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Severe pulmonary involvement in mixed connective tissue disease
The American Review of Respiratory Disease
|October 1, 1981
Summary
Pulmonary complications in mixed connective tissue disease (MCTD) can be severe. Standard corticosteroid treatment is often ineffective, suggesting a need for more aggressive therapies for lung disease in MCTD patients.
Area of Science:
- Rheumatology
- Pulmonology
- Pathology
Background:
- Mixed connective tissue disease (MCTD) pulmonary involvement is often presumed benign and responsive to corticosteroids.
- Severe, progressive lung disease in MCTD is less understood.
Observation:
- Five patients with MCTD and severe, rapidly progressive lung disease were studied.
- Lung disease manifestations included interstitial lung disease and pulmonary hypertension.
- Histologic, immunofluorescent, and electron microscopic findings were compared with related conditions.
Findings:
- Interstitial lung disease in MCTD showed distinct immunofluorescent and electron microscopic features compared to systemic lupus erythematosus.
- Pulmonary hypertension histology differed between MCTD and scleroderma.
- Corticosteroid therapy was inadequate for both interstitial lung disease and pulmonary hypertension in MCTD.
Implications:
- Current treatment paradigms for MCTD-related lung disease may be insufficient.
- Aggressive or cytotoxic therapies might be beneficial for severe pulmonary manifestations in MCTD.
- Further research into the distinct pathology of MCTD lung disease is warranted.