Shrinking Lung Syndrome in Systemic Lupus Erythematosus Department of Internal Medicine. La Rabta.University Hospital

In systemic lupus erythematosus (SLE), the respiratory system is frequently compromised. One of its uncommon manifestations is the shrinking lung syndrome (SLS), characterized by unexplained dyspnea, a restrictive pattern on pulmonary function tests, and an elevated hemidiaphragm. The pathogenesis of the SLS remains unknown. We report three consecutive cases of women with systemic lupus erythematosus, aged 32, 33, and 35 years. Clinical features included dyspnea and chest pain. Onset of the SLS manifestations occurred in the absence of a SLE flare, in patients treated with low dose of corticosteroids. Diagnosis was based on clinical and laboratory patterns, particularly chest RMI showing basilar atelectasis, elevated diaphragms, and small lung volumes in two cases. Corticosteroid treatment induced improvement in the three cases and no relapse was noted. Clinical, laboratory, physiopathological, and therapeutic aspects of this syndrome are reviewed, particularly RMI findings, never discussed in previous studies.