Coexistent sarcoidosis and lymphangioleiomyomatosis in a patient with cystic lung disease
De La Harpe Golden, Peter
Doddakula, Kishore Kumar
Murphy, Desmond M.
Wiley-Blackwell Publishing Ltd
A 45-year-old lady presented acutely with pleuritic chest pain, haemoptysis, and dyspnoea. Her background was significant for a 1.4 cm renal angiomyolipoma, and she was an ex-smoker without any relevant family history. A computed tomography (CT) pulmonary angiogram was negative for a pulmonary embolism but demonstrated diffuse cystic change throughout both lungs. A bronchoscopy confirmed a normal endobronchial tree, and pulmonary function tests demonstrated moderate airways obstruction, with reversibility and a normal diffusion capacity for carbon monoxide (DLCO). A video-assisted thoracoscopic surgery (VATS) lung biopsy showed non-caseating granulomas, and serum angiotensin converting enzyme (ACE) was elevated consistent with a diagnosis of pulmonary sarcoidosis. Further sectioning indicated focal areas that stained positive for Human Melanoma Black 45 (HMB-45), confirming lymphangioleiomyomatosis (LAM). A diagnosis of cystic lung disease secondary to coexistent sarcoidosis and LAM was made.
Cystic lung disease , Lymphangioleiomyomatosis , Sarcoidosis
Cullivan, S., De La Harpe Golden, P., Doyle, D., Doddakula, K.K., Burke, L. and Murphy, D.M., 2019. Coexistent sarcoidosis and lymphangioleiomyomatosis in a patient with cystic lung disease. Respirology case reports, 7(2), (3pp.) e00389. DOI: 10.1002/rcr2.389
© 2018 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.