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Related Experiment Videos

Relationship between cutaneous and pulmonary involvement in systemic sclerosis

S Morelli1, C Barbieri, A Sgreccia

  • 1Istituto di I Clinica Medica, Università La Sapienza, Rome, Italy.

The Journal of Rheumatology
|January 1, 1997
PubMed
Summary

Systemic sclerosis (SSc) skin and lung involvement severity do not correlate directly. Diffuse SSc patients show more restrictive lung disease, while limited SSc patients may have higher pulmonary hypertension rates.

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Area of Science:

  • Rheumatology
  • Pulmonology
  • Radiology

Background:

  • Pulmonary disease significantly impacts survival in systemic sclerosis (SSc) patients.
  • Pulmonary involvement in SSc can be asymptomatic, contrasting with prominent skin fibrosis.
  • Understanding the interplay between cutaneous and pulmonary manifestations is crucial for SSc management.

Purpose of the Study:

  • To investigate the relationship between skin fibrosis and pulmonary involvement in patients with systemic sclerosis.
  • To compare pulmonary involvement patterns between diffuse (dSSc) and limited (lSSc) forms of SSc.

Main Methods:

  • Study included 52 SSc patients (mean age 50.2 years, disease duration 13.8 years).
  • Patients underwent pulmonary function tests, high-resolution computed tomography (HRCT), and echocardiography.

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  • Skin and pulmonary interstitial fibrosis were quantified using a scoring system.

    • Main Results:

    • Diffuse SSc patients exhibited significantly reduced forced vital capacity and total lung capacity compared to limited SSc patients.
    • No significant difference in overall HRCT scores between dSSc and lSSc, but severe HRCT scores were more common in dSSc.
    • Pulmonary hypertension was observed in 27 patients (15 lSSc, 12 dSSc) with no significant difference between subgroups.
    • No significant correlation found between skin fibrosis scores and pulmonary function parameters or HRCT scores.

    Conclusions:

    • Cutaneous and pulmonary involvement in SSc are not directly correlated in severity or extent.
    • Distinct pulmonary involvement patterns exist between SSc subtypes: restrictive lung disease is more prevalent in dSSc.
    • A trend towards higher pulmonary hypertension prevalence was noted in lSSc patients.