Coexistent sarcoidosis and lymphangioleiomyomatosis in a patient with cystic lung disease

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Cullivan, Sarah
De La Harpe Golden, Peter
Doyle, Deirdre
Doddakula, Kishore Kumar
Burke, Louise
Murphy, Desmond M.
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Wiley-Blackwell Publishing Ltd
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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