Coexistent sarcoidosis and lymphangioleiomyomatosis in a patient with cystic lung disease. Issue 2 (28th November 2018)

• Record Type: Journal Article
• Authors: Cullivan, Sarah; De La Harpe Golden, Peter; Doyle, Deirdre; Doddakula, Kishore Kumar; Burke, Louise; Murphy, Desmond Michael
• Is Part Of: Respirology case reports. Volume 7:Issue 2(2019)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2018-11-28
• Subjects: Cystic lung disease -- lymphangioleiomyomatosis -- sarcoidosis; Respiratory organs -- Diseases -- Periodicals; 616.2005
• DOI: 10.1002/rcr2.389
• Languages: English
• ISSNs: 2051-3380

Abstract

Abstract : 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. Abstract : This is an interesting case of coexistent lymphangioleiomyomatosis and pulmonary sarcoidosis in a 45‐year‐old lady with cystic lung disease. This case highlights important clinical, radiological, and physiological features of both conditions and suggests a potential shared disease mechanism.