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1.
OBJECTIVES: To review the course, predisposing risk factors, treatment employed, and complications of autoimmune hepatitis (AIH) during pregnancy. Maternal and fetal outcomes will be discussed. METHODS: We reviewed the literature from February 1966 to January 2004 using MEDLINE and the key words autoimmune hepatitis, chronic active hepatitis, lupoid hepatitis, and pregnancy. An additional case of ours was included as she had AIH since childhood with worsening of liver disease during pregnancy. RESULTS: Including the present case, 58 pregnant women with AIH were reported in 17 case reports and series. In the 101 pregnancies documented in these cases, there were 47 flare-ups, 5 clinical improvements, 45 stabilizations of the disease during pregnancy, and 4 cases in which the disease course was not reported. Two maternal deaths occurred. A perinatal mortality of 4% and 19 fetal deaths were described. Most women were treated with prednisone alone; however azathioprine was used in a number of patients. CONCLUSIONS: Pregnancy course in patients with AIH is unpredictable. AIH exacerbates in some patients and is associated with a high rate of fetal complications including prematurity and death. Low-dose prednisone seems to be the preferred treatment. The use of azathioprine must be individualized and further studies are needed to better define its role and safety during pregnancy in patients with AIH. RELEVANCE: A better understanding of the course of pregnancy in patients with AIH should help design appropriate therapeutic schemes to improve pregnancy outcomes for both mother and fetus.  相似文献   

2.
Abstract Fertility is reduced in women with chronic active autoimmune hepatitis (AIH) and pregnancy is hazardous. This report describes a 33 year old woman with AIH and cirrhosis in whom a successful pregnancy was achieved following in vitro fertilization/embryo transfer. Disease exacerbation during pregnancy was controlled by azathioprine and an increased dose of prednisone, and a healthy child was delivered by Caesarean section at 36 weeks gestation. Since the perinatal care of preterm infants and the obstetric care available to women with complicated medical problems has improved markedly in recent years and since active disease can be controlled by adequate immunosuppressive therapy, we propose that it is justified to allow these patients access to in vitro fertilization programmes.  相似文献   

3.
Abstract: Little is known about the evolution of autoimmune hepatitis (AIH) during pregnancy. Some authors reported worsening of the liver disease during pregnancy, whereas others reported stable conditions. We present two untreated patients who had remission of the autoimmune hepatitis in the second half of their pregnancies. One of the patients exhibited this phenomenon twice during two consecutive pregnancies. We speculate that the immunosuppressive effect of pregnancy induced remission of the autoimmune hepatitis in our patients.  相似文献   

4.
"Overlap syndromes" have been reported among various autoimmune liver diseases, particularly between primary biliary cirrhosis and autoimmune hepatitis (AIH) in adults and between AIH and autoimmune cholangitis in children. The overlap syndrome of AIH and primary sclerosing cholangitis (PSC), however, has been scarcely reported. Furthermore, in most of the reported cases of AIH/PSC overlap syndrome, PSC and AIH were believed to occur simultaneously. We report a case of a 34-year-old woman who has ulcerative colitis and PSC (diagnosed by colonoscopy, histology, and cholangiogram) and 7 years later develops rapidly progressive liver failure and hemolytic anemia from AIH. Liver biopsy showed dense portal lymphoplasmacytic infiltrate with interface hepatitis and acidophil bodies confirming AIH. She responded well to immunosuppressive therapy with steroids, both with respect to her liver disease and her autoimmune hemolytic anemia. Additionally, her clinical symptoms of fatigue, jaundice, and pruritus improved markedly and quickly. Overlap or "crossover" syndrome should be considered in all patients with PSC when they present with sudden deterioration of the liver function and changes in liver enzymes. By making the diagnosis of AIH in a patient with well-established PSC, appropriate treatment can be initiated, resulting in the patient's prompt recovery.  相似文献   

5.
Management and outcome of pregnancy in autoimmune hepatitis   总被引:5,自引:0,他引:5       下载免费PDF全文
BACKGROUND: There is a paucity of data in the literature on the risks associated with, and optimal management of, pregnancy in patients with autoimmune hepatitis (AIH). AIMS: To assess maternal and fetal outcomes in relation to clinical management of pregnancy in a large cohort of patients with well defined AIH. METHODS: A review of all known pregnancies in 162 females with definite AIH attending our clinics between 1983 and 1998, with respect to treatment, natural history, and outcome. RESULTS: Thirty one live births (one twin) resulted from 35 pregnancies in 18 women (seven with cirrhosis). Median age at conception was 28 years (range 18-36). Two patients presented with AIH de novo during pregnancy. At conception, in 15 pregnancies patients had been receiving azathioprine alone or (in nine) with prednisolone, in seven prednisolone alone, and in one cyclosporin. Fetal loss at > or =20 weeks' gestation occurred in two instances. Flares in disease activity occurred during four pregnancies and within three months of delivery in a further four. Among the 31 children born (median follow up 10 years) only two abnormalities have been identified: Perthes' disease in one and severe mental and physical handicap in a second who was born prematurely following decompensation of the mother's liver disease. Neither mother was receiving azathioprine. CONCLUSIONS: Successful completion of pregnancy is a realistic expectation for patients with well controlled AIH. Treatment options vary, but azathioprine appears to be generally safe and without adverse outcomes for mother or baby. Vigilance is required, however, and patients need to be monitored carefully during pregnancy and for several months post partum.  相似文献   

6.
Liver test abnormalities have been described in up to 60% of patients with systemic lupus erythematodes (SLE) at some point during the course of their disease. Prior treatment with potentially hepatotoxic drugs or viral hepatitis is commonly considered to be the main cause of liver disease in SLE patients. However, in rare cases elevated liver enzymes may be due to concurrent autoimmune hepatitis (AIH). To distinguish whether the patient has primary liver disease with associated autoimmune clinical and laboratory features resembling SLE - such as AIH - or the elevation of liver enzymes is a manifestation of SLE remains a difficult challenge for the treating physician. Here, we present six female patients with complex autoimmune disorders and hepatitis. Patient charts were reviewed in order to investigate the complex relationship between SLE and AIH. All patients had coexisting autoimmune disease in their medical history. At the time of diagnosis of AIH, patients presented with arthralgia, abdominal complaints, cutaneous involvement and fatigue as common symptoms. All patients fulfilled the current diagnostic criteria of both, AIH and SLE. Remission of acute hepatitis was achieved in all cases after the initiation of immunosuppressive therapy. In addition to this case study a literature review was conducted.  相似文献   

7.
BACKGROUND AND AIMS: Autoimmune hepatitis (AIH) type 2 is identified by the presence in the serum of anti-liver/kidney microsome type 1 autoantibody. Anti-liver cytosol autoantibody has been reported in children with autoimmune liver disorders mostly in association with anti-liver/kidney microsome reactivity. However, its role as a sole marker of AIH type 2 is debated. We describe here a series of 18 children and adolescents (15 girls, 3 boys) with AIH with serum anti-liver cytosol type 1 (aLC1) as the only autoimmune marker. METHODS: A retrospective review was conducted from 3 pediatric hepatology units of all children with an autoimmune liver disease associated with aLC1 as found by immunofluorescence and/or immunodiffusion or immunoblotting. RESULTS: Age at first symptoms ranged from 11 months to 14 years; 12 children presented with acute hepatitis, 1 with progressive jaundice, and 5 were asymptomatic. Anti-liver/kidney microsome, antimitochondria, and anti-actin autoantibodies were not detected. Signs of cirrhosis were present in 11 children. Immunosuppressive treatment was effective in all except 2 children who had subfulminant hepatic failure and who required liver transplantation. Sixteen patients (14 with their native liver) currently are alive; 14 patients still are on immunosuppressive therapy after 1 to 22 years. According to the international scoring system for the diagnosis of AIH, 16 patients corresponded to a definite diagnosis and 2 corresponded to a probable diagnosis. CONCLUSIONS: The presence of aLC1 in children with acute or chronic liver disease of unknown origin strongly supports a diagnosis of AIH and is an indication for early immunosuppressive therapy.  相似文献   

8.
OBJECTIVE: LKM-positive, or type 2, autoimmune hepatitis is characterized by the presence of antibodies directed against liver-kidney microsomes (LKM1). Although described frequently in southern Europe and the Mediterranean, this subtype of autoimmune liver disease seems to be extremely rare in northern Europe and in the United States. We report here five cases of LKM-positive autoimmune hepatitis that were seen at our center in the period 1989-1999. METHODS: We reviewed the medical records of all patients with the diagnosis of AIH in our institution during the period 1989-1999, and found that five patients had type 2 AIH. All patients were female; four of five were young, and four of five presented with overt cirrhosis. RESULTS: One patient died, one underwent liver transplantation and two are currently awaiting liver transplantation. Response to conventional immunosuppressive therapy was poor and two patients required treatment with cyclosporine and tacrolimus respectively. Four of five patients had at least one associated autoimmune disorder, including IgE-induced IgA deficiency, idiopathic thrombocytopenic purpura (ITP), and arthritis. HLA class II DR4 was present in two patients. CONCLUSIONS: LKM-positive autoimmune hepatitis seems to be a subset of autoimmune hepatitis with distinct clinical features; although rare, it is occasionally encountered in the western United States. Prompt diagnosis and appropriate immunosuppressive treatment are recommended, as well as early referral to transplantation centers. Clinicians should be aware of this condition in the setting of young female patients with unexplained severe liver disease.  相似文献   

9.
This article describes cases of anti-tumor necrosis factor (TNF)-α-induced autoimmune hepatitis and evaluates the outcome of these patients in relation to their immunosuppressive strategy. A retrospective analysis of medical records was performed in our center, in order to detect cases of autoimmune hepatitis (AIH) associated with anti-TNF biologic agents. We describe and analyze eight cases of AIH following anti-TNF therapy, 7 with infliximab and 1 with adalimumab. A distinction should be made between induction of autoimmunity and clinically evident autoimmune disease. Liver biopsy is useful in detecting the role of the TNF-α antagonist in the development of AIH. The lack of relapse after discontinuing immunosuppressive therapy favors, as in this case series, an immune-mediated drug reaction as most patients with AIH have a relapse after treatment is suspended. Although AIH related to anti-TNF therapy is rare, a baseline immunological panel along with liver function tests should be performed in all patients with autoimmune disease before starting biologics.  相似文献   

10.
Overlap syndromes among autoimmune liver diseases   总被引:4,自引:0,他引:4  
The three major immune disorders of the liver are autoimmune hepatitis(AIH),primary biliary cirrhosis(PBC) and primary sclerosing cholangitis(PSC).Variant forms of these diseases are generally called overlap syndromes,although there has been no standardised definition.Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC.The AIH-PBC overlap syndrome is the most common form,affecting almost 10% of adults with AIH or PBC.Single cases of AIH and autoimmune cholangitis(AMA-negative PBC) overlap syndrome have also been reported.The AIH-PSC overlap syndrome is predominantly found in children,adolescents and young adults with AIH or PSC.Interestingly,transitions from one autoimmune to another have also been reported in a minority of patients,especially transitions from PBC to AIH-PBC overlap syndrome.Overlap syndromes show a progressive course towards liver cirrhosis and liver failure without treatment.Therapy for overlap syndromes is empiric,since controlled trials are not available in these rare disorders.Anticholestatic therapy with ursodeoxycholic acid is usually combined with immunosuppressive therapy with corticosteroids and/or azathioprine in both AIH-PBC and AIH-PSC overlap syndromes.In end-stage disease,liver transplantation is the treatment of choice.  相似文献   

11.
BACKGROUND: Autoimmune hepatitis (AIH) is a chronic inflammatory disease that is successfully treated with prednisone and/or azathioprine immunosuppressive therapy in 70% to 80% of patients. The remaining patients are intolerant or refractory to these standard medications. Budesonide, a synthetic glucocorticoid, undergoes a high degree of first-pass metabolism, reducing its systemic bioavailability, and has a 15-fold greater affinity for the glucocorticoid receptor than prednisolone. Budesonide may be a potentially useful systemic steroid-sparing immunosuppressive agent in the treatment of AIH. OBJECTIVE: To review the Canadian experience using budesonide to treat AIH. METHODS: Patients with AIH currently or previously treated with budesonide were identified through the Canadian Association for the Study of the Liver membership. Data were collected regarding their clinical and treatment history. RESULTS: A total of nine patients were identified. All patients were female, with an average age of 39 years (range 12 to 66 years). The indications for budesonide were adverse side effects of prednisone in two patients, noncompliance with prednisone and azathioprine in one patient and intolerance to azathioprine resulting in prednisone dependence in the remaining six patients. Patients were treated in doses ranging from 9 mg daily to 3 mg every other day for 24 weeks to eight years. Seven of nine patients had a complete response, defined as sustained normalization of the aminotransferase levels. The remaining two patients were classified as nonresponders (less than a 50% reduction in pretreatment aminotransferase levels). CONCLUSIONS: In Canada, budesonide has been successfully used in seven of nine patients with autoimmune hepatitis who were either intolerant to prednisone and azathioprine or prednisone-dependent. No adverse effects were reported with budesonide. Budesonide is potentially a valuable treatment option for AIH patients refractory or intolerant to standard therapy, and is deserving of further study.  相似文献   

12.
Of a total of 2342 patients with type-C chronic hepatitis treated with interferon (IFN) at this hospital, 3 patients developed jaundice during the course of IFN therapy, but all 3 of them exhibited negative conversion of hepatitis C virus (HCV)-RNA following readministration of IFN. All 3 patients were assessed as having "probable autoimmune hepatitis (AIH)" in accordance with the AIH scoring system, indicating association with an "autoimmune phenomenon". Readministration of IFN at a low-dose induced activation of the autoimmune phenomenon, leading to fulminant hepatocellular impairment. As a result, HCV-RNA content dropped dramatically, possibly contributing to the negative conversion of HCV-RNA noted following the readministration of IFN. At present, no adequate therapy has been established for type-C chronic hepatitis with autoimmune manifestations. However, in conclusion, our findings suggested that: IFN should be a frontline regiment: (1). if autoantibody titers are low or patients are rated as having "probable AIH" or lower in accordance with the AIH scoring system, indicating a strong link to chronic hepatitis or (2). if IFN is expected to be efficacious on the basis of genotype of HCV-RNA level; even if there is acute exacerbation of type-C chronic hepatitis, IFN should be re-administered in the HCV-RNA level has dropped subsequently.  相似文献   

13.
Diagnosis of acute onset autoimmune hepatitis (AIH) is the most challenging task because of atypical clinicopathological features. We examined the nature of acute onset AIH consisting of nonsevere, severe, and fulminant AIH based on our published data and other published papers, and propose how to diagnose and treat this intractable hepatitis. We analyzed clinical, biochemical, immunological, radiological, and histological features of acute onset AIH. Thirty percent of fulminant hepatitis was due to AIH and autoimmune acute liver failure (ALF) was not rare. The important characteristic of acute onset AIH is its histological, radiological, and clinical heterogeneity. Sometimes acute onset AIH develops into ALF in a sub-acute clinical course without appropriate diagnosis and treatment, and becomes resistant to immunosuppressive therapy and has poor prognosis. Unenhanced computed tomography (CT) often shows heterogeneous hypoattenuation in autoimmune ALF. The revised original scoring system (1999) performed better in patients with acute onset AIH than the simplified scoring system (2008). Liver regeneration from periportal progenitor cells to mature hepatocytes was impaired in ALF, resulting in resistance to immunosuppressive therapy. Precise histological evaluation (the presence of centrilobular necrosis/collapse) along with the revised original scoring system and CT findings of heterogeneous hypoattenuation after systematic exclusion of other causes 36 plays an important role in the diagnosis. The most important strategy for autoimmune ALF is to diagnose and treat acute onset AIH before its development into ALF. Liver transplantation should be considered before the occurrence of infectious complications in the case of fulminant liver failure.  相似文献   

14.
The pathogenesis of autoimmune hepatitis (AIH) is unclear, but viral infections have been proposed as a potential trigger in patients with genetic predisposition. We report a case of AIH following acute hepatitis A (AHA). A 57-year-old woman presented with fatigue and pitting edema for last 3 months. She had been diagnosed as an AHA 15 months ago based on clinical features, biochemical tests and positive HAV IgM antibody at a local clinic. Her biochemical tests was normalized one month after AHA diagnosis, but the serum levels of aminotransferase started to rise four months after AHA diagnosis. Antinuclear antibody was positive at a titer of 1:40, and anti-smooth muscle antibody was also positive. Hypergammaglobulinemia and liver pathology were typical for AIH. The patients had a score of 17 according to the International Autoimmune Hepatitis Group's system. She was given prednisolone and azathioprine and showed complete response to immunosuppressive therapy. The present case is the first report on AIH triggered by AHA in Korea.  相似文献   

15.
BACKGROUND: Controlled trials have firmly established the need for immunosuppressive therapy in autoimmune hepatitis. However, reports about long-term management and prognosis of the disease are scarce. PATIENTS AND METHODS: We reviewed the charts of 103 consecutive patients with a well-documented long-term course of autoimmune hepatitis who had been carefully managed over a mean observation period of 95 months (12-405 months). RESULTS: Under immunosuppressive therapy 94 patients (91.2%) reached complete remission after a mean treatment duration of 3 +/- 3 months. 28 of the 103 patients (27.2%) were eligible for a trial of treatment withdrawal after a mean treatment duration of 32.2 months (range: 12-81 months). 21 of these patients (75%) had a relapse following treatment withdrawal. 13.6% of patients had intolerance of or severe side effects to azathioprine. There was no increase in tumor risk during a cumulative observation period of 423 patient-years of azathioprine therapy. Corticosteroid side effects occurred mostly during induction therapy, but were usually minor and resolved upon dose reduction. During a cumulative observation period of 842 patient-years no liver related deaths occurred and no patient had to be referred to liver transplantation, even though 30 patients (29.1%) had histological evidence of cirrhosis at presentation. The overall 5- and 10-year survival of patients with autoimmune hepatitis was identical to an age- and sex-matched control population. CONCLUSION: Our study shows that the majority of patients with AIH do achieve a complete remission within 3 months, but require long-term or permanent immunosuppressive therapy that is usually well tolerated. Long-term survival in well-managed patients is excellent.  相似文献   

16.
Roberts EA 《Liver international》2011,31(10):1424-1431
Autoimmune hepatitis (AIH) is an important entity within the broad spectrum of autoimmune hepatobiliary disease comprised of AIH, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). Since the 1960s, AIH has been investigated with extensive clinical research aimed at effective therapeutic intervention. It was one of the first liver diseases where treatment was demonstrated to prolong survival. AIH occurs in children, as well as in adults. Its clinical manifestations in children may differ from classic adult AIH. These differences have elucidated certain aspects of AIH and hepatobiliary disease in general. There are two major patterns of AIH: type 1, with anti-smooth muscle antibodies and type 2, with anti-liver/kidney microsomal antibodies. The second type of AIH was first identified in children and is more common in younger patients. AIH often presents as acute disease in children and also in adults: the nomenclature has dropped the allusion to chronicity. Some children who have sclerosing cholangitis present with clinical disease closely resembling AIH; this AIH-like PSC, termed autoimmune sclerosing cholangitis (ASC), is also found in adults. Children with AIH may have identifiable monogenic disorders of immune regulation such as autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). Like adults with AIH, children with AIH usually respond very favourably to immunosuppressive treatment with corticosteroids ± azathioprine. True cures seem to be rare, although many children achieve a stable remission. Nonetheless children with AIH may develop cirrhosis and some require liver transplantation. Early diagnosis and improved treatment strategies may further improve the outlook for children with AIH.  相似文献   

17.
OBJECTIVE: In published studies there is a lack of data about the risks, management and how women with autoimmune hepatitis (AIH) decide on and are advised about pregnancy. The aim of this study was to investigate how women with AIH consider pregnancies, are advised and pharmacologically treated, as well as the outcome. MATERIAL AND METHODS: A questionnaire was mailed to 128 women with AIH diagnosed during their fertile period and data from the Swedish National Birth Register was also used for matched controls. RESULTS: There was an 83% response rate to the questionnaires. Sixty-three pregnancies were reported by 35 women. 48% did not consult their doctors before getting pregnant. More than half of the women reduced or stopped the immune suppression during pregnancy or breastfeeding. Some women were advised to abstain from pregnancy or even to have an abortion. Caesarean sections were performed more frequently in the AIH group (16% compared with 6.5% in the control group p<0.01).There were no significant differences in the number of stillborn infants or infants with malformations. However, 30% of the patients experienced flare-up after delivery. CONCLUSIONS: In general, the outcome of pregnancy in women with AIH seems to be good. Current pharmacological treatment appears to be safe, including azathioprine during pregnancy and lactation. After delivery an active preparedness to increase pharmacotherapy should be considered.  相似文献   

18.
BACKGROUND: Significant diversity in disease severity has been identified for autoimmune disorders among different ethnic groups. Current knowledge of both the natural history and management of autoimmune hepatitis (AIH) has been derived from European or Japanese patients, and there is limited information about the disease in patients from other ethnic groups. AIMS: To assess the clinical, histological and immunological features of AIH in patients from Somalia and to determine their response to therapy. METHODS: Retrospective review of a cohort of young Somalian men with atypical AIH compared with a control group of European patients. RESULTS: The six Somalian men were younger at presentation (median age 37 (range 24-59) years) than the seven female and three male European controls (55 (34-54) years, P = 0.06). The Somalians had slightly more severe disease at presentation-median modified Ishak stage of 2.5 compared with 2 in Europeans (P = 0.61) and four (66%) had features of cholestasis compared with only one (10%) European patient (P = 0.04). Therapy with prednisolone and azathioprine was completely effective for eight of 10 Europeans but only one of seven Somalians (P = 0.04). Analysis of human leucocyte antigen types revealed differences between the Somalian and European patients, although these differences did not reach statistical significance. CONCLUSIONS: Somalian men with AIH present with cholestatic features and respond poorly to standard immunosuppressive regimes.  相似文献   

19.
Autoimmune hepatitis (AIH) is a common cause of acute and chronic hepatitis in childhood. Once the diagnosis is established, treatment with corticosteroid or corticosteroid and azathioprine is indicated. Most children with AIH respond to such therapy and experience remission from active disease. Eliminating drug therapy while maintaining remission is the ultimate goal of therapy. The optimal duration of therapy before drug elimination is unclear. Relapse rate is inversely related to therapy duration before drug withdrawal; thus, discontinuing immunosuppressive treatment is considered only after at least 1 to 2 years of complete remission. When applying a slow and systematic approach, many children with AIH can successfully be weaned off immunosuppression completely. Even patients presenting in acute liver failure may avoid liver transplantation with early medical therapy. In about 10% of patients, treatment fails, requiring alternative therapies and/or liver transplantation as liver disease progresses. Less than 10% of children with autoimmune hepatitis die during 10 years of follow-up.  相似文献   

20.
目的比较自身免疫性肝炎(AIH)与系统性红斑狼疮(SLE)性肝损伤在临床表现、血清学检查及病理学上的异同点。方法回顾性分析44例自身免疫性肝炎与26例狼疮性肝损伤的临床资料、血清学检查及病理学特点。结果AIH以女性多发,发病高峰为(47.32±13.61)岁,临床表现以消化系统为主,以ALT、AST升高,高球蛋白血症及IgG升高为主要特征,存在多种自身抗体,伴发其他自身免疫性疾病比率高,病理有特征性改变,对激素及免疫抑制剂有效,病情呈波动性变化。SLE以女性多发,发病高峰(33.46±14.32)岁,临床表现多样化,肝损伤为初发患者一过性ALT、AST升高,有明显低补体血症,存在多种自身抗体,并发其他自身免疫性疾病比率低,病理无特征性改变,激素治疗有效。结论AIH、SLE均以女性多发,有过敏史的比率高于正常人群,激素治疗有效;AIH肝损伤重,呈波动性变化,易伴发其他自身免疫性疾病,SLE肝损伤轻,多为一过性,可累及全身多个系统,二者为相互独立的疾病。  相似文献   

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