A Rare Case of Autoimmune Hepatitis in an Immunocompromised Patient

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The American Journal of Gastroenterology



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Autoimmune hepatitis (AIH) is a rare condition of liver inflammation associated primarily with overactive CD4 cells, but also with CD8 cells in later stages. The few previous reports of AIH in HIV patients occurred only in those with preserved CD4 counts or reconstitution of immunity after starting antiretroviral therapy (ART). In these cases, corticosteroids remained the standard of care and most patients had a favorable response. Here, we present the first reported case of a biopsy confirmed AIH in a patient with newly diagnosed AIDS not previously on ART therapy. 42-year-old male with history of Type 2 DM, hypertension, and HIV presented with pruritus and jaundice worsening over months. He was diagnosed many years ago with HIV with preserved immunity, but has since then had no follow up. Physical exam revealed scleral icterus, jaundice, and distended abdomen with hepatomegaly. Current labs include CD4 94, viral load 349,000, AST 100, ALT 210, T-bili 11.2, D-bili 9.1, alk phos 345, ANA 1:640, anti-smooth muscle positive, and CMV PCR of 203 (normal<200). Liver biopsy showed moderate chronic hepatitis with plasma cells and peri-portal fibrosis (Image 1) while CMV tissue stain was negative (Image 2), confirming the diagnosis of AIH. After considering the severity of his clinical symptoms, ongoing liver damage, and risk of fulminant hepatic failure, patient was started on both Prednisone and ART therapy, to which he had rapid biochemical and clinical improvement and was discharged by day 3 of treatment. He will be monitored outpatient on his progress and for any signs of opportunistic infection. Autoimmune diseases are uncommon in AIDS patients. It appears a paradox to have compromised immunity co-exist with a disease of overactive immune system. Existing research shows that as HIV specifically inactivates CD4 cells, it can co-occur with autoimmunediseases involving CD8 cells—most often psoriasis, reactive arthritis, and uveitis. These can even be considered indicators of AIDS syndrome, and standard of care is symptom management and ART therapy. In this case, we had an HIV patient not on ART therapy whose initial AIDS manifestation was active AIH. We suspect that his worsening AIH correlated with his AIDS progression due to the relative increase in CD8 in proportion to CD4. This case could set precedent for further studies on the pathophysiology and establish treatment guidelines for more aggressive autoimmunediseases in the immunocompromised patient.