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[Shrinking lung syndrome and systemic auto-immune disease]
S Branger1, N Schleinitz, S Gayet
1Service de médecine interne, AP-HM, CHU de la Conception, 147, boulevard Baille, 13385 Marseille 05, France.
La Revue De Medecine Interne
|January 23, 2004
Summary
Shrinking lung syndrome, a rare condition causing shortness of breath and reduced lung volume, is linked to autoimmune diseases. Its exact cause and optimal treatment remain uncertain, requiring careful consideration in affected patients.
Area of Science:
- Pulmonology
- Rheumatology
- Autoimmune Diseases
Background:
- Shrinking lung syndrome presents with dyspnea and decreased lung volumes, often associated with elevated diaphragm.
- The syndrome is frequently linked to systemic autoimmune diseases, though its physiopathological mechanisms are debated.
Observation:
- Three cases of shrinking lung syndrome are presented, with two diagnosed concurrently with autoimmune disease onset.
- Patients were treated with corticosteroids, and two also received theophylline.
- A literature review identified 60 cases, aiding discussion on uncertain physiopathological mechanisms.
Findings:
- Diaphragmatic dysfunction, potentially due to myositis or neuropathy, is a key area of investigation.
- Abnormal transdiaphragmatic pressures are discussed as indicators of diaphragmatic dysfunction.
- The clinical, pathological, and functional features of shrinking lung syndrome are often controversial, complicating diagnosis.
Implications:
- Shrinking lung syndrome is rare but critical to consider in autoimmune patients experiencing dyspnea.
- Diagnostic challenges arise from the controversial features of the syndrome.
- The optimal treatment strategy for shrinking lung syndrome is currently unknown.