Older age onset of systemic sclerosis - Accelerated disease progression in all disease subsets

Publikation: Beitrag in FachzeitschriftForschungsartikelBeigetragenBegutachtung

Beitragende

  • Universität zu Köln
  • Charité – Universitätsmedizin Berlin
  • Justus-Liebig-Universität Gießen
  • Universitätsklinikum Schleswig-Holstein Campus Lübeck
  • Asklepios Klinik Altona
  • Eberhard Karls Universität Tübingen
  • Universität Heidelberg
  • Johanniter-Krankenhaus Treuenbrietzen
  • Universität Ulm
  • Immanuel Krankenhaus Berlin
  • Universitätsklinikum Freiburg
  • Westfälische Wilhelms-Universität Münster
  • Julius-Maximilians-Universität Würzburg
  • Ruhr-Universität Bochum
  • Cellitinnen-Krankenhaus St. Josef Wuppertal-Elberfeld
  • Universitätsklinikum Halle
  • Friedrich-Alexander-Universität Erlangen-Nürnberg
  • Universität Witten/Herdecke

Abstract

Objectives. Systemic sclerosis is a heterogeneous, multisystem disease. It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. There is controversial evidence on whether/how the age at disease onset affects their clinical phenotype. We here investigate the relationship between age at disease onset and symptoms in a large cohort of SSc patients (lcSSc, dcSSc and SSc-overlap syndromes). Methods. Clinical data of the registry of the German Network for Systemic Scleroderma including 3281 patients were evaluated and subdivided into three age groups at disease onset (<40 years, 40-60 years, >60 years). Results. Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age <40 years, and 22.5% were older than 60 years of age. In particular, older patients at onset developed the lcSSc subset significantly more often. Furthermore, they had pulmonary hypertension more often, but digital ulcerations less often. Remarkably, the course of the disease was more rapidly progressing in the older cohort (>60 years), except for gastrointestinal and musculoskeletal involvement. No significant difference was found for the use of corticosteroids. However, significantly, fewer patients older than 60 years received immunosuppressive treatment. Conclusion. In this large registry, -25% of patients developed SSc at an age above 60 years with an increased frequency of lcSSc. In this age group, an onset of internal organ involvement was significantly accelerated across all three subsets. These findings suggest that, in the elderly cohort, more frequent follow-up examinations are required for an earlier detection of organ complications.

Titel in Übersetzung
Beginn der systemischen Sklerose im höheren Alter – beschleunigtes Fortschreiten der Krankheit in allen Krankheitsuntergruppen

Details

OriginalspracheEnglisch
Seiten (von - bis)3380-3389
Seitenumfang10
FachzeitschriftRheumatology (United Kingdom)
Jahrgang59
Ausgabenummer11
PublikationsstatusElektronische Veröffentlichung vor Drucklegung - 24 Apr. 2020
Peer-Review-StatusJa

Externe IDs

PubMed 32333004
ORCID /0000-0002-4330-1861/work/151982039

Schlagworte

Schlagwörter

  • Age at disease onset, German Network for Systemic Scleroderma, Older age, Scleroderma, SSc, Systemic sclerosis

Bibliotheksschlagworte