[Shrinking lung syndrome and systemic auto-immune disease]

Shrinking lung syndrome usually manifest in dyspnea, decreased lung volume associated with elevated diaphragm. It reports with systemic autoimmune disease and physiopathological mechanism is controversial.

We report three shrinking lung syndrome observations in which two cases were diagnosed at the time to onset of autoimmune disease. The three patients were treated with corticosteroid, two of them necessitated theophylline. Review of the literature highlight 60 cases and permit to discuss physiopathological mechanisms which remain uncertain. Diaphragmatic dysfunction (because of myositis or neuropathy) represented by abnormal transdiaphragmatic pressures is actually discussed.

Shrinking lung syndrome is rare but must be considered in patient with autoimmune disease and dyspnea. The diagnosis can be difficult because of clinical, pathological and functional features which are controversial. The optimum treatment is unknown.