Verma Ajay Kumar, Joshi Ambarish, Mishra Amritesh Ranjan, Kant Surya, Singh Arpita
Department of Pulmonary and Critical Care Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India.
Department of Respiratory Medicine, King George's Medical University, Lucknow, Uttar Pradesh, India.
Lung India. 2018 Mar-Apr;35(2):154-156. doi: 10.4103/lungindia.lungindia_60_17.
Spontaneous pneumothorax is a very common medical emergency. Patients are often treated without treating the underlying cause. Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease. Until recently, diagnosis of LAM was a challenge with nearly 100% mortality in 10 years, but better understanding of the disease through research and better radiological techniques and newer drugs such as sirolimus has improved the survival in such patients. We are presenting a rare case of LAM presenting as a secondary spontaneous pneumothorax treated with sirolimus.
自发性气胸是一种非常常见的医疗急症。患者通常在未治疗潜在病因的情况下接受治疗。淋巴管平滑肌瘤病（LAM）是一种罕见的囊性肺病。直到最近，LAM的诊断仍是一项挑战，患者10年死亡率接近100%，但通过研究对该疾病有了更好的了解，以及更好的放射技术和西罗莫司等新药的出现，提高了此类患者的生存率。我们报告一例罕见的以继发性自发性气胸为表现的LAM病例，该病例采用西罗莫司治疗。
