Pregnancy unmasking symptoms of undiagnosed Lymphangioleiomyomatosis: Case report and review of literature

Publication date: Available online 23 November 2018Source: Respiratory Medicine Case ReportsAuthor(s): Karam Khaddour, Maryna Shayuk, Dipesh Ludhwani, Sateesh Gowda, Wendy L. WardAbstractCystic lung diseases are a group of disorders that appear similar on radiological studies on chest computed tomography. Each disorder is characterized by its own etiology, pathophysiology, course of progression and manifestation. Lymphangioleiomyomatosis (LAM) is one of the cystic lung diseases that can either be hereditary or sporadic. The sporadic form is a rare disease with no accurate prevalence reported but is believed to be less than 10 per million. LAM is associated with inappropriate activation of mammalian target of rapamycin (mTOR) signaling which regulates cellular growth. The sporadic form is almost confined to premenopausal female population and estrogen is believed to play an important role in the pathogenesis. Pregnancy and use of estrogen based oral contraceptives can aggravate symptoms of already existing LAM. Here we describe a case of LAM that was previously treated as asthma and was diagnosed after exacerbation of respiratory symptoms after pregnancy. We offer a review of the medical literature regarding the etiology, clinical course, diagnosis and treatment of LAM.
Source: Respiratory Medicine Case Reports - Category: Respiratory Medicine Source Type: research