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BackgroundSystemic sclerosis (SSc) is a heterogeneous autoimmune disease, with a high disease-related mortality and morbidity. A subset of patients show elevated CRP levels (20-35%), which has been reported as inflammatory SSc. Preliminary data suggest that this subset is characterized by a severe phenotype.ObjectivesTo analyse the phenotype and the survival of inflammatory compared with non-inflammatory SSc patient subsets.MethodsData from 8571 SSc patients with available CRP measurement from the EUSTAR cohort were analysed. Exclusion criteria included acute infection, missing follow-up and tocilizumab treatment. Patients with a CRP ≥5mg/l at ≥80% of visits were stratified as persistent inflammatory and as non-inflammatory if CRP was ≥5 mg/l at ResultsOut of 2883 patients with more than two visits, 404 (14%) showed persistent inflammation and 1032 (36%) a non-inflammatory phenotype. Out of 5619 patients with more than one visit, 1830 (33%) were stratified as inflammatory as defined by as single CRP measurement at baseline and 3789 (67%) as non-inflammatory. With both definitions, the inflammatory subset revealed a more severe phenotype than non-inflammatory patients, including more frequent diffuse-cutaneous disease, anti-Scl-70 autoantibodies, pulmonary fibrosis, pulmonary hypertension, higher modified Rodnan skin score, and lower forced vital capacity and diffusing capacity for carbon monoxide. Patients with persistent inflammation had a strongly increased risk of all-cause mortality (HR 7.1 [95%CI 3.7 to 13.5], pConclusionThe severe phenotype and decreased survival of the inflammatory SSc subset, which was most prominent in patients with persistently elevated CRP levels, suggest a distinct disease subset. Therefore both, the need for more regular monitoring of inflammatory parameters and implications for immune-modulating treatment, needs to be carefully analysed.References[1]Mitev, A., et al., Inflammatory stays inflammatory: a subgroup of systemic sclerosis characterized by high morbidity and inflammatory resistance to cyclophosphamide. Arthritis Res Ther, 2019. 21(1): p. 262. PMID: 31791379Figure 1.Overall mortality from baseline onward a. by persistent inflammatory phenotype, b. by inflammatory phenotype at baselineDisclosure of InterestsAdela-Cristina Sarbu: None declared, Sabina Guler: None declared, Odile Stadler: None declared, Yannick Allanore: None declared, Vera Bernardino: None declared, Jörg H.W. Distler: None declared, Armando Gabrielli: None declared, Anna-Maria Hoffmann-Vold: None declared, Marco Matucci-Cerinic: None declared, Ulf Müller-Ladner: None declared, Vera Ortiz-Santamaria: None declared, Simona Rednic: None declared, Valeria Riccieri: None declared, Vanessa Smith: None declared, Susanne Ullman: None declared, Ulrich Walker: None declared, Thomas Geiser: None declared, Oliver Distler: None declared, Britta Maurer Speakers bureau: Boehringer-Ingelheim, Consultant of: Novartis, Boehringer Ingelheim, Janssen-Cilag, Grant/research support from: AbbVie, Protagen, Novartis Biomedical Research, Florian Kollert Shareholder of: Roche, Consultant of: BMS, Actelion, Boehringer-Ingelheim, Pfizer, Grant/research support from: Roche, Gilead, Pfizer, Employee of: Roche