Abstract
Pulmonary sporadic lymphangioleiomyomatosis (LAM) is a female-dominant disease associated with smooth muscle cell proliferation, which results in cystic lung disease presenting commonly with dyspnea and pneumothorax. This article aims to present a patient with the common clinical features and complications of LAM so as to aid in the efficient diagnosis and treatment of future patients. Limited options in the management of LAM make early diagnosis key, as management focuses on supportive care to slow the progressive decline of pulmonary function. Workup includes a diagnosis of exclusion with specific antibodies or titers such as anti-Sjögren's syndrome type A (anti-SSA) antibodies, anti-Sjögren's syndrome type B (anti-SSB) antibodies, angiotensin-converting enzyme (ACE) levels, alpha-1-antitrypsin levels, and vascular endothelial growth factor (VEGF) antibodies with definitive diagnosis limited to tissue confirmation. Here, we discuss a 39-year-old female with dyspnea and spontaneous pneumothorax, who was subsequently diagnosed with LAM during her hospitalization and managed outpatient with sirolimus therapy.
Highlights
Lymphangioleiomyomatosis (LAM) is a disorder affecting multiple systems, such as the kidney and lymphatics, with primary pathology involving the lung [1,2]
Patients affected by LAM are primarily women of reproductive age who present with dyspnea, chest pain, coughing, or hemoptysis [2,3]
Pulmonary function testing may be significant for a decrease in forced expiratory volume in 1 second (FEV1) and diffusion capacity for carbon monoxide (DLCO) [4]
Summary
Lymphangioleiomyomatosis (LAM) is a disorder affecting multiple systems, such as the kidney and lymphatics, with primary pathology involving the lung [1,2]. Patients who have LAM without tuberous sclerosis are considered to have sporadic LAM, which is caused by abnormal proliferation of smooth muscle cells [2]. Patients affected by LAM are primarily women of reproductive age who present with dyspnea, chest pain, coughing, or hemoptysis [2,3]. Patients may receive sirolimus therapy to suppress respiratory decline and/or undergo pleurodesis to prevent complications such as pneumothorax [3,4]. LAM was managed via lung transplantation exclusively; with genetic testing and an increase in the patient study population, alternative management techniques are being researched [2]
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