General features of CAH

Chronic hepatitis ranges from an asymptomatic disease recognized only by biochemical abnormalities to one that is severe and progressively cirrhotogenic. Some cases of autoimmune hepatitis can have a long asymptomatic preclinical course, as pertains for other autoimmune disorders. Likewise, an insidious onset and clinical latency is usual for CAH associated with infection with HCV and, in many instances, with HBV. Cases of chronic active hepatitis will have some common general features related to liver dysfunction or cirrhosis, whatever the cause, and other features related to the individual etiological agent or process.

The general features include symptoms of hepatitis, including nausea, anorexia, jaundice, hepato-splenomegaly, and biochemical evidence of liver parenchymal damage, in particular high levels of transminase enzymes in serum. The biopsy of the liver may show either of two histological lesions, named as chronic persistent hepatitis which is indolent and nondestructive, or chronic active hepatitis in which the main morphological feature is the disruption of the peripheral limiting plate of the liver lobule, with a periportal 'spillover' of the inflammatory exudate into the liver parenchyma: 'piecemeal necrosis' (Figure 1). This lesion generates scarring and eventually macronodular cirrhosis (Figure 2). Hepatocellular carcinoma is a frequent late sequel in CAH due to chronic virus infection but is seldom seen in autoimmune hepatitis. There are features unique to each of the individual categories, as indicated below.

Figure 1 Histological appearance of liver biopsy specimen from a female aged 65 years with recent-onset autoimmune hepatitis, showing periportal and lobular hepatitis and extensive hepatocellular necrosis (HE x 200).
Figure 2 Liver from a female aged 45 years with autoimmune hepatitis, showing scarring and a coarse macronodular cirrhosis.
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