儿童自身免疫性肝炎1例

<正>自身免疫性肝炎(autoimmune hepatitis,AIH)可出现高丙种球蛋白血症和自身抗体阳性,临床表现缺乏特异性。多见于女性,且40岁以后发病率高~([1]),最小发病年龄为6个月~([2-4])。目前,儿童AIH的治疗仍以泼尼松联合或不联合硫唑嘌呤为一线用药~([5-7])。如不治疗,其5 a和10 a生存率分别为50%和10%~([8])。1病例摘要患儿女,5岁5个月,藏族。因"皮肤黄、眼黄、尿黄10     

Association of autoimmune hepatitis type 1 in a child with Evans syndrome

Autoimmune hepatitis(AIH) is a progressive liver disease that is often associated with extrahepatic autoimmune disorders. Evans syndrome(ES) is a rare autoimmune disorder, which is characterized by immune thrombocytopenia and autoimmune hemolytic anemia. Association of AIH with ES is rare, especially in children. We report a 3-year-old female with a past medical history of ES who presented with jaundice and significant transaminitis due to AIH type 1. She required multiple treatments with steroids as well as azathioprine, intravenous immunoglobulin and a course of rituximab.     

Eradication of hepatitis C virus infection disclosing a previously hidden,underlying autoimmune hepatitis: Autoimmune hepatitis and HCV

Chronic hepatitis C virus (HCV) infection and autoimmune disorders show a complex interplay, with HCV often being identified as the trigger of autoimmune phenomena or diseases. While there is evidence of successful HCV treatment with direct-acting antivirals (DAA) in patients with concomitant HCV and autoimmune hepatitis (AIH), there are also sparse reports of AIH developing during, or following, DAA treatment.Here we report a case of a patient with suspected concomitant HCV and AIH who underwent liver biopsy but showed no histological hallmarks of autoimmunity. The patient later developed a hepatitic flare following DAA-induced viral clearance, and a second liver biopsy showed features compatible with AIH. Response to corticosteroid and azathioprine treatment was seen. This reports demonstrates that patients with features of auto-reactivity and HCV after DAA-induced viral clearance require careful follow-up.     

Autoimmune hepatitis in a patient with systemic lupus erythematosus

We report a female patient with systemic lupus erythematous (SLE), hyperbilirubinemia and high serum value of ALT. International autoimmune hepatitis (AIH) score showed definite AIH before treatment, but autoantibodies could not make a differential diagnosis of AIH and SLE-associated hepatitis. Liver biopsy showed periportal hepatitis with lymphoplasmacytic infiltration, but neither parenchymal collapse nor rosette formation could be found. Pericarditis, pleuritis and nephritis were improved as well as liver injury after administration of prednisolone, and no repeated attack has been present these 4 years. Our case suggested invalidity of AIH score among patients of SLE, and liver histology should be inferred most important at present to make a differential diagnosis of lupus hepatitis or AIH in patients with SLE.AcknowledgementThe paper was reviewed by Dr. D. Murouzeck whose native language is English.     

Autoimmune reactivity of sera to hepatocyte plasma membrane in type 1 autoimmune hepatitis

Type 1 autoimmune hepatitis (AIH-1) is an organ-specific autoimmune liver disease for which no tissue-specific autoantigen has yet been identified. We examined the reactivity by sensitive immunoblotting with enhanced chemiluminescence (IB-ECL) of 43 sera from patients with AIH-1 and 182 sera from patients with other diseases on hepatocyte plasma membrane derived from rat or human liver (RHPM, HHPM) and separated by aqueous two-phase partition. The sera studied were from patients with AIH-1, primary biliary cirrhosis, chronic viral hepatitis, and systemic lupus erythematosus (SLE); and from normal subjects. Specificity of reactivity by IB-ECL was sought: (i) by testing sera on human or rat liver membrane; (ii) by testing sera on liver or kidney membrane; (iii) by serial titration of reactive sera; and (iv) by testing reactive sera from AIH-1 before and after successful treatment with prednisolone. The results were that in AIH-1 there were multiple reactive components which were not species-specific, since they were detected with both RHPM and HHPM, but were mostly tissue-specific for liver. There was no significant correlation between antinuclear antibodies (ANA) titer and the frequencies of sera reactivities against RHPM. Most of these reactive components were demonstrable at a lesser frequency in other liver diseases and in SLE. There was a striking decrease in reactivity by IB-ECL of AIH-1 sera with liver membrane after clinical remission, further suggesting that differences between AIH-1 and other inflammatory liver diseases and SLE are predominantly quantitative rather than qualitative. However, our study did point to candidate liver membrane antigens with molecular sizes of 136, 116, 81, and 49 kDa, additional to components previously described by others. The molecular identification of these prominent reactants with AIH-1 sera could prove informative for ascertaining pathogenesis. Received: July 23, 1999 / Accepted: September 24, 1999     

Autoimmune cholangitis with features of autoimmune hepatitis: successful treatment with immunosuppressive agents and ursodeoxycholic acid

We report a 42-year-old Chinese female with elevated serum levels of liver aminotransferases, alkaline phosphatase, gamma-glutamyl transpeptidase, cholesterol and immunoglobulin M. Serum antimitochondrial antibody was negative, but antinuclear antibody was strongly positive. Liver histology showed features of both autoimmune cholangitis and autoimmune hepatitis. Combination therapy with immunosuppressive (prednisone and azathioprine) and choleuretic agents (ursodeoxycholic acid) was given. Serum aminotransferases and biliary enzymes showed much improvement after treatment. A follow-up liver biopsy showed improvement of both hepatic necroinflammation and bile duct damage. Biliary enzymes rose after withdrawal of the immunosuppressive agents and declined again with reinstitution of prednisone. This case demonstrates that a combination of immunosuppressive agents and ursodeoxycholic acid may effectively treat patients with features of both autoimmune cholangitis and autoimmune hepatitis.     

Autoimmune hepatitis: a review

Autoimmune hepatitis (AIH) is an inflammatory liver disease that predominantly affects females. The disease is characterized histologically by interface hepatitis, biochemically by increased aspartate and alanine aminotransferase levels, and serologically by the presence of autoantibodies and elevated levels of immunoglobulin G. AIH affects both adults and children, and is particularly aggressive in the latter group. It is a relatively rare but devastating disease, which progresses rapidly unless immunosuppressive treatment is started promptly. Treatment is often successful at inducing remission of disease, and this can lead to a normal life expectancy. However, progression to cirrhosis can and does occur in some. For those with advanced-stage disease and complications, consideration of liver transplantation is appropriate.     

Autoimmune neutropenia disclosing AIDS in a child

A 15 month old child with a history of multiple infectious diseases was admitted to hospital for investigation of pyrexia and general ill health. She was anaemic with a persistent neutropaenia associated with hypergamma globulinemia, indicating intense autoimmune activity: cellular immunity was abnormal with a normal total lymphocyte count but a very low T4/T8 ratio. HIV serology was positive; the virus was isolated from a lymph node biopsy specimen. The parents for the child were HIV positive and the father went on to develop full blown AIDS. The neutropaenia was constant over the two years of follow-up and granulo-immunofluorescence studies suggested an autoimmune origin. In contrast to autoimmune thrombocytopaenia and haemolytic anaemia, autoimmune neutropaenia is a rare condition. A few cases have been reported in adult AIDS. Our case is of additional interest as it illustrates the vertical mode of transmission of AIDS.     

Autoimmune hepatitis with giant-cell transformation

Background/Aims: Giant-cell hepatitis (GCH), also known as postinfantile or syncytial giant cell hepatitis, is a frequent pattern of liver injury in the neonate, primarily seen in the first three months of life. Few cases in adults have been reported, some of them associated to autoimmune diseases such as autoimmune hepatitis.Methods: We present a case of autoimmune hepatitis with giant cell transformation in a 39 year old male with polyarthritis. We discuss his clinical presentation and course. We made a review of the literature of agents associated to this diagnosis, the mechanisms involved in the formation of giant hepatocytes, the histological findings, clinical course, treatment options and prognosis of this rare entity.Results and conclusions: In conclusion, the clinical course varies from normalization of hepatic histology to progression to cirrhosis and liver failure. The prognosis is dictated by the underlying liver disease and in the setting of autoimmune hepatitis the clinical course is usually severe with most of the patients progressing to cirrhosis. Prolonged treatment with corticosteroids and immunosuppressants is usually effective in rendering the cirrhosis inactive.     

Autoimmune hepatitis with membranous glomerulonephritis

We report on a case of autoimmune hepatitis (AIH) associated with membranous glomerulonephritis. A 61-year-old woman was admitted because of peripheral edema, proteinuria and abnormal liver function test findings. A diagnosis of AIH was made on the basis of an elevation of aminotransferase and serum IgG levels, the presence of positive antinuclear antibody and the characteristic histological features of chronic active hepatitis. Histological examination of a renal biopsy specimen disclosed membranous glomerulonephritis with granular deposits of IgG, IgM, C3 and C1q along the capillary walls. This condition is rare in AIH and should be carefully distinguished from systemic lupus erythematosus.