Shrinking lung syndrome in systemic lupus erythematosus successfully treated with rituximab

Learning point for clinicians Shrinking lung syndrome (SLS) is a systemic lupus erythematosus (SLE) specific complication. Restrictive respiratory disease, normal lung parenchyma and elevated hemidiaphragms on chest X-ray are characteristic. Diaphragmatic dysfunction, pleuritis and anti-Ro antibody are known associations.1–3 Prognosis is generally favourable on corticosteroids, but exceptions do occur. We report a patient with SLS, presenting with acute onset severe respiratory embarrassment, who was difficult to treat with corticosteroids and cyclophosphamide. A 38-year-old female presented with proximal muscle weakness, fever, arthritis and photosensitive skin rashes for 3 weeks and exertional shortness of …