Bronchobiliary fistula is a very rare entity that presents with bilioptysis. We present a noteworthy case involving a patient with portal cavernoma cholangiopathy complicated by cholangitis and bronchobiliary fistula. The diagnosis was established through high-resolution computed tomography of the thorax and bronchoscopic evaluation. Subsequently, the patient underwent endoscopic retrograde cholangiopancreatography with stenting of the common bile duct. Remarkably, the bronchobiliary fistula resolved 1 month after the procedure.

A 21-year-old man presented with acute onset of jaundice, abdominal pain, ascites, and hepatomegaly, along with a history of Budd-Chiari syndrome previously treated with vena cava angioplasty. Investigations revealed rapidly worsening jaundice, coagulopathy, elevated creatinine levels, reactive hepatitis B serology, and positive antiphospholipid antibodies, with scores indicating a poor prognosis for liver transplant-free survival.Abdominal computed tomography demonstrated a narrowed intrahepatic vena cava and new thrombosis in the right and middle hepatic veins. Renal biopsy, prompted by nephritic range proteinuria, indicated mesangioproliferative glomerulonephritis (MPGN) with immune complex deposition. The described case involves acute-on-chronic liver failure with acute insults from new onset hepatic vein thrombosis and hepatitis B reactivation, in a patient at a non-transplant center, who also had underlying antiphospholipid antibody syndrome, and MPGN. The patient was successfully treated with antiviral, anticoagulation, and antiplatelet agents, along with a sodium-glucose cotransporter 2 inhibitor and a direct intrahepatic portosystemic shunt, despite having a Model for End-Stage Liver Disease score of 35.

Bronchobiliary fistula is a very rare entity that presents with bilioptysis. We present a noteworthy case involving a patient with portal cavernoma cholangiopathy complicated by cholangitis and bronchobiliary fistula. The diagnosis was established through high-resolution computed tomography of the thorax and bronchoscopic evaluation. Subsequently, the patient underwent endoscopic retrograde cholangiopancreatography with stenting of the common bile duct. Remarkably, the bronchobiliary fistula resolved 1 month after the procedure.

A 21-year-old man presented with acute onset of jaundice, abdominal pain, ascites, and hepatomegaly, along with a history of Budd-Chiari syndrome previously treated with vena cava angioplasty. Investigations revealed rapidly worsening jaundice, coagulopathy, elevated creatinine levels, reactive hepatitis B serology, and positive antiphospholipid antibodies, with scores indicating a poor prognosis for liver transplant-free survival.Abdominal computed tomography demonstrated a narrowed intrahepatic vena cava and new thrombosis in the right and middle hepatic veins. Renal biopsy, prompted by nephritic range proteinuria, indicated mesangioproliferative glomerulonephritis (MPGN) with immune complex deposition. The described case involves acute-on-chronic liver failure with acute insults from new onset hepatic vein thrombosis and hepatitis B reactivation, in a patient at a non-transplant center, who also had underlying antiphospholipid antibody syndrome, and MPGN. The patient was successfully treated with antiviral, anticoagulation, and antiplatelet agents, along with a sodium-glucose cotransporter 2 inhibitor and a direct intrahepatic portosystemic shunt, despite having a Model for End-Stage Liver Disease score of 35.

Bronchobiliary fistula is a very rare entity that presents with bilioptysis. We present a noteworthy case involving a patient with portal cavernoma cholangiopathy complicated by cholangitis and bronchobiliary fistula. The diagnosis was established through high-resolution computed tomography of the thorax and bronchoscopic evaluation. Subsequently, the patient underwent endoscopic retrograde cholangiopancreatography with stenting of the common bile duct. Remarkably, the bronchobiliary fistula resolved 1 month after the procedure.

A 21-year-old man presented with acute onset of jaundice, abdominal pain, ascites, and hepatomegaly, along with a history of Budd-Chiari syndrome previously treated with vena cava angioplasty. Investigations revealed rapidly worsening jaundice, coagulopathy, elevated creatinine levels, reactive hepatitis B serology, and positive antiphospholipid antibodies, with scores indicating a poor prognosis for liver transplant-free survival.Abdominal computed tomography demonstrated a narrowed intrahepatic vena cava and new thrombosis in the right and middle hepatic veins. Renal biopsy, prompted by nephritic range proteinuria, indicated mesangioproliferative glomerulonephritis (MPGN) with immune complex deposition. The described case involves acute-on-chronic liver failure with acute insults from new onset hepatic vein thrombosis and hepatitis B reactivation, in a patient at a non-transplant center, who also had underlying antiphospholipid antibody syndrome, and MPGN. The patient was successfully treated with antiviral, anticoagulation, and antiplatelet agents, along with a sodium-glucose cotransporter 2 inhibitor and a direct intrahepatic portosystemic shunt, despite having a Model for End-Stage Liver Disease score of 35.