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Pulmonary Vascular Disease: Fellow Case Report Slide: Pulmonary Disorders |

Vanishing Lung

Venkata Ravi Kumar Angirekula, MD; Yasir Tarabichi, MD
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Case Western Reserve University/Metrohealth Medical Center, Cleveland, OH


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(4_S):1143A. doi:10.1016/j.chest.2016.08.1253
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Extract

SESSION TITLE: Fellow Case Report Slide: Pulmonary Disorders

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Tuesday, October 25, 2016 at 04:30 PM - 05:30 PM

INTRODUCTION: Shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus (SLE) with only 77 cases reported in a review published in 2009 (1).

CASE PRESENTATION: A 38 year-old woman with SLE complicated by inflammatory arthropathy and proteinuria managed with mycophenolate mofetil (MMF) and hydroxychloroquine was referred to our care with history of shortness of breath associated with pleuritic chest pain for 6 months. These symptoms started after her MMF was tapered down from 4,000 mg per day to 2,000 mg per day and her prednisone tapered off. Pulmonary function testing was mostly consistent with a severe restrictive process but radiographic evaluation with plain films and chest CT did not show evidence of interstitial or pleural pathology. Supine forced vital capacity decreased by 30% from upright values and MEP was diminished, implicating diaphragmatic dysfunction as an etiology. Prednisone was restarted at 40 mg per day with the patient reporting substantial improvement in her exertional limitations 6 weeks later.

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