Autoimmune hepatitis variants: Definitions and treatment
- Michael A Heneghan, MD, MMedSc, FRCPI
Michael A Heneghan, MD, MMedSc, FRCPI
- Consultant Hepatologist & Reader in Hepatology
- Clinical Director for Liver Services
- Institute of Liver Studies, King's College Hospital
Patients with variant forms of autoimmune hepatitis have clinical and serologic findings of autoimmune hepatitis plus features of other forms of chronic liver disease. As will be discussed below, the taxonomy of these variants (often referred to as overlap syndromes) is controversial. The overlap syndromes of autoimmune hepatitis with primary biliary cholangitis and primary sclerosing cholangitis will be reviewed here. The discussion is generally consistent with an international consensus statement published in 2011 .
Autoimmune hepatitis is a chronic hepatitis of unknown etiology characterized by hyperglobulinemia, the presence of circulating autoantibodies, and inflammatory changes on liver histology . Most cases respond to therapy with immunomodulating drugs such as prednisone and azathioprine or 6-mercaptopurine. (See "Autoimmune hepatitis: Disease classification" and "Autoimmune hepatitis: Treatment".)
Patients with certain cholestatic liver diseases (such as primary biliary cholangitis [PBC] and primary sclerosing cholangitis [PSC]) have clinical and serologic features suggesting the presence of autoimmune hepatitis and also may respond to immunosuppressive therapy. Conversely, some patients with features predominantly suggesting autoimmune hepatitis have findings compatible with other forms of chronic liver disease. In a report from the Mayo Clinic, for example, 225 patients with either type I autoimmune hepatitis, PBC, or PSC (162, 37, and 26 patients, respectively) defined by standard criteria, were analyzed for serologic and clinical features suggesting variant forms of autoimmune hepatitis . Among these patients, 18 percent had diseases with overlapping features, including:
●7 percent with autoimmune hepatitis/PBC
●6 percent with autoimmune hepatitis/PSC
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- SCORING SYSTEMS
- AUTOIMMUNE HEPATITIS-PBC OVERLAPS
- AUTOIMMUNE HEPATITIS-PSC OVERLAPS
- AMA-positive without histologic evidence of PBC
- ANA and/or SMA-positive with histologic changes suggesting PBC
- Autoimmune hepatitis-PSC overlap
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS