Hemobilia from Ruptured Hepatic Artery Aneurysm in Polyarteritis Nodosa
A 39-year-old man was admitted with severe right upper quadrant abdominal pain. The history was significant for partial small bowel resection for intestinal infarction about 5 years prior to this presentation. The patient was otherwise stable without any further investigations or management, and had been asymptomatic until the current episode.\nOn admission, his vital signs were as follows: blood pressure 160/100 mmHg, pulse rate 72/min, respiratory rate 18/min, temperature 36.3℃. He had minimal conjunctival injection and icteric sclerae. Abdominal examination revealed normal bowel sounds with no palpable abdominal mass or organomegaly. There was tenderness to palpation over the right upper quadrant and epigastrium. He did not have any rebound tenderness and Murphy's sign was negative. Laboratory studies showed WBC 5700/mm3, Hb 12.3 g/dL, hematocrit 35.0%, and platelets 183,000/mm3. Liver function tests revealed a total bilirubin of 5.2 mg/dL, AST 175 IU/L, ALT 185 IU/L, alkaline phosphatase 899 IU/L, and GTP 615 IU/L. Abdominal CT demonstrated multiple high attenuation areas in the biliary duct and gallbladder (). Endoscopic retrograde cholangiopancreatography (ERCP) showed a mild but active blood leak and adherent clots at the ampulla of Vater (). Cholangiography revealed multiple tubular filling defects in the common bile duct as well as the intrahepatic ducts (). Endoscopic sphincterotomy and nasobiliary drainage were performed. During the procedure, hemobilia spontaneously stopped. He reported an improvement in his abdominal pain after the procedure. However, the following day, the patient developed a massive hematemesis and hematochezia, with a drop in his blood pressure to 70/50 mmHg and Hb to 7.5 g/dL. Emergency selective hepatic angiogram demonstrated multiple microaneurysms in the branches of the hepatic arteries () and a slow leak of the contrast medium into the bile duct from the arteriobiliary fistula (). This fistulous tract was blocked with coil and gelfoam (). Following this procedure, his blood pressure stabilized rapidly. There was no evidence of rebleeding. Superior mesenteric, inferior mesenteric and renal artery angiography revealed multiple stenoses, smooth occlusions and microaneurysmal changes in small- to mid-sized vessels. Antinuclear antibody, antineutrophil cytoplasmic antibodies, rheumatoid factor, antiphospholipid antibody and lupus anticoagulant were all negative. A clinical diagnosis of polyarteritis nodosa was made and he was managed with prednisolone 25 mg per day, which was tapered to 5 mg per day, in combination with cyclophosphamide 50 mg per day. He is entirely asymptomatic at six month follow up.