Abstract

Introduction: UC is an idiopathic inflammatory disease results in diffuse inflammation and ulcers of the colonic mucosa. Hepatobiliary diseases are extraintestinal manifestations of UC. PSC is the most common condition; it shares a similar pathogenesis with IBD. However, some patients may develop AIH/overlapping syndrome, that may result from the chronic inflammation, alteration of the intestinal microbiota and mucosal barrier disruption resulting in toxins and bacterial translocation into the portal circulation. We aim to spot the light on a patient with severe UC complicated with AIH that resolved after colectomy. Case Description/Methods: A 37-year-old Caucasian female was diagnosed with UC at age 29, she had severe UC, colonoscopy showed pancolitis with extensive mucosal ulcerations proximal to the splenic flexure. LFTs have been persistently high during follow up with elevation of AST and ALT 7-8 times the upper limit of normal. Serology showed positive ANA, ASMA, and elevated IgG. Liver biopsy showed portal mononuclear cell infiltrates, nondestructive cholangitis, and mild fibrosis consistent with AIH. Due to persistent symptoms despite medical therapy, and long-standing disease, patient had a proctocolectomy with one stage ileal pouch-anal anastomosis. During a follow up period of 24 months after surgery, LFT has consistently trended down to normal, and the titer of ANA dropped to < 1:80. Discussion: Concurrent UC and AIH isn't commonly seen in practice. These patients have more tendency to develop the disease at younger age, severe disease, pancolitis, resistant to medical therapy, and higher risk of death or liver transplantation. Different pathogenesis behind this phenomenon have been suggested by researchers based on human or mice studies. Chronic inflammatory reaction, autoimmunity activation, and elevation of inflammatory markers could have resulted from the alteration of intestinal microbiota, disruption of the tight junctions proteins that increase the permeability of the colonic mucosa, resulting in toxins and bacterial translocation by the level of lipopolysaccharide to find their way to the liver through the portal circulation. Human studies showed that increased intestinal permeability correlated with the severity of the AIH. In our patient, we hypothesize that severity of the AIH has significantly improved after the patient had a colectomy in which the source of toxins has been eliminated. However, larger retrospective studies using the databases or prospective trials are suggested.

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