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Pulmonary manifestations of scleroderma
A C Arroliga1, D N Podell, R A Matthay
1Yale University School of Medicine, New Haven, Connecticut.
Journal of Thoracic Imaging
|March 1, 1992
Summary
Scleroderma frequently affects the lungs, causing interstitial and vascular diseases. Pulmonary complications are the leading cause of death, and treatments show promise but lack definitive proof of altering the disease course.
Area of Science:
- Rheumatology
- Pulmonology
- Pathology
Background:
- Scleroderma is a systemic collagen vascular disease with unknown etiology.
- Pulmonary involvement is common in scleroderma, affecting lungs, vasculature, and airways.
- Respiratory symptoms can be the initial presentation of scleroderma.
Purpose of the Study:
- To review the pulmonary manifestations of scleroderma.
- To discuss diagnostic methods and pathological findings in scleroderma-related lung disease.
- To explore treatment options and prognosis for pulmonary complications.
Main Methods:
- Review of literature on scleroderma and lung involvement.
- Analysis of pulmonary function tests, imaging (radiographs, HRCT), and bronchoalveolar lavage findings.
- Examination of pathological findings from premortem and postmortem studies.
Main Results:
- Common pulmonary abnormalities include restrictive ventilatory defects, airflow obstruction, and reduced diffusing capacity for carbon monoxide.
- Radiographic findings often show interstitial lung disease and honeycombed lung.
- Pulmonary vascular disease, distinct from interstitial lung disease, is also a significant finding, especially in limited scleroderma.
Conclusions:
- Pulmonary complications are the primary cause of mortality in scleroderma.
- High-resolution computed tomography (HRCT) is sensitive for detecting interstitial lung disease.
- While treatments like D-penicillamine, cyclophosphamide, and nifedipine show promise, their efficacy in altering the natural course of pulmonary disease requires further investigation.
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