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Shrinking lung syndrome: recognition, pathophysiology and therapeutic strategy
Delphine Carmier1, Elisabeth Diot, Patrice Diot
1Service de Pneumologie, CHRU and Université François Rabelais de Tours, 2 bis Bd. Tonnellé, INSERM U618, Tours Cedex, France.
Expert Review of Respiratory Medicine
|February 26, 2011
Summary
Shrinking lung syndrome, a rare autoimmune complication, causes unexplained shortness of breath with small lung volumes. Early diagnosis and treatment with steroids or immunosuppressors improve outcomes, though respiratory failure is possible.
Area of Science:
- Pulmonology
- Rheumatology
- Internal Medicine
Background:
- Shrinking lung syndrome (SLS) is a rare complication of systemic autoimmune diseases.
- It presents as unexplained dyspnea in patients with conditions like lupus erythematosus, Sjögren's syndrome, and polymyositis.
- SLS is characterized by reduced lung volumes, diaphragmatic elevation, and restrictive physiology without lung tissue damage.
Purpose of the Study:
- To review the clinical presentation, pathogenesis, diagnostic methods, and management of shrinking lung syndrome.
- To highlight the importance of suspecting SLS in autoimmune patients with unexplained respiratory symptoms.
Main Methods:
- Literature review of case reports and studies on shrinking lung syndrome.
- Discussion of diagnostic tools, including phrenic nerve stimulation.
- Overview of current and proposed treatment strategies.
Main Results:
- Pathogenesis is debated, with theories including diaphragm dysfunction, phrenic neuropathy, and pleural inflammation.
- Pleural adhesions and pain are likely significant contributors.
- Phrenic stimulation is a key investigation but not widely accessible.
Conclusions:
- No definitive treatment is validated, but steroids and immunosuppressors are commonly used.
- Biotherapies may offer benefits in refractory cases.
- While prognosis is generally good, respiratory failure remains a potential complication.
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