Probability of sporadic lymphangioleiomyomatosis in women presenting with spontaneous pneumothorax.

ABSTRACT:

Background

Sporadic lymphangioleiomyomatosis (S-LAM) is a rare low-grade neoplasm of young women characterized by multiple pulmonary cysts leading to progressive dyspnea and recurrent spontaneous pneumothorax (SP). The diagnosis of S-LAM may be delayed by several years. To reduce this delay, chest computed tomography (CT) screening has been proposed to uncover cystic lung disease in women presenting with SP. However, the probability to discover S-LAM in this population has not been determined precisely. The aim of this study was to calculate the probability of finding S-LAM in women presenting with (a) SP, and (b) apparent primary SP (PSP) as first manifestation of S-LAM.

Methods

Calculations were made by applying the Bayes theorem to published epidemiological data on S-LAM,

SUBMITTER: Suter A 

PROVIDER: S-EPMC10327335 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature