Clinical Case Summary
Introduction: PSC is closely associated with IBD and affects up to 3.6% of Crohn's disease patients, while AIH is also more prevalent in IBD patients, in up to 16% of patients.
Case report: We report a female patient who, at the age of 14, presented with right hypochondrial pain and abnormal liver biochemistry (SGOT: 44 U/l, SGPT: 145 U/l, γ-GT: 300 U/l, ALP: 421 U/l, TBL: 1 mg/dL). The patient underwent MRCP (Figure A) which revealed local strictures with prostenotic dilatation in both intrahepatic and extrahepatic bile ducts, suggestive of PSC. One year later the patient presented with severe abdominal pain and bloody diarrhea. Ileocolonoscopy showed terminal ileitis (Figure B) and segmental colitis. Histological evaluation (Figure C) showed disruption of the architecture, focal erosions of the epithelium, mild mucopenia, inflammatory infiltrate in the dermis, sites of cryptitis and focal formation of crypt abscesses with extension of inflammation to the muscularis mucosae, suggestive of Crohn’s Disease. Two years later, in a regular laboratory test, the patient had abnormal liver function tests (SGOT: 211 U/l, SGPT: 603 U/l, γ-GT: 54 U/l, ALP: 105 U/l, TBL: 0.9 mg/dl). Further evaluation with immunoassay revealed ANA (+) 1/80, AMA (-), SMA (+) 1/160, + F-actin, anti-LKM (-), anti-LC (-), anti-LM (-), p ANCA (+) 1/80, so the patient underwent percutaneous liver biopsy with findings of portal spaces with presence of waxy-containing macrophages, thin fibrous septa, parenchyma with centrolobular necrosis with aggregates of inflammatory cells and waxy macrophages, suggestive of Autoimmune Hepatitis. Today, the patient is in clinical remission under treatment with Ursodeoxycholic acid, Ustekinumab and Mycophenolate Mofetil.
Conclusion: The association of IBD with PSC, AIH and variant forms is now known, however the complexity of the diseases presents a challenge for the clinician and the provision of individualized care.
References
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