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Respiratory complications in mixed connective tissue disease
1Division of Pulmonary, Critical Care, and Internal Medicine, Mayo Medical School, Rochester, Minnesota, USA.
Clinics in Chest Medicine
|January 26, 1999
Summary
Mixed connective tissue disease (MCTD) combines features of lupus, scleroderma, and polymyositis. High anti-U1-snRNP antibodies are key, with significant respiratory involvement common in patients.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Mixed connective tissue disease (MCTD) is an autoimmune disorder characterized by overlapping clinical manifestations of systemic lupus erythematosus, scleroderma, and polymyositis.
- Diagnosis requires high titers of antibodies against uridine-rich RNA-small nuclear ribonucleoprotein (U1-snRNP).
Purpose of the Study:
- To describe the spectrum of respiratory manifestations in patients diagnosed with mixed connective tissue disease.
- To highlight the incidence and severity of pulmonary involvement in MCTD.
Main Methods:
- Review of clinical features and diagnostic criteria for MCTD.
- Analysis of reported respiratory manifestations and their prevalence in the literature.
Main Results:
- Respiratory involvement affects 20% to 80% of MCTD patients.
- Common respiratory issues include interstitial pneumonitis/fibrosis (20-65%), pleural effusion (50%), and pulmonary hypertension (10-45%).
- Other pulmonary complications include vasculitis, thromboembolism, and diaphragmatic dysfunction.
Conclusions:
- Respiratory involvement is a frequent and significant complication of MCTD.
- Pulmonary hypertension represents a particularly severe complication, associated with rapid deterioration and mortality.
- Effective management strategies for respiratory complications in MCTD are crucial.