Daas et al. Journal of Medical Case Reports 2011, 5:233 http://www.jmedicalcasereports.com/content/5/1/233 Discussion Our review of the literature revealed 12 cases of autoim- mune hepatitis in patients with HIV infection [2-8]. Three had co-infections with hepatitis C virus and were receiving interferon therapy [6-8] and six more had AIH before starting HAART and one pediatric patient’s data were missing. Only three patients developed AIH after starting HAART, similar to our patient. The clinical characteristics of the reviewed cases and our case are shown in Table 1. All patients who received HAART prior to AIH had a significant rise in CD4 count and undetectable HIV RNA before AIH was diagnosed. Two patients had other concomitant autoimmune diseases, one with Grave’s disease and the other with diffuse infil- trative lymphocytic syndrome. This review illustrates that AIH in patients with HIV infection on HAART is rare. It has been encountered in women who had signif- icant elevations in CD4 count, suggesting the emergence of AIH during immune restoration. It presented insi- diously with non-specific manifestations. The diagnosis is usually based on AIH score, the absence of other con- ditions and characteristic histopathological findings. In patients who develop liver function abnormalities while receiving HAART, it is important to exclude drug-induced liver disease. In our patient, the pathogno- monic findings on the liver biopsy and the fact that she had been taking these medications long before she developed symptoms indicate that a drug reaction was not likely. The prognosis associated with AIH in patients with HIV infection appears to be variable based on a review of 11 out of the 12 reported cases (one reported case’s data were missing) of AIH in patients with HIV infec- tion. Two patients died while receiving interferon ther- apy for hepatitis C virus that triggered fulminant AIH, and one died as a result of severe Pneumocystis jiroveci Page 3 of 4 pneumonia while receiving high-dose steroids for the treatment of AIH. Our patient had evidence of SLE in addition to AIH. Whether AIH is a manifestation of SLE or is unrelated is unclear. Liver involvement associated with AIH is relatively rare, ranging from 1.2% to 2% in patients with SLE who do not have HIV infection [9]. To date, it has not been reported simultaneously with SLE in patients with HIV infection. The precise mechanism causing the emergence or unmasking of autoimmune conditions in patients who are HIV-positive who commence anti-retroviral therapy is complex and involves multiple cytokines and lympho- cyte subsets [10-14]. Th17 cells have recently been implicated in association with chronic autoimmunity phenomena and especially in patients with HIV infec- tion and primates with simian immunodeficiency virus [10]. An additional subset of CD4+ regulatory T cells (Treg) has been described. It constitutively expresses CD25 and the transcription factor FoxP3 and has regu- latory functions [1]. Although the levels of Treg in patients who are HIV-positive with autoimmune mani- festations have not been reported, it seems plausible to propose that preferential depletion of Treg in some indi- viduals could account for the increased autoimmune phenomena in some patients with acquired immunodefi- ciency syndrome [15-18]. Conclusion The present case report and review of the literature describes a rare complication of immune restoration in patients with HIV infection in the era of HAART. Recognizing AIH in the context of immune reconstitu- tion and initiating appropriate therapy can be lifesaving. Treatment of these patients appears to be similar to that of patients without HIV infection. Table 1 Characteristics of 11 reported patients with concomitant HIV and AIHa Case reports CD4b/CD4c VLb/VLc Patient age (years)/gender Onset AIH score Outcome Other AI diseases ART German et al. [2] 38d/man Chronic 216/384 81,000/< 50 Probable Excellent Vetilligo Yes Coriat and Podevin [7] 48/woman Acute 250 Undetectable Probable Died None Yes Puius et al. [3] 29/man Chronic 259/174 7122/27,732 Probable Excellent None Yes 45d/woman Chronic 253/297 8687/< 50 Probable Excellent DILS Yes 65/woman Acute 200/922 Undetectable/< 75 Definite Excellent None Yes O’Leary et al. [4] 44d/woman Chronic 269/526 4927/< 50 Definite Excellent Grave’s disease Yes Wan et al. [5] 56/man Chronic 331/NA 232,734/NA Probable Died None 54/man Chronic 357/213 5104/NA Probable Probable Cirrhosis Yes 55/woman Chronic 174/NA < 50/NA Probable Probable Poor Yes 49/woman Acute 286/NA 69,318/NA Definite Definite Died Yes Our patient 42d/woman Acute 157/232 120,000/< 50 Definite Excellent SLE Yes aTwo patients are not included because of lack of complete clinical data. HIV = human immunodeficiency virus; AIH = autoimmune hepatitis; AI, autoimmune; ART = antiretroviral treatment; VL = viral load; DILS = diffuse infiltrative lymphocytic syndrome; SLE = systemic lupus erythematosus; bprior to initiating highly active antiretroviral treatment (HAART); cafter initiating HAART; dpatients with significant viral suppression on HAART who developed AIH.