Jornal de Biomarcadores Moleculares e Diagnóstico

IgG4 Sclerosing Cholangitis and Post Infantile Giant Cell Hepatitis: A Case Report of an Extraordinary Co-presentation

Abstract

Maha M El-Sabaawy, Eman Abdel-Sameea, Ayat R Abdallah, Ahmed El-Refaey, Mervat Soltan and Nemine Ehsan

Background: Giant cell hepatitis is rarely described in adults; referred to as post infantile giant cell hepatitis (PIGCH). Most reports have mentioned PIGCH's association with systemic lupus erythromatosis, autoimmune hepatitis, lymphoma, and leukemia. However, links with other medical disorders are still evolving.

Reports of primary sclerosing cholangitis (PSC) presenting as IgG4-SC has been typically described in association with other IgG4-related disorders, most frequently autoimmune pancreatitis. However, some cases of isolated IgG4-SC have been reported. Herein; we report a case of IgG4-SC presented by PIGCH.

Case description: A 29-year-old gentleman presented with two month jaundice and biochemical evidence of acute hepatitis. He reported no history of drug exposure, had no gall bladder or pancreatic disease, nor prior similar attacks. The work up revealed negative serology for viral hepatitis. Markers of autoimmune liver disease were negative except for pANCA, and serum levels of pancreatic enzymes, copper and ceruplasmin were normal, and urinary copper was normal.

Results: Abdominal sonography and MRCP showed normal pancreas and biliary tract. ERCP showed that the common bile duct had a single short narrowed segment with thickened walls. Histological examination of colonoscopic biopsies taken from the terminal ileum and colon demonstrated no pathological alterations. Liver histology showed evidence of parenchymal extinction with extensive giant cell transformation, ductular proliferation, cholestasis, and positive IgG4 staining, a picture suggestive of PSC and PIGCH.

Discussion: In this case, we did not test for serum levels of IgG4, and resorted to immune-staining of liver tissue, as this is the hallmark for diagnosing IgG4-SC. Histopathological features and, more definitely, the positive IgG4 immunostaining were present in the liver tissue, which were crucial in diagnosing this case as IgG4-SC (IAC).

Conclusion: This case presented with both IgG4-SC and biopsy proven PIGCH, and had a favourable outcome with biliary drainage and immuno-suppression therapy