A Rare, Atypical Presentation of Decompensated Cirrhosis: Isolated Transudative Hepatic Chylothorax Without Ascites

Hepatic hydrothorax is an uncommon presentation of decompensated liver cirrhosis and usually presents after other complications of portal hypertension such as ascites. We report the case of a 73-year-old female with autoimmune hepatitis (AIH) treated with budesonide, presenting with a right-sided hepatic chylothorax secondary to AIH and subsequent diagnosis of primary biliary cholangitis (PBC). Pleural fluid analysis revealed a transudative chylothorax, whilst serology, liver elastography, hepatic venous pressure gradients and biopsy diagnosed advanced fibrosis with portal hypertension secondary to AIH-PBC overlap syndrome. Commencement of diuretics led to the resolution of the recurrent pleural effusion. Chylothorax is typically an exudative effusion; however, in very rare cases of decompensated liver cirrhosis, it may present as an isolated transudative effusion in the absence of other signs of portal hypertensive complications such as ascites. This is the first reported case of decompensated cirrhosis secondary to AIH-PBC overlap syndrome, presenting as an isolated unilateral transudative chylothorax.