Clinical and histological features of autoantibody-negative autoimmune hepatitis in Chinese patients: a single center experience.

Objective This study aimed to define the clinical features of Chinese patients with autoantibody-negative autoimmune hepatitis (AIH) and to refine the diagnosis and management of these atypical patients in a single Chinese center. Methods  A retrospective evaluation of 167 Chinese patients with AIH was performed. Patients meeting comprehensive criteria with the absence of antinuclear antibodies, smooth muscle antibodies, liver-kidney microsomal-1 antibodies and antimitochondrial antibodies were defined as autoantibody-negative patients. Results  In total, 17 (10.2%) of the 167 patients with AIH were autoantibody-negative. The general status and biochemical tests between the classical and autoantibody-negative patients were not significantly different (P > 0.05). Serum immunoglobulin G levels of the autoantibody-negative AIH patients were lower than those of the classical AIH ones (P = 0.004). There was no significant difference in the histological inflammatory grades between the two groups; however, advanced histological stages were more common in the autoantibody-negative AIH group (P < 0.001). In the autoantibody-negative AIH patients, 11 (64.7%) had a possible diagnosis and 6 (35.3%) had a definite diagnosis according to the comprehensive criteria. While with the simplified criteria only 3 patients (17.6%) had a possible diagnosis, and none had a definite diagnosis of AIH. The complete biochemical remission rate was 86% within 24 months of immunosuppressive therapy, which was comparable to classical AIH (P = 0.658). Conclusion Autoantibody-negative AIH is not uncommon in Chinese AIH patients, and has a good response to immunosuppressive treatment comparable to classical patients.