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Coexistent sarcoidosis and lymphangioleiomyomatosis in a patient with cystic lung disease

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 embol...

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Detalles Bibliográficos
Autores principales: Cullivan, Sarah, De La Harpe Golden, Peter, Doyle, Deirdre, Doddakula, Kishore Kumar, Burke, Louise, Murphy, Desmond Michael
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6261928/
https://www.ncbi.nlm.nih.gov/pubmed/30519468
http://dx.doi.org/10.1002/rcr2.389
Descripción
Sumario: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.