Autoimmune Hepatitis in Untreated AIDS: Case Report and Literature Review

Abstract

Autoimmune hepatitis (AIH) is a disorder of the immune system, in which autoantibodies are created resulting in inflammation and liver injury. Proposed mechanisms include cell mediated cytotoxicity of CD4 cells and direct toxicity of antigen-antibody complexes, with viruses implicated as triggers. AIH is rare, with an incidence of < 1% per year. In literature, AIH has usually been reported in HIV in the setting of HAART therapy or immune reconstitution syndrome. We present a case of AIH in a patient with untreated AIDS with extremely low CD4 count and viremia. A 49 year old female with HIV for >5 years presented with one month history of jaundice, abdominal pain, and pruritus. She denied any medications, alcohol or intravenous drug use, and was not on HAART therapy. Exam revealed jaundice, scleral icterus, and no stigmata of chronic liver disease. Labs showed ALT of 1040 U/L, AST of 1394 U/L, ALP of 238 U/L, and total bilirubin of 14.5 mg/dL. CD4 count was 18 #/mm and HIV viral load was 29,422 copies/mL. Extensive diagnostic workup for liver disease was performed. ANA and anti-LKMA were negative, however IgG was elevated at 2510mg/dL and ASMA was elevated at 1:160. Liver biopsy showed chronic active hepatitis with mild interface activity and plasma cell rich infiltrate. She scored 20 on the revised criteria of the International Autoimmune Hepatitis Group, consistent with AIH. The patient was started on Prednisone with both symptomatic and laboratory improvement. HAART therapy was initially deferred due to liver injury, but will ultimately be started. AIH is an extremely rare disease, and diagnosis relies on clinical criteria as well as pathological data. It is even more rare in patients with HIV, with only 12 cases of AIH in HIV patients reported in the literature. The CD4 counts of those reported patients were exclusively greater than 157. This case is, to our knowledge, the lowest reported CD4 count of an HIV-positive patient who developed AIH. It highlights the importance of maintaining autoimmune disorders within the differential in patients with advanced AIDS. Furthermore, treatment poses an interesting dilemma, as there has been success in treating with HAART therapy as well as standard measures including steroids. Overall, the case highlights that immunosuppressive therapy can be safely and effectively given to HIV patients with AIH with very low CD4 counts ( < 200). This can be done short term without concurrent HAART therapy, if needed.Figure 1Figure 2Figure 3