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• W3210944189 abstract "Introduction: Autoimmune hepatitis-Primary biliary cholangitis (AIH-PBC) overlap syndrome is supported by serologic and/or histologic features. The Paris criteria have been developed for the diagnosis of AIH-PBC overlap syndrome. There is no consensus regarding the role of imaging thus serologic and histologic findings are necessary and lack of a definitive diagnosis may delay treatment. We present a case of AIH-PBC overlap syndrome confirmed by liver biopsy, initially with incongruent findings on MRI and elastography. Case Description/Methods: A 53-year-old woman presented for evaluation of elevated liver tests. Medical history included atrial fibrillation and current medications were Eliquis and metoprolol. The patient did not report alcohol use or other hepatotoxins. Physical exam was unremarkable for jaundice, icterus, or abdominal pain. Laboratory studies revealed ALT/AST/ALP/GGT of 162/128/379/270, respectively. Bilirubin and INR were normal. Given the uncertain etiology, further workup was obtained. Hepatitis serologies showed HAV and HBV non-immunity with Twinrix vaccination underway and a negative HCV antibody. Antinuclear antibodies (ANA) were elevated in a 1:1280 nuclear membrane pattern, suggestive of PBC. IgG was elevated to 1978 mg/dL, suggestive of AIH. Additional laboratory workup was noncontributory. MRI displayed mild hepatomegaly, a 9.2% hepatic fat fraction, hemangioma, and a lesion suggestive of focal nodular hyperplasia. Vasculature was patent and there were no findings of portal hypertension. Elastography revealed a controlled attenuation parameter score of 308 dB/m and stiffness of 17 kiloPascal, consistent with advanced fibrosis. Due to this incongruence between imaging and elastography, and elevated ANA and IgG levels liver biopsy was obtained. Biopsy was consistent with AIH-PBC overlap syndrome. Treatment focused on the predominant pattern of PBC and ursodiol 13-15 mg/kg/daily was initiated. Discussion: Accurate and timely diagnosis of AIH-PBC overlap syndrome is crucial in preventing disease progression. Diagnosis is based on autoimmune markers and elevated liver tests, with liver biopsy as the gold standard. Although both MRI and elastography may have a role for non-invasive evaluation, our case displays the need for laboratory evaluation and biopsy to confirm the diagnosis. If laboratory and imaging evaluations are non-unifying, biopsy should not be delayed to facilitate accurate and early management of the disease.Figure 1.: A. Liver biopsy with H&E stain demonstrating mild to moderate lymphoplasmacellular inflammation in portal tracts with damaged bile ducts (florid duct lesions), focal ductular reaction, and bile duct loss, suggestive of PBC. B. Trichrome stain demonstrating dense periportal and sinusoidal fibrosis with non-bridging fibrous septa, suggestive of AIH.Table 1.: Paris criteria for AIH-PBC overlap syndrome diagnosis." @default.
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• W3210944189 date "2021-10-01" @default.
• W3210944189 modified "2023-09-23" @default.
• W3210944189 title "S2640 Autoimmune Hepatitis, Primary Biliary Cholangitis or Overlap? A Case Beyond the Single Diagnosis" @default.
• W3210944189 doi "https://doi.org/10.14309/01.ajg.0000784092.52963.3c" @default.
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