Pulmonary Lymphangioleiomyomatosis Presenting As Spontaneous Pneumothorax Treated with Sirolimus - A Case Report

Overview

Journal Lung India

Specialty Pulmonary Medicine

Date 2018 Mar 1

PMID 29487252

Citations 4

Authors

Ajay Kumar Verma

Ambarish Joshi

Amritesh Ranjan Mishra

Surya Kant

Arpita Singh

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

Spontaneous Hydropneumothorax After Pleurodesis in a Patient With Pulmonary Lymphangioleiomyomatosis With Associated Endometrial Cancer.

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The benefit of indwelling pleural catheter with ambulatory pneumothorax device and autologous blood patch pleurodesis in lymphangioleiomyomatosis with persistent air leak.

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Sirolimus in lymphangioleiomyomatosis: A case in point for research in 'orphan' diseases.

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Efficacy of sirolimus for the prevention of recurrent pneumothorax in patients with lymphangioleiomyomatosis: a case series.

Zhou L, Ouyang R, Luo H, Ren S, Chen P, Peng Y Orphanet J Rare Dis. 2018; 13(1):168.

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