Unusual association of diseases/symptoms BMJ Case Reports: first published as 10.1136/bcr-2017-222946 on 17 January 2018. Downloaded from CASE REPORT Budd-Chiari syndrome: a rare and life-threatening complication of Crohn’s disease Camila C Simoes,1 Yezaz A Ghouri,2 Shehzad N Merwat,2 Heather L Stevenson1 1Department of Pathology, SUMMARY CASE PRESENTATION University of Texas Medical Budd-Chiari syndrome (BCS) is characterised by A 27-year-old African-American man was admitted Branch, Galveston, Texas, USA 2 obstruction of hepatic venous outflow and may be to our institution as a transfer from an outside Department of Internal triggered by the prothrombotic state associated with hospital to receive a higher level of care due to Medicine, University of Texas Medical Branch, Galveston, inflammatory bowel disease (IBD). We reported a case new-onset ascites and bilateral lower extremity Texas, USA of Crohn’s disease (CD) that presented with anasarca, oedema for the previous 2 weeks. He had a history ascites, symptomatic hepatomegaly, elevated liver of intermittent abdominal pain, haematochezia and Correspondence to enzymes, increased prothrombin time and low albumin. diarrhoea for the past year, when he was diagnosed Dr Heather L Stevenson, Oesophagogastroduodenoscopy and colonoscopy with IBD at an outside hospital. At that time, he hlsteven@ utmb. edu confirmed active CD.A bdominal CT showed hepatic vein was prescribed mesalamine and steroids, which he thrombosis. Liver biopsy revealed severe perivenular CCS and YAG contributed took for a short period with improvement in his equally. sinusoidal dilation with areas of hepatocyte dropout, diarrhoea although he never achieved complete bands of hepatocyte atrophy and centrizonal fibrosis, remission. Due to the cost of the medications, Accepted 28 December 2017 suggestive of BCS. The patient was treated with steroids he discontinued them and was lost to follow-up for CD and systemic anticoagulants for BCS. His liver for approximately 6 months. On admission, he function and enzymes normalised, and he reported complained of new onset of dyspnoea on exertion, symptomatic improvement. The precise mechanism fatigue and abdominal swelling. He had developed responsible for increased hypercoagulability in IBD severe bloody diarrhoea over the last 1 month, remains unclear. Early recognition and treatment for suggestive of an acute flare in the setting of chron- possible thrombotic complications of CD is critical to ically uncontrolled disease. His physical examina- prevent potentially fatal events like pulmonary embolism tion revealed an alert and oriented individual with or liver failure. pale conjunctiva and no signs of icterus. He had prominent tachycardia (heart rate 124/min), bilat- eral pitting pedal oedema and a distended abdomen BACKGROUND with the presence of shifting dullness, suggestive of http://casereports.bmj.com/ Crohn’s disease (CD) is a type of inflammatory ascites. The remainder of his physical examination bowel disease (IBD) that can involve any part of the was normal with no changes noted on the skin and gastrointestinal tract. It causes a systemic inflam- he had intact arterial pulses in all four extremities. matory state that can involve the joints, eyes, liver, skin and haematological system. Generally, the INVESTIGATIONS systemic inflammatory response consists of fevers, Laboratory studies determined that he had iron chills, night sweats, myalgias and chronic fatigue. deficiency anaemia (haemoglobin 5.7 g/dL, mean Elevation of acute phase reactants like the eryth- corpuscular volume 57 fL, undetectable serum iron rocyte sedimentation rate and C reactive protein and ferritin of 9 mg/dL) from chronic gastrointes- can also be observed. Common complications are tinal blood loss, normal bilirubin (total: 0.8 mg/ on 2 October 2021 by guest. Protected copyright. gastrointestinal (GI) stricture and fistula forma- dL and unconjugated: 0.4 mg/dL) and creatinine tion, perforation, haemorrhage, malabsorption (0.6 mg/dL) levels, increased prothrombin time and hypercoagulability. The disease may induce a (16.6 s), hypoalbuminaemia (2.2 g/dL) and elevated prothrombotic state leading to occlusion in both liver enzymes (alkaline phosphatase: 138 U/L; arteries and veins. Thrombosis have been reported alanine transaminase: 83 U/L; aspartate transam- to occur in hepatic veins, portal veins and in the inase: 81 U/L). Stool examination revealed posi- inferior vena cava (IVC).1 Hepatic venous outflow tive faecal leucocytes but was otherwise negative obstruction is the underlying cause of Budd-Chiari for faecal culture for enteric pathogens including syndrome (BCS), classically presenting with a clinical Clostridium difficile toxin, Giardia antigen, Cryp- triad of abdominal pain, ascites and hepatomegaly. tosporidium antigen and other ova and parasites. CD as a precipitant of BCS, although recognised, The elevation of his liver enzymes remained unex- To cite: Simoes CC, is extremely rare and there have been few reported plained, as he had no history of alcohol abuse and Ghouri YA, Merwat SN, et al. cases.2–7 Here, we are presenting a unique case of serological studies for hepatotropic viruses were BMJ Case Rep Published Online First: [please BCS in a patient with CD who concomitantly devel- negative. Laboratory studies performed to evaluate include Day Month Year]. oped hepatic vein and IVC thrombosis leading to for Wilson’s disease, alpha-1 antitrypsin deficiency, doi:10.1136/bcr-2017- the development of cirrhosis and showed substan- autoimmune hepatitis, non-alcoholic steatohepatitis 222946 tial improvement with treatment. and primary biliary cholangitis were also negative. Simoes CC, et al. BMJ Case Rep 2018. doi:10.1136/bcr-2017-222946 1 Unusual association of diseases/symptoms BMJ Case Reports: first published as 10.1136/bcr-2017-222946 on 17 January 2018. Downloaded from with hepatic cirrhosis due to BCS. Except for the liver fibrosis, no other extraintestinal manifestations of CD were present. TREATMENT The patient was transfused with packed red blood cells for his symptomatic anaemia and started on oral prednisone and a low dose of 20 mg intravenous furosemide every 12 hours for 2 days followed by oral spironolactone and furosemide for his CD and anasarca, respectively. To prevent detrimental changes in osmo- lality, no albumin infusion was given to the patient, since he had already received multiple packed red blood cell transfusions which raises serum osmolality. He was also started on heparin intravenous infusion and then transitioned to oral apixaban for long-term anticoagulation therapy. He was not evaluated for an underlying hypercoagu- Figure 1 Endoscopic image of second portion of duodenum showing lable state during his illness due to financial constraints and his multiple superficial ulcers with mucosal erythema. uninsured status. OUTCOME AND FOLLOW-UP Abdominal ultrasound showed hepatomegaly with moderate Eventually the patient’s clinical status improved and he was ascites. Ascitic fluid was sampled which showed <250 neutro- discharged. He was followed up in clinic and was started on phils and serum ascites albumin gradient of >1.1 consistent with azathioprine and sulfasalazine with a plan to slowly taper his portal hypertension. Oesophagogastroduodenoscopy and colo- steroids. This was followed by initiation of adalimumab therapy. noscopy to further evaluate his bleeding and history of IBD were During his sequential follow-up visits, his abdominal pain performed. Oesophagogastroduodenoscopy revealed erythema improved, GI bleeding was absent, bowel movements were in the antrum of stomach and multiple superficial duodenal reduced to about two per day, he gained 10 kg of weight, lower ulcers (figure 1). Biopsies from the upper and lower GI tract showed gastroduodenitis, a granuloma in the stomach (figure 2) extremity swelling resolved and abdominal distension nearly and terminal ileitis. Colonoscopy revealed prominent ulceration normalised. in terminal ileum and caecum with few areas of erythema in the rest of colon. Based on these features, including the endoscopic DISCUSSION appearance and histology findings, a diagnosis of CD was made. The incidence of IBD has increased dramatically during the last He underwent abdominal CT scan, which showed evidence of four decades, with approximately 5 million individuals affected 8 thrombi in the hepatic vein, IVC and portal vein; hepatomegaly in North America and Europe. Although IBD primarily affects with areas of liver injury and relative sparing of caudate lobe and the bowel, there are many known secondary effects on patients large volume ascites (figure 3). A transcutaneous liver biopsy was with IBD that are now widely accepted, including an increased http://casereports.bmj.com/ obtained which showed stage 4, primarily centrizonal fibrosis, risk of venous thromboembolic events, liver disease, cancer with partially obliterated central veins, areas of hepatocyte and bone disease.8–10 Thrombosis in IBD is characterised by dropout and clusters of perivenular haemosiderin-laden macro- both systemic venous thromboembolic events and focal micro- phages (figure 4). The histopathological features were consistent thrombi in the vasculature of the inflamed intestine.11 There are on 2 October 2021 by guest. Protected copyright. Figure 2 (A) Gastric mucosal biopsy showing non-necrotising granulomatous lymphocytic inflammation in a background of chronic active gastritis, consistent with the patient’s diagnosis of Crohn’s disease (CD) (H&E