Acute presentation of autoimmune hepatitis: Does it exist? A published work review

Abstract

Autoimmune hepatitis (AIH), initially called chronic aggressive hepatitis, is conceptually classified as a primary chronic disease; periportal fibrous expansion and periportal infiltration of mononuclear cells, including plasma cells, have also been considered to be histologically important diagnostic signs of AIH. However, several manuscripts which reported the acute presentation of AIH have been published recently and the reported cases of acute presentation in these manuscripts contained two different clinical entities. One is acute exacerbation of chronic AIH (acute exacerbation form) and the other is genuine acute AIH without chronic histological changes (acute form). It is clinically important to distinguish the acute form from the acute exacerbation form. The reports of the acute form revealed that the existence of centrilobular necrosis without chronic changes was the most important histological finding related to the acute form. Because the elevation of serum levels of immunoglobulin G and antinuclear antibody are not observed in some acute presentation AIH patients, the physician may not consider AIH when they encounter such patients. Therefore, it is very important to bear in mind a possibility of acute presentation AIH when the physician encounters patients with hepatic dysfunction of unknown cause because it became clear that delay of the diagnosis and start of therapy lead to the poor prognosis of AIH. In this review, we outline the current state of acute presentation of AIH including the genuine acute form based on the published clinical studies and case reports.

IgG4-Related Disease without Overexpression of IgG4: Pathogenesis Implications

“…Because high serum IgG4 and abundant infiltration of IgG4-bearing plasma cells are prominent characteristics, it was suggested that these findings could define the diagnosis of IgG4-related disease [5]. However, the pathogenesis of IgG4 overexpression remains unclear [6], and it is unknown whether IgG4 is related to the cause of the disease. …”

Autoimmune Hepatitis: Recent Advances in the Pathogenesis and New Diagnostic Guidelines in Japan

“…Acute presentation is histologically characterized by portal fibrosis and moderate to severe inflammatory cell infiltration and consists of the acute exacerbation phase and acute hepatitis phase, which are characterized by acute exacerbation of chronic liver disease and findings indicative of acute hepatitis, respectively (Table 2) (52). Some patients in the acute hepatitis phase show lower serum levels of IgG and negative autoantibody test results, which make confirming the diagnosis difficult (9,53). The possibility of AIH should always be considered when treating patients with acute hepatitis of an unknown cause.…”

“…CN itself is a nonspecific finding observed in cases of drug-induced hepatic injury, heart failure, acute viral hepatitis or ischemia and not a histopathological finding of AIH. According to a review by Takahashi et al, CN is found at a frequency of 53-100% in cases of acute hepatitis of AIH, and CN without portal inflammation is particularly characteristic in the acute hepatitis phase of AIH (53). In addition, in some cases of AIH with an acute presentation, heterogeneous low-density areas are observed on plain CT scans and can be used to differentiate the disease from acute hepatic failure of other causes (57,58).…”

Acute autoimmune hepatitis

“…We read with great interest the report of Takahashi et al ., 1 which is a review of accumulated literature regarding acute presentation of autoimmune hepatitis (AIH), including fulminant cases. They nicely stated that when a physician encounters a patient with acute hepatitis or fulminant hepatic failure of unknown cause, acute AIH should be kept in mind and an early liver biopsy should be performed, if possible.…”

“…They nicely stated that when a physician encounters a patient with acute hepatitis or fulminant hepatic failure of unknown cause, acute AIH should be kept in mind and an early liver biopsy should be performed, if possible. Early diagnosis and timely immunosuppression may save many patients 1 . We would like to make some comments regarding the diagnostic and clinical aspects of acute AIH.…”