Clinical manifestations of autoimmune liver disease

  Autoimmune liver diseases are a group of autoimmune diseases in which the liver is the organ of relatively specific immunopathological damage. They mainly include: autoimmune hepatitis (AIH), primary biliary cirrhosis (PBS) and primary sclerosing cholangitis (PSC) and overlapping syndromes between any of these three diseases, often combined with extrahepatic immune diseases. The diagnosis is based on specific biochemical abnormalities, autoantibodies and dry tissue features. With the improvement of understanding and diagnosis, the prevalence of autoimmune liver disease has been reported to be increasing year by year both at home and abroad, thus receiving increasing attention and concern.  Autoimmune hepatitis is a chronic inflammatory disease of the liver caused by autoimmune reactions. The liver tissue changes are consistent with chronic viral hepatitis, but serum viral markers are negative.  Clinical manifestations Clinically, there are various manifestations of autoimmunity, such as jaundice, fever, rash, arthritis and other various symptoms, and hypergamma-globulinemia, accelerated hematocrit and positive autoantibodies in the blood are seen. About 20% to 25% of patients have an onset similar to that of acute viral hepatitis, manifesting as jaundice, poor appetite and abdominal distension.  Vulnerable groups Clinical manifestations are more common in women, mainly in adolescence. In addition, menopausal women are also more common. The onset of the disease is mostly insidious or slow, and may include joint pain, low-grade fever, malaise, rash, amenorrhea, etc.  The disease is characterized by extrahepatic manifestations, and sometimes other symptoms can mask the existing liver disease.  The extrahepatic manifestations of the disease include: (1) symmetric, wandering arthritis, which may be recurrent without joint deformity; (2) low-grade fever, rash, cutaneous vasculitis and subcutaneous bleeding; (3) endocrine disorders, acne, hirsutism, amenorrhea in women, gynecomastia, hyperthyroidism, and diabetes mellitus; (4) tubular acidosis, glomerulonephritis; (5) pleurisy, interstitial pneumonia, pulmonary atelectasis, and (5) pleurisy, interstitial pneumonia, pulmonary atelectasis, fibrous alveolitis; (6) ulcerative colitis, dry syndrome.  Primary biliary cirrhosis is a chronic progressive and often fatal bilious liver disease that occurs mainly in middle-aged women and is characterized by nonsuppurative truncated destruction of intrahepatic bile ducts, portal inflammation, scarring, and ultimately cirrhosis, portal hypertension, and liver failure. The etiology of the disease is not clear, but viral, bacterial, and fungal infections, environmental toxicological factors, selenium deficiency, toxicity, and genetic factors are involved.  Since the etiology is not clear, its pathogenesis is also unknown. It is generally believed that, on the basis of pre-existing genetic susceptibility, persistent infections or toxic effects occur, leading to autoimmune reactions and eventually primary biliary cirrhosis. Patients with this disease have functional abnormalities of the immune system, including significantly elevated serum immunoglobulins, reduced number of T lymphocytes in peripheral blood, dysfunction and deregulation. The characteristic manifestations of the disease are women over middle age with pruritus, hepatomegaly, and the presence of macular tumors.