Sporadic lymphangioleiomyomatosis with lymphocyte-predominant pleural effusion misdiagnosed as tuberculosis: a case report.

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women of reproductive age. The disease may involve extrapulmonary sites, including the lymphatic system, and can present with a lymphocyte-predominant pleural effusion mimicking tuberculosis. We report a 35-year-old woman who presented with a three-year history of progressive dyspnea and recurrent lymphocyte-predominant exudative pleural effusions. She was empirically started on anti-TB therapy at a local hospital but showed no clinical improvement. Upon evaluation at a tertiary center, there was decreased air entry over the posterior third of the chest. Repeat pleural fluid analysis demonstrated a milky appearance with elevated triglyceride levels, consistent with chylous effusion. High-resolution CT showed diffuse, bilateral, thin-walled pulmonary cysts and bilateral pleural effusion with retroperitoneal lymphatic involvement, which confirmed the diagnosis of LAM. Treatment with sirolimus was started and showed significant clinical improvement, but the patient discontinued therapy after two months due to financial constraints. In this patient, LAM presented as tuberculous pleuritis that delayed the definitive diagnosis. This should alert clinicians in high TB burden settings to consider this rare disease in reproductive-aged women who present with progressive dyspnea and a lymphocyte-predominant pleural effusion, particularly when there is no response to anti-TB therapy.