A 54-year-old woman with a background history of pulmonary embolism and erythema nodosum was admitted with a 5-day history of pyrexial illness and right hypochondrial pain.
She did not smoke and drank alcohol only in moderation. The only medication she took was warfarin.
On examination, she was pyrexial and was found to be tender in the right hypochondrium. Her initial blood results showed a low platelet count of 99 (normal 140–440) but normal white cell count and haemoglobin. Liver function tests (LFTs) showed a slightly elevated bilirubin of 27 μmol/l (normal 1–20), an increased alkaline phosphatase of 168 μ/l (normal 30–130), a raised γ-glutamyl transpeptidase (GGT) of 225 μ/l (normal 1–42) and also a raised alanine transaminase (ALT) of 260 μ/l (normal 1–41). Her international normalised ratio (INR) was significantly elevated at greater than 10. Her immunoglobulins, autoantibody screen and hepatitis B surface antigen and hepatitis C antibody were negative. Ferritin was significantly elevated at 988 μg/l (normal 12–300) but iron studies were normal.
An ultrasound scan was arranged, which showed a normal liver, gallbladder and biliary tree but there was a trace of free-fluid in the pelvis.
During her hospital stay her pain worsened and radiated to her right shoulder. Her C reactive protein (CRP) and temperature increased so abdominal ultrasound was repeated. This showed mild thickening of the gall bladder wall and a trace of fluid in the gall bladder fossa. There was also thought to be some intrahepatic biliary dilatation and common bile duct dilatation. However, MR cholangiopancreatography (MRCP) examination did not show any duct dilatation.
As her symptoms continued to deteriorate a CT scan of the abdomen was done and this showed ascites and change in the liver architecture. There were multiple areas of low attenuation in the peripheral aspects of both hepatic lobes and appearances were suggestive of an inflammatory or infective process. Due to her continuing abdominal symptoms a laparotomy was performed, which showed a diffuse nodular liver and free-fluid within the abdomen. Liver biopsy was performed at the time of laparotomy and histology revealed widespread necrosis, inflammation and cirrhosis.
Her ascitic fluid serum ascites albumin gradient was more than 11, which indicated ascites due to portal hypertension.
Four months later, she was re-admitted with a 3-day recurrence of epigastric pain. She was found to be pyrexial but her abdomen was soft .Her platelet count was low at 106 (normal 140–440), and her LFTs were abnormal with bilirubin of 25 μmol/l (normal 1–20), an increased alkaline phosphatase of 251 μ/l (normal 30–130), a raised GGT of 209 u/l (normal 1–42) and a raised ALT of 53 μ/l (normal 1–41). Her INR was 3.3 and CRP was >120. A septic screen was performed and she was started on an intravenous antibiotic (cefuroxime) for a presumed diagnosis of cholangitis. Ultrasound of her abdomen showed only a small amount of free-fluid within the abdomen. CT abdomen showed multiple areas of low attenuation within the liver and appearance suggestive of a web in the inferior vena cava (IVC) (). BCS was suspected. This was confirmed on venogram and was treated with venoplasty. Thrombophilia screen established antiphospholipid syndrome as the underlying cause. She made remarkable recovery and she is doing well on her regular outpatient follow-up.
CT scan of the abdomen showing narrowing of the inferior vena cava.