Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide.
Shrinking lung syndrome (SLS) is an uncommon feature of systemic lupus erythematosus (SLE) characterized by dyspnea, pleuritic chest pain, diaphragmatic elevation, restrictive ventilatory defect and reduced respiratory muscle strength as measured by volitional tests. We report the case of a 28-year-old woman with overlapping features of SLE and Sjögren syndrome who developed severe SLS while receiving corticosteroids and azathioprine for severe polyarthritis. She was treated with a combination of rituximab and cyclophosphamide, which led to a dramatic improvement in her clinical condition and respiratory function tests. The increase in vital capacity was one of the highest among 35 published cases of SLS. Thus, restoring a near-normal lung function is an achievable goal in SLS, and the use of rituximab, with or without concomitant cyclophosphamide, certainly deserves further study in this setting.
Department of Respiratory Medicine, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Elisabeth
E
Langenskiöld
Alessandro
A
Bonetti
Jean William
JW
Fitting
Raphaël
R
Heinzer
Jean
J
Dudler
François
F
Spertini
Romain
R
Lazor
D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D018501:Antirheumatic Agents / Q000008:administration & dosage; D002637:Chest Pain / Q000209:etiology / Q000503:physiopathology; D003520:Cyclophosphamide / Q000008:administration & dosage*; D016903:Drug Monitoring / Q000379:methods; D004359:Drug Therapy, Combination; D004417:Dyspnea / Q000209:etiology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D010994:Pleura / Q000503:physiopathology; D010998:Pleurisy / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D012129:Respiratory Function Tests / Q000379:methods*; D012132:Respiratory Muscles / Q000503:physiopathology; D000069283:Rituximab; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D016896:Treatment Outcome
D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents; D000069283:Rituximab; D003520:Cyclophosphamide