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Renal involvement in scleroderma.

Y Desai1, M A Ghanekar, R D Siquera

  • 1Department of Medicine, TNM College, Bombay.

The Journal of the Association of Physicians of India
|October 1, 1990
PubMed
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This study analyzed 24 patients with progressive systemic sclerosis, detailing common symptoms like skin changes and Raynaud's phenomenon. Findings highlight varied manifestations and organ involvement in systemic sclerosis progression.

Area of Science:

  • Rheumatology
  • Dermatology
  • Internal Medicine

Background:

  • Progressive systemic sclerosis is a complex autoimmune disease.
  • Understanding its diverse clinical manifestations is crucial for patient management.

Purpose of the Study:

  • To analyze the clinical characteristics and disease progression in a cohort of patients with progressive systemic sclerosis.
  • To identify common symptoms, major organ involvement, and outcomes in this patient group.

Main Methods:

  • Retrospective analysis of 24 patients diagnosed with progressive systemic sclerosis.
  • Detailed review of patient demographics, initial symptoms, disease course, and organ involvement.
  • Inclusion of data from kidney biopsies for clinically normal patients.

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Main Results:

  • The cohort comprised 20 females and 4 males, aged 10-65 years.
  • Common initial symptoms included cutaneous and vasospastic issues; major manifestations were skin changes, Raynaud's phenomenon, and articular/gastrointestinal involvement.
  • Significant findings included cardiac (3), overlap syndrome (3), and renal involvement (2), with kidney biopsies abnormal in 50% of clinically normal patients.
  • Three patients died due to cardiac and pulmonary complications.

Conclusions:

  • Progressive systemic sclerosis presents with a wide spectrum of clinical features.
  • Early identification and monitoring of organ involvement, particularly renal and cardiac, are essential.
  • The study underscores the heterogeneous nature and potential severity of systemic sclerosis.