A Unique Triad of Ulcerative Colitis With Autoimmune Hemolytic Anemia and Primary Sclerosing Cholangitis

Abstract

Inflammatory Bowel Diseases (IBD) lead to chronic intestinal inflammation along with extra-intestinal manifestations which could present prior to, subsequent with or after the diagnosis of IBD. We present a unique case of Ulcerative Colitis (UC), Autoimmune Hemolytic Anemia (AIHA) and Primary Sclerosing Cholangitis (PSC) diagnosed simultaneously in a patient. The unique triad of these diseases gives important clues to the immunological factors involved in the pathogenesis of these diseases. An 18-year-old male presented with 1 week of bloody diarrhea, abdominal cramps and nausea. Vitals were stable. Initial labs showed Hb 9.5 g/dL, MCV 102 fL, WBC 22.4 x 103/μL, total bilirubin 2.4 mg/dL, direct bilirubin 0.7 mg/dL, alkaline phosphatase 436 units/L, ALT 133 units/L and AST 83 units/L. Hepatitis panel and Clostridium difficile toxin assay were negative. Further work-up revealed reticulocyte count > 17%, LDH 529 units/L, haptoglobin < 10 mg/dl and positive direct Coomb's test. Peripheral smear showed hemolysis by a warm antibody. Hydrocortisone was started for AIHA. CT abdomen showed mild colonic wall thickening. Colonoscopy showed moderate pancolitis and biopsies showed findings consistent with UC. Mesalamine was started. MRCP showed irregular beaded appearance of intra and extrahepatic bile ducts. ERCP was performed and biopsies taken from the ducts showed chronic inflammation but no evidence of malignancy. ASMA and ANCA were positive. Liver biopsy ruled out overlap syndrome with autoimmune hepatitis. The patient clinically improved and was discharged to follow-up with a gastroenterologist. In patients with UC, extra-intestinal manifestations involving cutaneous, hepatobiliary and ocular systems are common. The concomitant presence of PSC is 2-7% and AIHA is 0.2 -1.7% in patients with UC. The extra-intestinal manifestations of UC could be due to associated autoimmune diseases or they could be immune-mediated due to damaged mucosal wall. The exact pathogenesis of PSC and AIHA is unknown. The absorption of toxins and antigenic substances from the mucosa in the circulation mediates inflammation in the biliary system leading to PSC. This also leads to warm anti-E antibody production which cross-react with intestinal microbiome and the E antigen at the surface of RBCs. In UC patients, a vigorous workup for early detection of other autoimmune diseases is important to improve the quality of life and to prevent mortality associated with them.Figure: Peripheral smear showing polychromatic RBCs and microspherocytes.Figure: Axial CT demonstrating mild wall thickening and featureless appearance of the rectosigmoid colon compatible with chronic ulcerative colitis (left). Descending colon at 4X magnification showing crypt distortion, cryptitis and crypt abscess (right).Figure: Left: Coronal thin section T2 weighted image showing a beaded appearance of the left hepatic and intrahepatic bile ducts with alternating strictures (arrows) and dilatation (arrowheads). Right: A) Fluoroscopic image from an ERCP depicting beading of the bilateral intrahepatic bile ducts due to segmental strictures (thin arrows). Guidewire in the left intrahepatic bile duct (thick arrow). B) ERCP image after biliary stent placement (arrowhead) with decompression of the biliary tree.