AB0637\u2005IS VERY EARLY SYSTEMIC SCLEROSIS (SSC) A COMBINATION OF MILD AND EARLY DISEASE?

Abstract

Background: SSc has a high morbidity and mortality, therefore it is important to detect the disease at an early stage. Very early detection of SSc allows early management, which has been shown to profoundly impact on the disease course in different inflammatory rheumatic diseases. However, patients fulfilling criteria for the diagnosis of very early SSc could also be patients with a very mild, very slowly progressing, long-standing disease. Objectives: To identify a subgroup of patients with long-standing, very mild SSc among the patients fulfilling the criteria for the very early diagnosis of SSc by analyzing clinical, epidemiological and immunological characteristics of the Zurich patient cohort. Methods: Baseline data of the SSc patients from the University Hospital of Zurich cohort are analyzed in this study. Demographic and disease characteristics of the patients enrolled between January 2009 and June 2018 into the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) cohort were analyzed. Patients fulfilling the aCR/EULAR2013 criteria and patients with primary Raynaud phenomenon were excluded. Disease duration was calculated as the difference between the baseline visit date and the date of the first Raynaud’s phenomenon (RP) symptom reported by the patient. For further analysis, the cohort was divided into two subgroups using a cut off value of </≥5 years disease duration. Data were expressed as absolute and relative frequencies (n/total valid cases (%)) and percentages for categorical variables or as means ± SD and medians ± interquartile range for continuous variables. Results: A total of 107 patients met the inclusion criteria. The patients’ demographic and disease characteristics are shown in Table 1 and Table 2. The majority of patients had a short disease duration measured from first Raynaud attack of < 5 years. However, 43/107 patients (40.2%) had a long-standing disease with long-standing Raynaud’s (Figure 1). Further analysis of the clinical characteristics of early patients with </≥5years disease duration showed no significant differences in demographics and disease manifestations between the subgroups.Abstract aB0637 Table 1 Description of the study cohorts at baseline (total N=107) Age (years) 53 (43,64) Musculoskeletal Female 96 (89.7) Synovitis 13/103 (12.6) Disease duration RP (years)* 4 (1, 10.25) Tendon friction rubs 1/103 (0.9) Disease duration NRP (years)* 4 (1.75, 7) Muscle weakness 4/17 (23.5) Raynaud’s Phenomenon 100/107 (93.5) Cardiopulmonary Puffy fingers (ever) 19/101 (18.8) Dyspnea (NYHA) Stage I/II 88/88 (100) Digital ulcers (ever) 2/80 (2.5) Dyspnea (NYHA) Stage III/IV 0/88 Modified Rodnan skin score 0 Lung fibrosis on HRCT 4/80 (5.0) Laboratory markers Forced vital capacity<80% 8/97 (8.2) ANA 99/103 (96.1) Total lung capacity<80% 2/15 (13.3) ACA 49/103 (47.6) DLCO<70% 8/97 (8.2) Anti-Scl70 1/103 (47.6) PAH by right heart catheterization 0/0 Anti-U1RNP 3/92 (3.2) Gastrointestinal symptoms 36/103 (35.0) Anti-RNA polymerase III 5/96 (5.2) Immunosuppressive treatment 2/107 (1.9) Creatine kinase elevation 12/103 (11.7) Abnormal nailfold capillaroscopy 62/80 (77.5) C reactive protein elevation 7/101 (6.9 Telangiectasia 14/86 (16.3) ACA, aNA, anti-Scl70, U1-RNP- anticentromere, antinuclear, anti-topoisomerase I, anti-U1 ribonucleoprotein antibodies, respectively. DLCO, diffusing capacity of the lung for carbon monoxide; PAH, pulmonary arterial hypertension. ACA, aNA, anti-Scl70, U1-RNP- anticentromere, antinuclear, anti-topoisomerase I, anti-U1 ribonucleoprotein antibodies, respectively. DLCO, diffusing capacity of the lung for carbon monoxide; PAH, pulmonary arterial hypertension. Conclusion: This analysis is showing that patients fulfilling VEDOSS criteria include a subgroup of patients with very mild, long-standing disease. This observation has major impact on the management of VEDOSS patients, as this subgroup requires different follow up and treatment strategies compared to VEDOSS patients with early, progressive disease. Disclosure of interests: Elisabeth Blaja: None declared, Suzana Jordan: None declared, Carina Mihai Consultant for: Received consulting fees or other remuneration from actelion, Geneva, Roche, and Rofarm, Consultant for: F. Hoffmann-La Roche, actelion, Geneva Romfarm, Rucsandra Dobrota: None declared, Mike O. Becker: None declared, Britta Maurer Grant/research support from: Grant/research support from abbVie, Protagen, Novartis; congress support from MSD, Pfizer, Roche, and actelion, Marco Matucci-Cerinic Grant/research support from: actelion, MSD, Pfizer, BMS, Chemomab, Sanipedia, Speakers bureau: actelion, BMS; MSD, Janssen, Oliver Distler Grant/research support from: Prof. Distler received research funding from actelion, Bayer, Boehringer ingelheim and Mitsubishi Tanabe to investigate potential treatments of scleroderma and its complications, Consultant for: Prof. Distler has/had consultancy relationship within the last 3 years with actelion, anaMar, Bayer, Boehringer ingelheim, ChemomAb, espeRare foundation, Genentech/Roche, GSK, inventiva, Italfarmaco, iQvia, Lilly, medac, MedImmune, Mitsubishi Tanabe Pharma, Pharmacyclics, Novartis, Pfizer, Sanofi, Serodapharm and UCB in the area of potential treatments of scleroderma and its complications. In addition, he had/has consultancy relationship within the last 3 years with a. Menarini, amgen, abbvie, GSK, Mepha, MSD, Pfizer and UCB in the field of arthritides and related disorders