Abstract Introduction HPS is a pulmonary complication, which causes intrapulmonary vascular dilatation leading to intrapulmonary arteriovenous shunting resulting in hypoxemia. It is frequently described in patients with cirrhosis and non-cirrhotic portal hypertension. In this case, we report a unique case of drug-induced hepatitis leading to HPS in a patient with familial pulmonary fibrosis Case 72-year-old-male with a past medical history significant for familial pulmonary fibrosis was admitted to the in-patient service due to new onset platypnea and progressive hypoxic respiratory failure progressing from use of nasal cannula of 8L to Non-rebreather on exertion in 8 months. Pertinent medications included pirfenidone 801 mg three times daily, which he had been taking for the past three years. On PFTs, he had shown a decline in Forced Vital Capacity (FVC) from 130% to 114%. CT chest showed a stable probable UIP pattern of imaging. A TTE with agitated saline study showed normal cardiac function. It was positive for bubble study, He underwent a RHC, which showed late bubbles in the left atrium confirming intrapulmonary shunting and ruled out intracardiac shunt A technetium macroaggregated albumin (MAA) study showed the presence of a 22% right to left shunt index. CTA of the chest ruled out major arteriovenous malformation. CT angiogram of the abdomen ruled out portosystemic malformations. A transjugular liver biopsy (TJLB) with portal pressure measurement was done, which showed hepatic venous pressure gradient (HVPG) of 3 mmHg. The final pathology of liver biopsy showed patchy portal and centrilobular inflammation without evidence of fibrosis. At this time, drug-induced hepatitis, from pirfenidone, leading to hepatopulmonary syndrome was considered as the leading cause. He was initiated on prednisone 60 mg and discharged on a slow taper. On outpatient visit in 3 months, he only required 2 L on exertion. 6 months from his hospital discharge, he did not require any form of supplemental oxygen at rest or on exertion. He was weaned off prednisone completely Discussion Although classically reported in patients with cirrhosis and non-cirrhotic portal hypertension, rare cases of HPS have been reported in patients with acute hepatitis A and granulomatous hepatitis. This is the first case to report pirfenidone-induced hepatitis as a cause of HPS. Although liver transplantation is the treatment of choice, corticosteroids are an acceptable treatment strategy in this case. This abstract is funded by: -
Tharwani et al. (Fri,) studied this question.