/participation in company-sponsored speaker's bureau from Amgen, BMS, Eli-Lilly, MSD, Roche, and UCB. S. C. is also assistant professor of the cátedra EPID-Future, funded by UAM-Roche, Universidad Autónoma de Madrid (UAM), Spain; R. B. received grants/research support from AbbVie, MSD, and Roche, and had consultation fees/participation in a company-sponsored speaker's bureau from AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD. The rest of the authors have declared no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Published
2024
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37. Similarities and differences between systemic juvenile idiopathic arthritis and adult-onset Still's disease: a multicenter Spanish study.

Author

Antón J, Mosquera JM, Calzada J, Iglesias E, Zacarías A, Olivé A, Bittermann V, Lorenzo TR, Remesal A, Quintana-Ortega C, Nuño-Nuño L, Robles-Marhuenda A, de Inocencio J, Martín-López M, Carreira PE, Brandy-García AM, Holgado S, Camacho-Lovillo M, Ruiz-Román A, Clemente D, Narváez J, Campos J, Sánchez-Manubens J, Bernabéu P, Graña J, Vargas C, Ortiz-Santamaria V, Castañeda S, de Yébenes MJG, and Carmona L

Abstract

To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year. Descriptive variables (classification criteria, clinical manifestations, complications, family, and personal history) were collected at disease onset and during follow-up. We present the clinical characteristics of 326 patients, 67% of whom had a diagnosis of sJIA and 33% of AOSD. Clinical manifestation frequencies were similar between the two groups, except for odynophagia, which was significantly more frequent in AOSD than in sJIA (78.4% vs. 25.5%; p < 0.0001). Among the complications, macrophage activation syndrome (MAS) was significantly more common in sJIA than in AOSD (24.4% vs. 9.5%; p = 0.002), to the extent that an sJIA diagnosis significantly increased the risk of MAS, together with serositis presence, and the need for biological therapy. Patients with sJIA and AOSD showed similar characteristics, supporting the idea that they are both part of Still's disease, but are expressed at different ages. Differences in manifestations and complications might be due to different management between diseases and immune response maturity., (© 2024. The Author(s).)

Published
2024
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38. Does the Impact of COVID-19 on Patients With Systemic Sclerosis Change Over Time?

Author

Deibel E, Carreira PE, Vonk M, Del Papa N, Bečvář R, Guillén-Del-Castillo A, Campochiaro C, Poormoghim H, Liem S, Lazzaroni MG, Giollo A, Mekinian A, de Vries-Bouwstra J, De Santis M, Balbir-Gurman A, Mihai C, De Luca G, Moiseev S, Zanatta E, Foti R, Rednic S, Denton C, Cutolo M, Belloli L, Airo P, Garzanova L, Moroncini G, İnanç M, Panopoulos S, Tandaipan JL, Chatelus E, Rosato E, Kuwana M, Yavuz S, Alegre-Sancho JJ, Smith V, Szűcs G, Henes J, Rodríguez-Pintó I, Atzeni F, Spierings J, Truchetet ME, Milchert M, Brito de Araujo D, Riemekasten G, Bernardino V, Martin T, Del Galdo F, Vacca A, Mendoza F, Midtvedt Ø, Murdaca G, Santiago T, Codullo V, Cacciapaglia F, Walker U, Brunborg C, Tirelli F, Allanore Y, Furst DE, Matucci M, Gabrielli A, Distler O, and Hoffmann-Vold AM

Subjects
Male, Humans, COVID-19 Testing, COVID-19, Scleroderma, Systemic complications, Scleroderma, Systemic diagnosis, Scleroderma, Systemic epidemiology, Scleroderma, Localized, Hypertension
Abstract

Objective: The outcome of patients with COVID-19 improved over the pandemic, including patients with systemic rheumatic diseases. However, data on patients with systemic sclerosis (SSc) are lacking. This study aimed to assess the outcome of patients with both SSc and COVID-19 over several waves., Methods: Patients with both SSc and COVID-19 who were registered in the European Scleroderma Trials and Research group (EUSTAR) were collected between April 2020 and April 2021. Patients were assigned to waves 1, 2, or 3 depending on the date of their COVID-19 diagnosis. Primary endpoints were death, intensive care unit stay, or ventilatory support (severe outcome). Subgroup analyses of patients who were hospitalized or died were conducted. General and SSc-specific characteristics and treatment were compared over the waves. Descriptive statistics and multivariate logistic regression were applied., Results: A total of 333 patients were included; 57 patients (17%) had a severe outcome, and 30 patients (9%) died. Compared to wave 1, significantly fewer patients with SSc suffered from severe COVID-19 in waves 2 and 3 (28.2% vs 9.8% and 12.7%; P < 0.001), fewer patients required hospitalization (46.7% vs 19.6% and 25.5%; P < 0.001) or ventilatory support (24.0% vs 8.7% and 10.9%; P = 0.001), and fewer patients died (15.7% vs 5.0% and 7.5%; P = 0.011). Patients were significantly younger, more often men, had less frequent arterial hypertension, and less SSc cardiac involvement over waves 1 to 3. Patients received significantly less medium to high doses of corticosteroids as they did SSc treatment., Conclusion: The outcome of patients with both SSc and COVID-19 improved significantly over time because of intrinsic and extrinsic factors., (© 2023 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Published
2024
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39. Immunoglobulins in systemic sclerosis management. A large multicenter experience.

Author

Tandaipan J, Guillén-Del-Castillo A, Simeón-Aznar CP, Carreira PE, De la Puente C, Narváez J, Lluch J, Rubio-Rivas M, Alegre-Sancho JJ, Bonilla G, Moriano C, Casafont-Sole I, García-Vicuña R, Ortiz-Santamaría V, Riera E, Atienza-Mateo B, Blanco R, Galisteo C, Gonzalez-Martin JJ, Pego-Reigosa JM, Pros A, Heredia S, and Castellví I

Subjects
Female, Humans, Male, Immunoglobulins, Intravenous therapeutic use, Retrospective Studies, Skin, Multicenter Studies as Topic, Observational Studies as Topic, Scleroderma, Systemic complications, Scleroderma, Systemic drug therapy, Myositis drug therapy
Abstract

Objective: To analyze the effectiveness and safety of intravenous immunoglobulin (IVIG) given in routine care to patients with systemic sclerosis (SSc)., Methods: A retrospective multicenter observational study was conducted in SSc patients treated with IVIG. We collected data on epidemiological parameters and clinical outcomes. Firstly, we assessed changes in organ manifestations during IVIG treatment. Secondly, we analyzed the frequency of adverse effects. The following parameters were collected from baseline to the last follow-up: the patient's weight, modified Rodnan Skin Score (mRSS), modified manual muscle strength scale (MRC), laboratory test(creatine kinase(CK), hemoglobin and protein levels), The University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA GIT 2.0) questionnaire, pulmonary function tests, and echocardiography., Results: Data were collected on 78 patients (82% females; 59% with diffuse SSc). Inflammatory idiopathic myopathy was the most frequent concomitant overlap disease (41%). The time since Raynaud's phenomenon and SSc onset were 8.8 ± 18 and 6.2 ± 6.7 years respectively. The most frequent IVIG indication was myositis (38/78), followed by gastrointestinal (27/78) and cutaneous (17/78) involvement. The median number of cycles given were 5. 54, 53 and 9 patients have been treated previously with glucocorticoids, synthetic disease-modifying antirheumatic drugs and biologic therapies respectively. After IVIG use we found significant improvements in muscular involvement (MRC ≥ 3/5 92% IVIG, p = 0.001 and CK levels from 1149 ± 2026 UI to 217 ± 224 UI, p = 0.02), mRSS (15 ± 12.4 to 13 ± 12.5, p = 0.015) and improvement in total score of UCLA GIT 2.0 (p = 0.05). None Anti-RNA polymerase III patients showed an adequate response in gastrointestinal involvement (0/7) in comparison with other antibodies (0 vs. 25, p = 0,039). Cardiorespiratory involvement remained stable. A total of 12 adverse events were reported with only one withdrawn due to serious adverse effect., Conclusions: this study suggest that IVIG may improve myositis, gastrointestinal and skin involvement in SSc patients treated in routine care and seems to have a good safety profile., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Published
2023
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40. The Impact of Progressive Pulmonary Fibrosis in Systemic Sclerosis-Associated Interstitial Lung Disease.

Author

Martín-López M and Carreira PE

Abstract

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis, typically affecting the skin, with variable internal organ involvement. Interstitial lung disease (ILD), with a prevalence between 35 and 75%, is the leading cause of death in patients with SSc, indicating that all newly diagnosed patients should be screened for this complication. Some patients with SSc-ILD experience a progressive phenotype, which is characterized by worsening fibrosis on high-resolution computed tomography (HRCT), a decline in lung function, and premature mortality. To assess progression and guide therapeutic decisions, regular monitoring is essential and should include pulmonary function testing (PFT), symptom assessment, and repeat HRCT imaging when indicated. Multidisciplinary discussion allows a comprehensive evaluation of the available information and its consequences for management. There has been a shift in the approach to managing SSc-ILD, which includes the addition of targeted biologic and antifibrotic therapies to standard immunosuppressive therapy (particularly mycophenolate mofetil or cyclophosphamide), with autologous hematopoietic stem-cell transplantation and lung transplantation reserved for refractory cases.

Published
2023
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41. Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma: Data From the International EUSTAR Database.

Author

Lescoat A, Huang S, Carreira PE, Siegert E, de Vries-Bouwstra J, Distler JHW, Smith V, Del Galdo F, Anic B, Damjanov N, Rednic S, Ribi C, Bancel DF, Hoffmann-Vold AM, Gabrielli A, Distler O, Khanna D, and Allanore Y

Subjects
Female, Male, Humans, Fibrosis, Prognosis, Scleroderma, Systemic complications, Scleroderma, Systemic epidemiology, Scleroderma, Diffuse complications, Scleroderma, Diffuse diagnosis, Scleroderma, Diffuse epidemiology, Lung Diseases, Interstitial complications, Telangiectasis etiology, Telangiectasis complications
Abstract

Importance: Systemic sclerosis (SSc) sine scleroderma (ssSSc) is a subset of SSc defined by the absence of skin fibrosis. Little is known about the natural history and skin manifestations among patients with ssSSc., Objective: To characterize the clinical phenotype of patients with ssSSc compared with patients with limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) within the EUSTAR database., Design, Setting, and Participants: This longitudinal observational cohort study based on the international EUSTAR database included all patients fulfilling the classification criteria for SSc assessed by the modified Rodnan Skin score (mRSS) at inclusion and with at least 1 follow-up visit; ssSSc was defined by the absence of skin fibrosis (mRSS = 0 and no sclerodactyly) at all available visits. Data extraction was performed in November 2020, and data analysis was performed from April 2021 to April 2023., Main Outcomes and Measures: Main outcomes were survival and skin manifestations (onset of skin fibrosis, digital ulcers, telangiectasias, puffy fingers)., Results: Among the 4263 patients fulfilling the inclusion criteria, 376 (8.8%) were classified as having ssSSc (mean [SD] age, 55.3 [13.9] years; 345 [91.8%] were female). At last available visit, in comparison with 708 patients with lcSSc and 708 patients with dcSSc with the same disease duration, patients with ssSSc had a lower prevalence of previous or current digital ulcers (28.2% vs 53.1% in lcSSc; P < .001; and 68.3% in dcSSc; P < .001) and puffy fingers (63.8% vs 82.4% in lcSSc; P < .001; and 87.6% in dcSSc; P < .001). By contrast, the prevalence of interstitial lung disease was similar in ssSSc and lcSSc (49.8% and 57.1%; P = .03) but significantly higher in dcSSc (75.0%; P < .001). Skin telangiectasias were associated with diastolic dysfunction in patients with ssSSc (odds ratio, 4.778; 95% CI, 2.060-11.081; P < .001). The only independent factor for the onset of skin fibrosis in ssSSc was the positivity for anti-Scl-70 antibodies (odds ratio, 3.078; 95% CI, 1.227-7.725; P = .02). Survival rate was higher in patients with ssSSc (92.4%) compared with lcSSc (69.4%; P = .06) and dcSSc (55.5%; P < .001) after up to 15 years of follow-up., Conclusions and Relevance: Systemic sclerosis sine scleroderma should not be neglected considering the high prevalence of interstitial lung disease (>40%) and SSc renal crisis (almost 3%). Patients with ssSSc had a higher survival than other subsets. Dermatologists should be aware that cutaneous findings in this subgroup may be associated with internal organ dysfunction. In particular, skin telangiectasias in ssSSc were associated with diastolic heart dysfunction.

Published
2023
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42. Glucocorticoids prescribing practices in systemic sclerosis: an analysis of the EUSTAR database.

Author

Iudici M, Mongin D, Siegert E, Carreira PE, Distler J, Henes J, Zanatta E, Hachulla E, De Luca G, de Souza Müller C, Santiago T, Tandaipan JL, Valdetaro Bianchi B, De Santis M, Hoffmann-Vold AM, Gabrielli A, Distler O, and Courvoisier DS

Subjects
Male, Female, Humans, Middle Aged, Databases, Factual, Data Collection, Glucocorticoids therapeutic use, Scleroderma, Systemic drug therapy, Scleroderma, Systemic complications
Abstract

Objectives: To estimate the prevalence of long-term exposure to glucocorticoids (GCs) and to identify factors associated with, and variations in prescribing practices over time and across recruiting countries., Methods: We included patients with SSc having a visit recorded in the EUSTAR database from January 2013 onward. We analysed the prevalence and the main features of GCs users, their exposure to GCs over time, and their GCs dosages. Multivariable linear regression was used to analyse the factors identified as associated with GCs intake duration. Time trends, and variations in GCs utilization across recruiting countries were explored. Missing data were imputed using multiple imputation with chained equations., Results: The 9819 patients included were mostly females (85%), the majority had lcSSc (73%), and the median age was 58 years. At baseline, 34% of patients (n = 2769/8109) (48% dcSSc vs 29% lcSSc) were on GCs, and the median dose was 7.5 mg/day. GCs users were more frequently males and anti-Scl70 positive, and more commonly had dcSSc and more severe disease. On average, GCs users spent 25% of their follow-up time (median 33.2 months) on GCs, with no significant between-subsets difference. Notably, 33% (n = 971/2959) and 22% (n = 647/2959) of patients followed up for >1 year had received GCs for >6 and >12 months, respectively. Multivariable analysis showed that patient and disease characteristics poorly explained the variability in GCs exposure (adjusted-R2 = 0.06, P < 0.001). GCs utilization varied within and across countries, and gradually decreased over time (36% in 2013 vs 23% in 2018)., Conclusions: GCs are widely and long-term prescribed in SSc, with significant between-countries and within-country differences. A gradual decrease in their utilization has been observed., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Published
2023
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43. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products.

Author

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

Subjects
Humans, Antiviral Agents therapeutic use, COVID-19, Autoimmune Diseases drug therapy, Rheumatic Diseases drug therapy, Rheumatic Diseases epidemiology, Respiratory Distress Syndrome
Abstract

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

Published
2023
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44. Late skin fibrosis in systemic sclerosis: a study from the EUSTAR cohort.

Author

Hughes M, Huang S, Alegre-Sancho JJ, Carreira PE, Engelhart M, Hachulla E, Henes J, Kerzberg E, Pozzi MR, Riemekasten G, Smith V, Szücs G, Vanthuyne M, Zanatta E, Distler O, Gabrielli AG, Hoffmann-Vold AM, Steen VD, and Khanna D

Subjects
Humans, Fibrosis, Skin pathology, Scleroderma, Diffuse complications, Scleroderma, Diffuse pathology, Scleroderma, Systemic complications, Scleroderma, Systemic epidemiology, Scleroderma, Systemic pathology, Skin Diseases pathology
Abstract

Objectives: The early trajectory of skin fibrosis provides insights into the disease course of systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. The aims of our study were to ascertain the prevalence and characteristics of late skin fibrosis in SSc., Methods: We developed and tested three conceptual scenarios of late (>5 years after first non-RP feature) skin fibrosis including new worsening of skin disease, and failure to improve after worsening within 5-year window. We defined skin worsening as change in modified Rodnan skin score (mRSS) ≥5 units or ≥25%. Using strict inclusion criteria including complete mRSS, we identified 1,043 (out of 19 115) patients within the EUSTAR database for our analysis. We further restricted analysis within 887 (out of 1043) patients who had lcSSc or dcSSc at baseline., Results: One-fifth of patients among the whole cohort (n = 208/1043, 19.9%) experienced mRSS worsening, including in patients with lcSSc or dcSSc at baseline (n = 193/887, 21.8%). This was largely due to new skin worsening after the 5-year window or failure to improve with worsening within the 5-year window. Patients with lower baseline mRSS and lcSSc were more likely to develop late skin fibrosis. Anti-Scl-70 was associated with progression from baseline lcSSc to dcSSc, and anticentromere was protective., Conclusions: Late skin fibrosis is not uncommon in SSc. We have identified different patterns relevant to clinical practice and trial design. Late skin fibrosis is a neglected manifestation of SSc and warrants further investigation including to determine clinical outcomes and optimal therapeutic strategy., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Published
2023
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45. Retrotransposon instability dominates the acquired mutation landscape of mouse induced pluripotent stem cells.

Author

Gerdes P, Lim SM, Ewing AD, Larcombe MR, Chan D, Sanchez-Luque FJ, Walker L, Carleton AL, James C, Knaupp AS, Carreira PE, Nefzger CM, Lister R, Richardson SR, Polo JM, and Faulkner GJ

Subjects
Humans, Mice, Animals, Retroelements genetics, DNA Transposable Elements genetics, Mutation, Long Interspersed Nucleotide Elements genetics, Induced Pluripotent Stem Cells
Abstract

Induced pluripotent stem cells (iPSCs) can in principle differentiate into any cell of the body, and have revolutionized biomedical research and regenerative medicine. Unlike their human counterparts, mouse iPSCs (miPSCs) are reported to silence transposable elements and prevent transposable element-mediated mutagenesis. Here we apply short-read or Oxford Nanopore Technologies long-read genome sequencing to 38 bulk miPSC lines reprogrammed from 10 parental cell types, and 18 single-cell miPSC clones. While single nucleotide variants and structural variants restricted to miPSCs are rare, we find 83 de novo transposable element insertions, including examples intronic to Brca1 and Dmd. LINE-1 retrotransposons are profoundly hypomethylated in miPSCs, beyond other transposable elements and the genome overall, and harbor alternative protein-coding gene promoters. We show that treatment with the LINE-1 inhibitor lamivudine does not hinder reprogramming and efficiently blocks endogenous retrotransposition, as detected by long-read genome sequencing. These experiments reveal the complete spectrum and potential significance of mutations acquired by miPSCs., (© 2022. The Author(s).)

Published
2022
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46. Recommendations for prevention of infection in systemic autoimmune rheumatic diseases.

Author

Rúa-Figueroa Fernández de Larrinoa Í, Carreira PE, Brito García N, Díaz Del Campo Fontecha P, Pego Reigosa JM, Gómez Puerta JA, Ortega-Castro R, Tejera Segura B, Aguado García JM, Torre-Cisneros J, Valencia-Martín JL, Pereda CA, Nishishinya-Aquino MB, Otón Sánchez MT, Silva Fernández L, Maese Manzano J, Chamizo Carmona E, and Correyero Plaza M

Subjects
Adult, Humans, Autoimmune Diseases, Rheumatic Diseases complications, Rheumatic Diseases drug therapy
Abstract

Objectives: To develop recommendations for the prevention of infection in adult patients with systemic autoimmune rheumatic diseases (SARD)., Methods: Clinical research questions relevant to the objective of the document were identified by a panel of experts selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network criteria. Specific recommendations were made., Results: Five questions were selected, referring to prevention of infection by Pneumocystis jirovecii with trimethoprim/sulfamethoxazole, primary and secondary prophylactic measures against hepatitis B virus, vaccination against human papillomavirus, vaccination against Streptococcus pneumoniae and vaccination against influenza virus, making a total of 18 recommendations, structured by question, based on the evidence found for the different SARD and/or expert consensus., Conclusions: There is enough evidence on the safety and efficacy of vaccinations and other prophylactic measures against the microorganisms reviewed in this document to specifically recommend them for patients with SARD., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Published
2022
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47. Treatment of Raynaud phenomenon and ischemic ulcers associated to systemic sclerosis with hyperbaric oxygen.

Author

Ahijón-Lana M, Baragaño-Ordóñez E, Veiga-Cabello R, de la Cruz-Tapidor C, and Carreira PE

Subjects
Humans, Ulcer complications, Ulcer drug therapy, Hyperbaric Oxygenation, Raynaud Disease complications, Raynaud Disease therapy, Scleroderma, Systemic complications, Scleroderma, Systemic therapy
Abstract

We describe 4 patients with Raynaud's phenomenon associated with systemic sclerosis, 3 with ischaemic ulcers, successfully treated with hyperbaric oxygen. This therapy has been useful in the treatment of chronic wounds due to its anti-inflammatory, antimicrobial and angiogenic effects. Hyperbaric oxygen treatment could be a therapeutic option in patients with Raynaud's phenomenon refractory to conventional treatment., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Published
2022
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48. Development and validation of a patient-reported outcome measure for systemic sclerosis: the EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire.

Author

Becker MO, Dobrota R, Garaiman A, Debelak R, Fligelstone K, Tyrrell Kennedy A, Roennow A, Allanore Y, Carreira PE, Czirják L, Denton CP, Hesselstrand R, Sandqvist G, Kowal-Bielecka O, Bruni C, Matucci-Cerinic M, Mihai C, Gheorghiu AM, Mueller-Ladner U, Sexton J, Kvien TK, Heiberg T, and Distler O

Subjects
Cohort Studies, Disability Evaluation, Female, Humans, Male, Middle Aged, Patient Reported Outcome Measures, Quality of Life, Reproducibility of Results, Severity of Illness Index, Surveys and Questionnaires, Rheumatology, Scleroderma, Localized, Scleroderma, Systemic complications
Abstract

Objectives: Patient-reported outcome measures (PROMs) are important for clinical practice and research. Given the high unmet need, our aim was to develop a comprehensive PROM for systemic sclerosis (SSc), jointly with patient experts., Methods: This European Alliance of Associations for Rheumatology (EULAR)-endorsed project involved 11 European SSc centres. Relevant health dimensions were chosen and prioritised by patients. The resulting Systemic Sclerosis Impact of Disease (ScleroID) questionnaire was subsequently weighted and validated by Outcome Measures in Rheumatology criteria in an observational cohort study, cross-sectionally and longitudinally. As comparators, SSc-Health Assessment Questionnaire (HAQ), EuroQol Five Dimensional (EQ-5D), Short Form-36 (SF-36) were included., Results: Initially, 17 health dimensions were selected and prioritised. The top 10 health dimensions were selected for the ScleroID questionnaire. Importantly, Raynaud's phenomenon, impaired hand function, pain and fatigue had the highest patient-reported disease impact. The validation cohort study included 472 patients with a baseline visit, from which 109 had a test-retest reliability visit and 113 had a follow-up visit (85% female, 38% diffuse SSc, mean age 58 years, mean disease duration 9 years). The total ScleroID score showed strong Pearson correlation coefficients with comparators (SSc-HAQ, 0.73; Patient's global assessment, Visual Analogue Scale 0.77; HAQ-Disability Index, 0.62; SF-36 physical score, -0.62; each p<0.001). The internal consistency was strong: Cronbach's alpha was 0.87, similar to SSc-HAQ (0.88) and higher than EQ-5D (0.77). The ScleroID had excellent reliability and good sensitivity to change, superior to all comparators (intraclass correlation coefficient 0.84; standardised response mean 0.57)., Conclusions: We have developed and validated the EULAR ScleroID, which is a novel, brief, disease-specific, patient-derived, disease impact PROM, suitable for research and clinical use in SSc., Competing Interests: Competing interests: MB personal fees from Amgen and Bayer, outside the submitted work. RDo reports grants from Articulum Fellowship, sponsored by Pfizer (2013-2014), grants from Actelion, personal fees from Actelion and Boehringer-Ingelheim, outside the submitted work. AG none with respect to the study. RDe none with respect to the study.KF none with respect to the study. ATK none with respect to the study. AR none with respect to the study. YA reports grants and personal fees from Bayer, Boehringer-Ingelheim, Bristol Myers Squibb, Curzion, Inventiva, Roche and Sanofi during the conduct of the study. PEC reports consultancy relationship and/or research grants from: Abbvie, Actelion, Bayer, Boehringer Ingelheim, Corbus Pharma, Emerald Health Pharmaceuticals, Galapagos, Gesynta Pharma, Inventiva, iQvia, Mitsubishi Tanabe Pharma, MSD, Roche and Sanofi Genzyme. LC none with respect to the study. CPD reports grants and personal fees from GSK, Mitsubishi Tanabe Pharma, Boehringer-Ingelheim, Servier, Arxx Therapeutics, Bayer, Inventiva, Galapagos, Horizon, Roche, CSL Behring, Sanofi during the conduct of the study.RH reports personal lecture fees from Actelion Pharmaceuticals Sweden AB, Boehringer-Ingelheim Sweden AB, and Roche Sweden AB, and has been co-investigator in clinical trials for United Therapeutics and Actelion Pharmaceuticals Sweden AB, and has been involved in research advisory boards for Actelion Pharmaceuticals Sweden AB and Boehringer-Ingelheim Sweden AB. GS none with respect to the study. OK-B reports personal fees and/or congress support from Bayer, Boehringer Ingelheim, CSL Behring, Gilead, Inventiva, Medac, MSD, Novartis, Pfizer, Roche, Sandoz, outside the submitted work. CB reports personal fees from Actelion, personal fees from Boehringer-Ingelheim, personal fees from Eli Lilly, grants from Gruppo Italiano Lotta alla Sclerodermia (GILS), grants from New Horizon Fellowship, grants from European Scleroderma Trials and Research (EUSTAR), grants from Foundation for Research in Rheumatology (FOREUM), grants from Fondazione Italiana per la Ricerca sull’Artrite (FIRA), outside the submitted work. MM-C reports grants and personal fees from Actelion, personal fees from Biogen, personal fees from Bayer, personal fees from Boehringer Ingelheim, personal fees from CSL Behring, personal fees from Eli-Lilly, outside the submitted work. CM reports personal fees from Boehringer Ingelheim, Eli-Lilly, Mepha, MEDTalks Switzerland and congress support from Actelion and Roche, outside the submitted work. AMG reports consultancy fees from Boehringer-Ingelheim and congress support from Abbvie and Novartis, outside the submitted work. UM-L none with respect to the study. JS none with respect to the study. TKK none with respect to the study. TH reports no conflicts of interest.OD has/had consultancy relationship and/or has received research funding in the area of potential treatments of scleroderma and its complications from (last three years): Abbvie, Acceleron Pharma, Alexion, Amgen, AnaMar, Arxx Therapeutics, Baecon Discovery, Bayer, Boehringer Ingelheim, Catenion, Drug Development International, CSL Behring, ChemomAb, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, Italfarmaco, iQvia, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Target Bio Science and UCB In addition, he has a patent mir-29 for the treatment of systemic sclerosis issued (US8247389, EP2331143)., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Published
2022
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49. Clinical Characteristics of Juvenile Idiopathic Inflammatory Myopathy and Comparison With Adult Patients: Analysis From a Multicentric Cohort in Spain.

Author

Loarce-Martos J, Larena C, Blázquez MÁ, Joven BE, Carreira PE, Martínez-Barrio J, Monteagudo I, López-Longo FJ, Ruiz L, López-Robledillo JC, Almodóvar R, Llorente I, Tomero E, García-de la Peña P, Moruno H, Pérez A, Cobo-Ibáñez T, Lojo Oliveira L, Barbadillo MC, García-De Yébenes MJ, and Nuño-Nuño L

Subjects
Adult, Cohort Studies, Humans, Longitudinal Studies, Retrospective Studies, Spain epidemiology, Myositis diagnosis, Myositis drug therapy, Myositis epidemiology
Abstract

Methods: This study reviewed the medical records of patients from the REMICAM cohort, a multicentric longitudinal study carried out in patients with IIM, followed up between 1980 and 2014 in 12 hospitals in Madrid, Spain. Patients with definite or probable JPM, JDM, adult DM, and adult PM according to the modified Bohan and Peter criteria were selected. We compared the characteristics between JDM and JPM, and between JIIM and adult IIM., Results: Eighty-six juvenile patients (75 JDMs and 11 JPMs) and 283 adult patients (133 DMs and 150 PMs) were included. Compared with patients with JDM, patients with JPM were older at diagnosis, had more fever and arthritis, and were less frequently treated with disease-modifying antirheumatic drugs (these differences were not statistically significant). Compared with patients with adult DM, those with JDM presented more frequently with calcinosis (33.8% vs 6.9%, p < 0.0001) and had less severe infections (4.3% vs 23.4%, p < 0.0001), malignancies (1.3% vs 25.6%, p < 0.0001), and mortality (3.5% vs 33%, p < 0.0001). Patients with JDM were treated less frequently with azathioprine (10.8% vs 44.7%, p < 0.0001)., Conclusions: Our findings confirm that JIIMs are a heterogeneous group of diseases with relevant differences compared with adult IIMs., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Published
2022
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50. Antifibrotics in systemic sclerosis.

Author

Martin-Lopez M and Carreira PE

Subjects
Fibrosis, Humans, Immunosuppressive Agents therapeutic use, Lung, Lung Diseases, Interstitial drug therapy, Lung Diseases, Interstitial etiology, Scleroderma, Systemic drug therapy
Abstract

Systemic sclerosis (SSc) is a rare and complex disease, involving multiple organs, with high morbidity and mortality. Fibrosis is the hallmark of SSc, although vascular and inflammatory mechanisms are also implicated in its pathogenesis. Disease management is challenging, due to its heterogeneous presentation, and to the limited number of controlled clinical trials to guide treating clinicians. Immunosuppressive agents have been used to prevent progression, especially in the lung, before irreversible injury occurs, with some, although modest, benefit. Nintedanib, a tyrosine kinase inhibitor, has recently demonstrated safety and efficacy in interstitial lung disease (ILD) associated with SSc, and many other antifibrotics are being assessed as possible beneficial therapies, with promising results. An important unmet need remains, to clarify to which patients, when, and with which agent therapy should be initiated, to achieve optimal outcomes. This review summarizes available evidence for current and emerging antifibrotic therapies in SSc patients., Competing Interests: Declaration of competing interest PEC had consultancy relationship and/or received speaking fees from Actelion, Abbie, Boehringer Ingellheim, CLS Behring, Corbus, Emerald Health Pharmaceuticals, Galapagos, Gesynta Pharma, GSK, Mitsubishi Tanabe Pharma, Roche, Sanofi Genzyme and VivaCell. MML had consultancy relationship and/or received speaking fees from Boehringer Ingelheim, Novartis and Merck Sharp & Dohme., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Published
2021
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