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Progressive systemic sclerosis in south India.

V Krishnamurthy1, R Porkodi, S Ramakrishnan

  • 1Madras Medical College.

The Journal of the Association of Physicians of India
|March 1, 1991
PubMed
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Progressive systemic sclerosis (PSS) predominantly affects women in their fourth decade. Skin and joint involvement are common, but internal organ complications, particularly pulmonary hypertension, can be severe.

Area of Science:

  • Rheumatology
  • Immunology
  • Pulmonology

Background:

  • Progressive systemic sclerosis (PSS) is a chronic autoimmune disease.
  • Understanding PSS demographics and clinical features is crucial for patient management.

Purpose of the Study:

  • To analyze clinical, hematological, biochemical, immunological, and radiological findings in Indian PSS patients.
  • To compare these findings with existing Indian and Western studies.

Main Methods:

  • Retrospective analysis of 78 PSS patients over 14 years.
  • Inclusion of clinical, laboratory, and radiological investigations.
  • Comparison with international PSS data.

Main Results:

  • Female preponderance (3.9:1) and peak onset in the fourth decade.

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  • Common symptoms include arthralgia (53.8%) and skin thickening (70.5%); Raynaud's phenomenon is less common (28.2%).
  • High rates of skin (100%), joint (66.7%), and internal organ (52.6%) involvement; 55% showed restrictive airway patterns.

    • Conclusions:

    • Indian PSS patients exhibit distinct epidemiological and clinical characteristics compared to Western cohorts.
    • Pulmonary hypertension is a significant cause of mortality in PSS.
    • Early diagnosis and comprehensive management are vital for improving outcomes.