Ab0215 a Late Onset of Systemic Sclerosis Is Associated With a More Rapidly Progressing Clinical Phenotype in LCSSC Patients

Abstract

BackgroundSSc is a heterogeneous multisystem connective tissue disease. The majority of patients (pts) develop initial clinical symptoms between the ages of 30-50 years (yrs).It is not yet known whether the age of onset has an influence on the development of a distinct clinical phenotype.ObjectivesInvestigating the relationship between age at disease onset and the development of clinical characteristics using data of the German Network for Systemic Scleroderma.MethodsWe evaluated 2928 patient data, subdivided them into 3 age groups at disease onset (<40 years, 40-60 years, and >60 years) and correlated the age at disease onset with specific clinical characteristics.ResultsOverall, 24% of pts developed first non-RP symptoms at the age of <40yrs, 53% between the age of 40-60yrs, and 23% were older than 60yrs. In particular, SSc pts with disease onset >60yrs developed significantly (p<0.001) more often the lcSSc subtype (64%) as well as ACA antibodies (42%) with a significantly lower mRSS. They, however, also suffered more often from pulmonary hypertension (PH) and developed less often digital ulcers. Especially lcSSc pts were associated with a more rapidly progressing clinical phenotype, i.e., 25% of the elderly lcSSc pts developed PH after five years. In contrast, less than 5% diagnosed with lcSSc at the age of <40yrs suffered from PH after five yrs (p<0.001).ConclusionIn this registry, approximately one quarter of pts developed SSc at an age above 60yrs, predominantly having a lcSSc subtype. Although these pts have been diagnosed with the mild form of SSc, pts with a lcSSc subtype at a higher age (>60yrs) had more frequent a PH and showed a more rapid disease progression than the youngest pts. These findings may have an important influence on recommendations on diagnosis, frequency of follow-ups and therapy of SSc in different age groups.Disclosure of Interests Pia Moinzadeh Speakers bureau: Actelion, Kathrin Kuhr: None declared, Ulf Müller-Ladner Grant/research support from: Projekt supported by an unrestricted educational grant from Celgene GmbH., Elise Siegert Shareholder of: Astra Zeneca, Grant/research support from: Actelion, Consultant for: AEC Partners, Speakers bureau: Actelion, Norsk, Jörg Henes: None declared, Gabriela Riemekasten Consultant for: Chugai, F. Hoffmann-La Roche, Speakers bureau: Chugai, F. Hoffmann-La Roche, Norbert Blank Grant/research support from: SOBI and Novartis, Speakers bureau: Novartis and SOBI, Marc Schmalzing Grant/research support from: Pfizer, Chugai, MSD, Janssen-Cilag, BMS, Celgene, UCB, Consultant for: Abbvie, Chugai, Genzyme, Hexal/Sandoz, MSD, Novartis, Roche, Sanofi Pasteur, Speakers bureau: Actelion, Baxalta/Shire, BMS, Celgene, Chugai, Janssen-Cilag, MSD, Novartis, Pfizer, Roche, UCB, Ina Koetter: None declared, Claudia Guenther Grant/research support from: Pfizer, Novartis, Employee of: 20 years ago, Novartis, Speakers bureau: Celtis, Tim Schmeiser Speakers bureau: Actelion, UCB, Pfizer, Gabriele Zeidler Grant/research support from:MSD Sharp & Dohme GmbHActelion Pharma...