Shrinking lung syndrome as a presenting manifestation of systemic lupus erythematosus in a female Kuwaiti

The shrinking lung syndrome (SLS) is a rare manifestation in patients with established systemic lupus erythematosus (SLE). Only two cases have been reported in which this syndrome was the presenting manifestation of SLE. We describe a 21-year-old female Kuwaiti who presented with SLS. In addition to clinical and serological features of lupus, she had dyspnea, respiratory muscle dysfunction, characteristic chest radiographic findings of small lung volumes, elevated right hemidiaphragm, and basilar atelectasis. There was no pulmonary parenchymal or pulmonary vascular involvement. Nerve conduction study showed right phrenic nerve palsy. She responded well to treatment with corticosteroids.