Answer: Celiac hepatitis
Endoscopic biopsy of this severely scalloped duodenal mucosa demonstrated characteristic findings of gluten-sensitive enteropathy, or celiac disease. Celiac disease involvement of the liver is a common extraintestinal manifestation of this immune-mediated disorder, termed celiac hepatitis. Celiac hepatitis affects 40% of adults with celiac disease.1 The pathogenesis is poorly understood, but posited to be related to autoimmunity or toxin-mediated liver injury in the setting of gluten exposure, gut permeability, chronic inflammation, and host susceptibility, among other mechanisms.1-3
Clinical manifestations of celiac hepatitis range from unexplained enzyme elevations in the absence of known liver disease to autoimmune hepatitis to hepatic steatosis, and even cirrhosis.1 The initial presentation can also be elevated liver enzymes in the setting of known celiac disease, without known hepatic disease. Histology of the liver is similarly variable, from a mild or a chronic hepatitis to steatohepatitis and even fibrosis.2 Elevated transaminases less than five times the upper limit of normal when found at celiac diagnosis suggest celiac hepatitis, and do not require further workup.1 For these individuals, response to a gluten-free diet should be monitored and liver chemistries should be repeated at 6–12 months. Persistently elevated aminotransferases should prompt further workup.1 Generally, enzyme elevation and even the histologic appearance of the liver improve after implementation of a gluten-free diet, although not all.2 In celiac hepatitis associated with autoimmune liver disease, immunosuppression may be required in addition to abstaining from gluten.3 Our patient was found to have a tissue transglutaminase level > 100 U/mL (normal, < 4 U/mL). He began a gluten-free diet guided by a nutritionist 4 weeks ago, with rapid improvement in abdominal symptoms, and will be followed to ensure normalization of liver enzymes, which can take up to 1 year.
1. Rubio-Tapia A, Murray JA. Liver involvement in celiac disease. Minerva Med. 2008;99:595-604.
2. Majumdar K, Sakhuja P, Puri AS, et al. Coeliac disease and the liver: spectrum of liver histology, serology and treatment response at a tertiary referral centre. J Clin Pathol. 2018;71:412-9.
3. Marciano F, Savoia M, Vajro P. Celiac disease-related hepatic injury: insights into associated conditions and underlying pathomechanisms. Dig Liver Dis. 2016;48:112-9.