title: Successful treatment of shrinking lung syndrome with rituximab in a patient with systemic lupus erythematosus.
abstract: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE). We report the case of a 27-year-old woman with SLE presenting with a 2-year history of chest pain and progressive dyspnea. Respiratory function tests demonstrated severe restrictive ventilatory impairment. Chest x-ray demonstrated elevated hemi diaphragms and chest computed tomography showed no evidence of interstitial fibrosis, significant pleural disease or pulmonary emboli. Based on a diagnosis of SLS the patient received 4 months of high dose corticosteroids, mycophenolate and pain management with opiates. Her condition deteriorated and she was given a trial of rituximab. This resulted in marked improvement of the clinical condition and respiratory function tests that was maintained for 18 months. Subsequently, the patient represented with a similar clinical picture and another course of rituximab again produced remission. This is the first case report of reproducible remission of SLS in SLE treated with rituximab.
authors.affiliation: Department of Rheumatology, Princess Alexandra Hospital, Queensland, Australia. helenh@gmp.usyd.edu.au
authors.forename: Helen
authors.initials: H
authors.lastname: Benham
authors.forename: Luke
authors.initials: L
authors.lastname: Garske
authors.forename: Phillip
authors.initials: P
authors.lastname: Vecchio
authors.forename: B W
authors.initials: BW
authors.lastname: Eckert
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008171:Lung Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D011859:Radiography; D012074:Remission Induction; D000069283:Rituximab; D013577:Syndrome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab