I read with interest the excellent review by Czaja on acute severe autoimmune hepatitis (AIH) [1], which greatly increased my awareness of the clinical and therapeutic characteristics of this extremely serious disease. The precise and extensive description of this medical problem provided by Czaja can be of upmost importance for the management of this specific subgroup of patients by clinicians. AIH is a chronic hepatitis of unknown etiology, and its clinical manifestations are highly variable, sometimes following a fluctuating course [2, 3]. An acute, severe, abrupt presentation is also possible and is secondary to variable causes [1]. The diagnosis of AIH is based on a clinical, biochemical, serological and histological patterns, and sometimes has to be confirmed by the response of the disease to immunosuppressive treatment [1–4]. Compared to patients presenting with a more chronic course, those presenting with acute onset more frequently have interface and centrilobular hepatitis, lobular disarray and central necrosis, but perhaps less fibrosis and cirrhosis [1, 2, 5]. Liver biopsy features in the acute presentation of AIH may be difficult to distinguish from those seen in acute hepatitis due to the effects of the virus or drugs [1, 5]. It is not yet clear whether the clinical presentation of AIH in the setting of drugs used for other diseases unmask and/or induce a typical AIH or simply result in a drug-induced hepatitis with features that mimic AIH [6]. Therefore, the histological assessment of acute and chronic liver is based on an accurate description of the pattern of injury within the tissue specimen. Interpretation of the injury pattern requires review of the characteristic clinical and laboratory features, and the morphological findings only achieve relevance if they satisfactorily explain the clinical syndrome [7]. Thus, the importance of the results of the histological analysis of liver samples with respect to the diagnosis and clinical management of severe acute AIH may be unclear and, occasionally, biopsy results may actually delay therapeutic decisions; in other words, biopsy results may be unnecessary for the rapid initiation of treatment given that early steroid therapy may save lives [8–11]. It has recently been reported that patients with typical laboratory features of AIH rarely need liver biopsy for diagnosis. Most patients presenting with features of AIH based on laboratory tests are likely to have a compatible liver histology, and few patients have atypical histology. Consequently, tissue findings may have little impact on patient management. Thus, biopsy samples might not need to be collected from patients who meet other typical criteria for AIH [9]. When the scoring system based upon titers of autoantibodies, immunoglobulin G (IgG) levels and the exclusion of viral hepatitis is used as a diagnostic tool, a score of six points can be used as a simplified criterium of a probable diagnosis of AIH [5]. At admission, the recommended analyses include general biochemistry, conventional autoantibodies, quantification of immunoglobulins and IgG serum levels. It is important to exclude other virus infections, such as hepatitis A and E viruses, hepatitis C virus (serum HCV-RNA R. Moreno-Otero (&) Liver Unit, and Instituto de Investigacion Sanitaria (IIS-IP), Digestive Diseases Service (Planta 3), Hospital Universitario La Princesa, Universidad Autonoma de Madrid, Diego de Leon, 62, 28028 Madrid, Spain e-mail: rmoreno.hlpr@salud.madrid.org; rmorenoo@salud.madrid.org
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Sep 1, 2020
Aymen Tezeghdenti + 5
Open Access