Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Novel Insights from Clinical Practice Respiration 2012;84:144–149 DOI: 10.1159/000334947 Shrinking Lung Syndrome Successfully Treated with Rituximab and Cyclophosphamide Elisabeth Langenskiöld a Alessandro Bonetti c Jean William Fitting a Raphaël Heinzer a Jean Dudler d François Spertini b Romain Lazor a, e Departments of a Respiratory Medicine and b Immunology, Centre Hospitalier Universitaire Vaudois, and c Private Practice, Lausanne, and d Department of Rheumatology, Hôpital Fribourgeois, Fribourg, Switzerland; e Reference Center for Rare Lung Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University, Lyon, France of a 28-year-old woman with overlapping features of SLE and Sjögren syndrome who developed severe SLS while receiv- ing corticosteroids and azathioprine for severe polyarthritis. She was treated with a combination of rituximab and cyclo- phosphamide, 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. Copyright © 2012 S. Karger AG, Basel Key Words Systemic lupus erythematosus  Shrinking lung syndrome  Sjögren syndrome  Rituximab  Cyclophosphamide  Diaphragm  Respiratory paralysis Abstract Shrinking lung syndrome (SLS) is an uncommon feature of systemic lupus erythematosus (SLE) characterized by dys- pnea, pleuritic chest pain, diaphragmatic elevation, restric- tive ventilatory defect and reduced respiratory muscle strength as measured by volitional tests. We report the case Received: July 5, 2011 Accepted after revision: November 9, 2011 Published online: January 31, 2012 Dr. Romain Lazor Service de Pneumologie, Centre Hospitalier Universitaire Vaudois Rue du Bugnon, 46 CH–1011 Lausanne (Switzerland) Tel. +41 21 314 4746, E-Mail romain.lazor  @  chuv.ch © 2012 S. Karger AG, Basel 0025–7931/12/0842–0144$38.00/0 Accessible online at: www.karger.com/res Established Facts • The treatment of shrinking lung syndrome is not standardized and the efficacy of various treatment modalities is not well established. Novel Insights • Rituximab combined with an immunosuppressive agent may be highly beneficial in shrinking lung syndrome and deserves further study. • Restoration of near-normal lung function is an achievable goal in shrinking lung syndrome.