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Systemic sclerosis in the elderly.

L Czirják1, Z Nagy, G Szegedi

  • 1Department of Medicine, University Medical School of Debrecen, Hungary.

Clinical Rheumatology
|December 1, 1992
PubMed
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Systemic sclerosis (SSc) in older adults presents aggressively. This study highlights that elderly-onset SSc often involves diffuse skin disease, rapid progression, and severe organ complications, leading to poor outcomes.

Area of Science:

  • Rheumatology
  • Internal Medicine
  • Geriatrics

Background:

  • Systemic sclerosis (SSc) is a complex autoimmune disease characterized by fibrosis, vascular abnormalities, and immune dysregulation.
  • Understanding SSc characteristics in specific patient subgroups, such as the elderly, is crucial for tailored management and improved prognostication.

Purpose of the Study:

  • To investigate the clinical characteristics and outcomes of systemic sclerosis (SSc) in patients aged 60 years and older.
  • To identify specific features and disease trajectories within the elderly-onset SSc subgroup.

Main Methods:

  • Retrospective analysis of 114 patients diagnosed with systemic sclerosis (SSc).
  • Detailed examination of a subgroup of 9 patients with disease onset at age 60 or older.
  • Clinical data collection focusing on disease subtype, skin involvement, organ manifestations, and survival.

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Main Results:

  • Among 9 elderly-onset SSc patients, 7 exhibited diffuse cutaneous systemic sclerosis (dcSSc) with extensive skin involvement.
  • A significant proportion (5/9) of these elderly patients experienced mortality within two years of SSc onset.
  • Seven patients demonstrated a rapid disease course, characterized by cardiac, pulmonary, and/or renal involvement; secondary Sjögren's syndrome, calcinosis, and myositis were notably absent.

Conclusions:

  • Elderly-onset systemic sclerosis (SSc) is frequently characterized by the diffuse cutaneous subtype and aggressive progression.
  • Older patients with SSc face a high risk of rapid disease advancement and severe multi-organ complications, contributing to poor survival rates.
  • The absence of certain features like secondary Sjögren's syndrome, calcinosis, and myositis in this elderly cohort warrants further investigation.