Tuberculosis (TB) is a rare cause of chylothorax. We describe a case and the results of a systematic review of all reported cases of TB-chylothorax. We identified 37 cases of TB-chylothorax. The symptoms at presentation were constitutional (85.7%; 30/35), dyspnea (60.6%; 20/33), and cough (54.5%; 18/33). Chylothorax developed subsequent to the diagnosis of TB in 27.8% (10/36) of the patients, after a median of 6.75 weeks (IQR 4–9). Chylothorax developed during an immune reconstitution syndrome (IRS) in 16.7% (10/36) of the patients, including immunocompetent ones. TB was disseminated in 45.9% (17/37) of the patients at the diagnosis of chylothorax. Chylothorax developed in the absence of any mediastinal lymphadenopathy in 45.9% (17/37) of the patients; 13.5% (5/37) had isolated tubercular empyema alone. The diagnosis of TB was established microbiologically in 72.2% (26/36) and by biopsy alone in 27.8% (9/36) of the patients. Anti-TB treatment (ATT) was administered for a median of 7.57 months (IQR 6–9). Steroids were administered to 22.9% (8/35) of the patients, often for suspected IRS. Thoracic duct ligation and octreotide were required for only 17.1% (6/35) and 8.6% (3/35) of the patients, respectively. In all, 94.4% (34/36) of the patients had resolution of chylothorax and completed treatment successfully; only 5.6% (2/36) died. In conclusion, TB-chylothorax may develop without obvious mediastinal lymphadenopathy and be associated with tubercular empyema alone. TB-chylothorax can develop during treatment of TB due to IRS, even in immunocompetent patients. ATT and dietary manipulation are associated with good resolution and low mortality, and duct ligation is needed for only a small minority of patients.

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