丙肝患者HCV-RNA阳性与自免肝抗体相关性研究

目的探讨丙肝患者HCV-RNA阳性感染与自免肝抗体产生的相关性。方法对330例丙肝抗体Ig G(+)患者进行HCV-RNA检测及相关的自免肝抗体检测。结果 330例肝炎患者中,有182例HCV-RNA阳性,阳性率为(55.1%),148例患者HCV-RNA阴性,阴性率为(44.8%)。将其分为两组,第一组:丙肝抗体Ig G(+),HCV-RNA均为阳性,测得AMA-M2阳性13例(7.1%),SLA阳性23例(12.6%),LKM-1阳性40例(22%),LC-1阳性11例(6%);第二组:丙肝抗体Ig G(+),HCV-RNA阴性,测得AMA-M2阳性4例(2.7%),SLA阳性6例(4.1%),LKM-1阳性19例(12.8%),LC-1阳性4例(2.7%)。两组自免肝抗体阳性率差异有统计学意义(χ2=93.4,P<0.05)。结论丙肝患者自免肝抗体检测阳性率与丙肝患者HCV-RNA阳性呈正相关。

自免肝抗原谱的血清学检测对于PBC与AIH诊断的临床意义

目的:原发性胆汁肝硬化(PBC)与抗线粒体抗体、抗核点抗体、抗早幼粒细胞白血病蛋白抗体等多种抗体相关.自免肝抗原谱中的这些抗体是PBC患者血清学检测的标志,也有助于鉴别原发性胆汁性肝硬化(PBC)和自身免疫性肝炎(AIH).结果:应用欧蒙印迹法对499例不同类型血清进行检测,其中150例PBC患者血清中有93%的标本至少可检测到AMA-M2、M2-3E、gp210、SP100、PML中的一种抗体,相对于病毒性肝炎组和本院职工健康体检组其特异性为99%.LKM-1、LC-1、SLA/LP抗体为AIH的血清学标志,其特异性几乎为100%.结论:自免肝抗原谱中检测AMA-M2、M2-3E、gp210、SP100、PML、LKM-1、LC-1、SLA/LP和R0-52九种自身抗体对于PBC的诊断及PBC和AIH的鉴别诊断提供重要的实验依据.

探讨早期免疫抑制辅助熊去氧胆酸治疗原发性胆汁性肝硬化合并自免肝的疗效

目的评价早期免疫抑制辅助熊去氧胆酸(UDCA)治疗原发性胆汁性肝硬化(PBC)合并自身免疫性肝病(AIH)的疗效。方法研究时间以2016年1月—2019年1月为准,回顾52例PBC合并AIH患者的资料。其中对照组单纯应用UDCA治疗,研究组加用早期免疫抑制剂辅助治疗。对比两组疗效,治疗前后患者肝功能、免疫学各项变化情况。结果研究组治疗有效率92.31%显著高于对照组69.23%(χ^2=4.457,P<0.05)。治疗前,研究组AST、ALT、ALP、TBIL、IgG、IgM水平分别为(86.47±12.08)U/L、(32.28±4.52)U/L、(192.85±23.15)U/L、(77.23±12.41)μmol/L、(20.13±3.21)g/L、(5.20±1.02)g/L,与对照组比较,差异无统计学意义(P>0.05);治疗后较治疗前,两组均有显著改善,但研究组(80.14±13.23)U/L、(26.29±5.82)U/L、(92.67±10.43)U/L、(20.54±4.03)μmol/L、(12.30±3.02)g/L、(3.39±0.74)g/L显著优于对照组,差异有统计学意义(P<0.05)。结论熊去氧胆酸治疗原发性胆汁性肝硬化合并自免肝有一定的效果,但早期应用免疫抑制剂更能有效调节免疫,改善肝功能,值得推广。

强的松联用常规保肝药治疗干燥综合征合并自免肝的疗效

目的观察强的松联用常规保肝药物在干燥综合征合并自免肝疗效。方法将我院2014年5月～2016年5月收治的60例干燥综合征合并自免肝患者随机分为对照组（常规保肝药物治疗）及观察组（强的松联合常规保肝药物治疗）各30例，比较两组临床疗效。结果观察组血生化指标数值优于对照组，差异有统计学意义（P〈0．05）。结论强的松联用常规保肝药物治疗干燥综合征合并自免肝疗效确切。

探讨早期免疫抑制辅助熊去氧胆酸治疗原发性胆汁性肝硬化合并自免肝的疗效

目的:探讨早期免疫抑制辅助熊去氧胆酸治疗原发性胆汁性肝硬化合并自免肝的疗效。方法:选取甘肃省天水市第一人民医院消化内科2019年1月至2022年1月收治的原发性胆汁性肝硬化合并自免肝患者92例作为实验对象,运用随机数字表法予以平均分组,对照组46例患者运用熊去氧胆酸治疗方式,观察组46例患者在此基础上运用早期免疫抑制辅助治疗,着重就其疗效指标予以观察,深入探讨其应用效果。结果:用药前两组患者的血清透明质酸、Ⅲ型前胶原肽、层粘连蛋白、Ⅳ型胶原蛋白4项血清纤维化指标值接近,谷草转氨酶、谷丙转氨酶、碱性磷酸酶、谷氨酰转肽酶4项肝功能指标值接近(P>0.05),对比差异无统计学意义;用药半年后,观察组患者的血清透明质酸、Ⅲ型前胶原肽、层粘连蛋白、Ⅳ型胶原蛋白4项血清纤维化指标值均低于对照组患者,谷草转氨酶、谷丙转氨酶、碱性磷酸酶、谷氨酰转肽酶4项肝功能指标值均低于对照组患者,观察组患者的治疗总有效率89.13%高于对照组患者的治疗总有效率65.22%,对比差异具统计学意义(P<0.05)。结论:早期免疫抑制辅助熊去氧胆酸治疗原发性胆汁性肝硬化合并自免肝的疗效显著,能够抑制患者机体自身免疫系统,从而改善其肝功能、血清纤维化指标,减轻肝胆系统的损伤,达到有效治疗的目的。

EB病毒感染和自身免疫病的相关性研究

EB病毒是一种感染人类B淋巴细胞的疱疹病毒。现综述EB病毒与多种自身免疫疾病,如系统性红斑狼疮、多发性硬化症、类风湿性关节炎、自身免疫甲状腺病;自身免疫性肝疾病之间的相关性,并尝试用Pender的假说,解释这一现象。

Autoimmune liver serology:Current diagnostic and clinical challenges

Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseas-es(AiLD),namely autoimmune hepatitis types 1 and 2(AIH-1 and 2),primary biliary cirrhosis(PBC),and the sclerosing cholangitis variants in adults and children.AIH-1 is specified by anti-nuclear antibody(ANA) and smooth muscle antibody(SMA).AIH-2 is specified by antibody to liver kidney microsomal antigen type-1(anti-LKM1) and anti-liver cytosol type 1(anti-LC1).SMA,ANA and anti-LKM antibodies can be present in de-novo AIH following liver transplantation.PBC is specified by antimitochondrial antibodies(AMA) react-ing with enzymes of the 2-oxo-acid dehydrogenase complexes(chiefly pyruvate dehydrogenase complex E2 subunit) and disease-specific ANA mainly react-ing with nuclear pore gp210 and nuclear body sp100.Sclerosing cholangitis presents as at least two variants,first the classical primary sclerosing cholangitis(PSC) mostly affecting adult men wherein the only(and non-specific) reactivity is an atypical perinuclear antineutro-phil cytoplasmic antibody(p-ANCA),also termed peri-nuclear anti-neutrophil nuclear antibodies(p-ANNA) and second the childhood disease called autoimmune sclerosing cholangitis(ASC) with serological features resembling those of type 1 AIH.Liver diagnostic serol-ogy is a fast-expanding area of investigation as new purified and recombinant autoantigens,and automatedtechnologies such as ELISAs and bead assays,become available to complement(or even compete with) tradi-tional immunofluorescence procedures.We survey for the first time global trends in quality assurance impact-ing as it does on(1) manufacturers/purveyors of kits and reagents,(2) diagnostic service laboratories that fulfill clinicians' requirements,and(3) the end-user,the physician providing patient care,who must properly interpret test results in the overall clinical context.

Features of hepatocellular carcinoma in cases with autoimmune hepatitis and primary biliary cirrhosis

AIM: To characterize the clinical features of hepatocellular carcinoma (HCC) associated with autoimmune liver disease, we critically evaluated the literature on HCC associated with autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). METHODS: A systematic review of the literature was conducted using the Japana Centra Revuo Medicina database which produced 38 cases of HCC with AIH (AIH-series) and 50 cases of HCC with PBC (PBC-series). We compared the clinical features of these two sets of patients with the general Japanese HCC population. RESULTS: On average, HCC was more common in men than in women with AIH or PBC. While many patients underwent chemolipiodolization (CL) or transcatheter arterial embolization (TAE) (AIH-series: P = 0.048 (vs operation), P = 0.018 (vs RFA, PEIT); PBC-series: P = 0.027 (vs RFA, PEIT), others refused therapeutic interventions [AIH-series: P = 0.038 (vs RFA, PEIT); PBC-series: P = 0.003 (vs RFA, PEIT)].Liver failure was the primary cause of death among patients in this study, followed by tumor rupture. The survival interval between diagnosis and death was fairly short, averaging 14 ± 12 mo in AIH patients and 8.4 ± 14 mo in PBC patients. CONCLUSION: We demonstrated common clinical features among Japanese cases of HCC arising from AIH and PBC.

Clinical features and management of autoimmune hepatitis

Autoimmune hepatitis(AIH) is a chronic hepatitis of unknown etiology which can progress to cirrhosis.Its clinical manifestations are highly variable and some-times follow a fluctuating course.Diagnosis is based on characteristic histologic,clinical,biochemical and sero-logical findings.Anti-inflammatory/immunosuppressive treatment frequently induces remission but long-term maintenance therapy is often required.Liver transplan-tation is generally successful in patients with decompen-sated cirrhosis unresponsive to or intolerant of medical therapy.

Aetiopathogenesis of autoimmune hepatitis

The histological hallmark of autoimmune hepatitis（AIH） is a dense portal mononuclear cell infiltrate that invades the surrounding parenchyma and comprises T and B lymphocytes,macrophages,and plasma cells.An unknown but powerful stimulus must be promoting the formation of this massive inflammatory cellular reaction that is likely to initiate and perpetuate liver damage.An autoimmune attack can follow different pathways to inflict damage on hepatocytes.Liver damage is likely to be orchestrated by CD4^＋ T lymphocytes recognizing an autoantigenic liver peptide.To trigger an autoimmune response,the peptide must be embraced by an HLA class Ⅱ molecule and presented to naive CD4^＋ T helper（Th0） cells by professional antigen presenting cells,with the co-stimulation of ligand-ligand fostering interaction between the two cells.Th0 cells become activated,differentiate into functional phenotypes according to the cytokines prevailing in the microenvironment and the nature of the antigen,and initiate a cascade of immune reactions determined by the cytokines produced by the activated T cells.Th1 cells,arising in the presence of the macrophage-derived interleukin（IL） -12,secrete mainly IL-2 and interferon-gamma（IFN-γ）,which activate macrophages,enhance expression of HLA classⅠ（increasing liver cell vulnerability to a CD8^＋ T cell cytotoxic attack）,and induce expression of HLA class Ⅱ molecules on hepatocytes.Th2 cells,which differentiate from Th0 if the microenvironment is rich in IL-4,produce mainly IL-4,IL-10,and IL-13 which favour autoantibody production by B lymphocytes.Physiologically,Th1 and Th2 antagonize each other.Th17 cells,a recently described population,arise in the presence of transforming growth factor beta（TGF-β） and IL-6 and appear to have an important effector role in inflammation and autoimmunity.Theprocess of autoantigen recognition is strictly controlled by regulatory mechanisms,such as those exerted by CD4^＋CD25^＋ regulatory T cells,which derive from Th0 in the presence of TGF-β,but in the absence of IL-6.If regulatory mechanisms fail,the autoimmune attack is perpetuated.Over the past three decades different aspects of the above pathogenic scenario have been investigated.In particular,a defect in immunoregulation affecting CD4^＋CD25^＋ regulatory T cells（T-regs） has been demonstrated in AIH,particularly at diagnosis or during relapse.Advances in the study of autoreactive T cells have occurred mostly in AIH type 2,since the knowledge that CYP2D6 is the main autoantigen has enabled the characterization of both CD4 and CD8 T cells targeting this cytochrome.CD4 T cells from patients with type 2 AIH positive for the predisposing HLA allele DRB10701 recognize seven regions of CYP2D6,five of which are also recognized by CD8 T cells.High numbers of IFN-γ producing CD4 T cells and CD8 T cells are associated with biochemical evidence of liver damage,suggesting a combined cellular immune attack.

Budesonide induces complete remission in autoimmune hepatitis

AIM： Prednisone and azathioprine represent the standard treatment for autoimmune hepatitis （AIH）. However, only 65% of the patients enter complete histological remission. Recently, budesonide （BUD） was reported to be a promising alternative. In this study we assessed the efficacy and safety of BUD in AIH. METHODS： Eighteen patients （12 women, 6 men; mean age 45.4±21 years） with AIH were treated with BUD （Budenofalk） 3 mg thrice daily and followed up for at least 24 wk. Seven patients also had features of primary biliary cirrhosis （n = 5） or primary sclerosing cholangitis （n = 2）. Advanced liver fibrosis or cirrhosis was present in RESULTS： Fifteen （83%） patients had a complete clinical and biochemical remission. Ten patients, including five with acute hepatitis, were given BUD as first-line therapy, of which seven enter remission. Three patients, two with liver cirrhosis, did not improve. All patients with second-line therapy experienced long-term remission. A histological remission was also seen in three patients. Clinically relevant BUD-induced side effects were recorded only in patients with liver cirrhosis （n = 4）. CONCLUSION： BUD is effective in remission induction in the majority of our patients with AIH. Side effects and treatment failure was mainly observed in patients with liver cirrhosis.

Inhibition of p38 mitogen-activated protein kinase attenuates experimental autoimmune hepatitis: Involvement of nuclear factor kappa B

To investigate the role of p38 mitogen-activated protein kinase （p38MAPK） in murine experimental autoimmune hepatitis （EAH）.METHODS： To induce EAH, the syngeneic S-100 antigen emulsified in complete Freud＇s adjuvant was injected intraperitoneally into adult male C57BI/6 mice. Liver injury was assessed by serum ALT and liver histology. The expression and activity of p38 MAPK were measured by Western blot and kinase activity assays. In addition, DNA binding activities of nuclear factor kappa B （NF-KB） were analyzed by electrophoretic mobility shift assay. The effects of SB203580, a specific p38 MAPK inhibitor, on liver injuries and expression of proinflammatory cytokines （interferon-y, IL-12, IL-1β and TNF-α） were observed.RESULTS： The activity of p38 MAPK and NF-~：B was increased and reached its peak 14 or 21 d after the first syngeneic S-100 administration. Inhibition of p38 MAPK activation by SB203580 decreased the activation of NF-~：B and the expression of proinflammatory cytokines. Moreover, hepatic injuries were improved significantly after SB203580 administration.

Overlap syndromes among autoimmune liver diseases

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.

Historical reflections on autoimmune hepatitis

Autoimmune hepatitis(AIH),initially known as chronic active or active chronic hepatitis(and by various other names),first came under clinical notice in the late 1940s.However,quite likely,chronic active hepatitis(CAH) had been observed prior to this and was attributed to a persistently destructive virus infection of the liver.An earlier(and controversial) designation in 1956 as lupoid hepatitis was derived from associated L.E.cell test positivity and emphasized accompanying multisystem features and immunological aberrations.Young women featured prominently in early descriptions of CAH.AIH was first applied in 1965 as a descriptive term.Disease-characteristic autoantibodies were defined from the early 1960s,notably antinuclear antibody(ANA),smooth muscle antibody(SMA) and liver-kidney microsomal(LKM) antibody.These are still widely used diagnostically but their relationship to pathogenesis is still not evident.A liver and disease specific autoantigen has long been searched for but unsuccessfully.Prolonged immunosuppressive therapy with predisolone and azathioprine in the 1960s proved beneficial and remains standard therapy today.AIH like many other autoimmune diseases is associated with particular HLA alleles especially with the "ancestral" B8,DR3 haplotype,and also with DR4.Looking forwards,AIH is one of the several enigmatic autoimmune diseases that,despite being(relatively) organ specific,are marked by autoimmune reactivities with non-organ-specific autoantigens.New paradigms are needed to explain the occurrence,expressions and pathogenesis of such diseases.

Hepatitis A vaccine associated with autoimmune hepatitis

To describe a case of probable relapsing autoimmune hepatitis associated with vaccination against hepatitis A virus （HAV）. A case report and review of literature were written concerning autoimmune hepatitis in association with hepatitis A and other hepatotropic viruses. Soon a^er the administration of formalin-inactivated hepatitis A vaccine, a man who had recently recovered from an uncharacterized but self-limiting hepatitic illness, experienced a severe deterioration （AST 1687 U/L, INR 1.4）. Anti-nuclear antibodies were detectable, and liver biopsy was compatible with autoimmune hepatitis. The observation supports the role of HAV as a trigger of autoimmune hepatitis. Studies in helper T-cell activity and antibody expression against hepatic proteins in the context of hepatitis A infection are summarized, and the concept of molecular mimicry with regard to other forms of viral hepatitis and autoimmunity is briefly explored.

Factoring the intestinal microbiome into the pathogenesis of autoimmune hepatitis

The intestinal microbiome is a reservoir of microbial antigens and activated immune cells. The aims of this review were to describe the role of the intestinal microbiome in generating innate and adaptive immune responses, indicate how these responses contribute to the development of systemic immune-mediated diseases, and encourage investigations that improve the understanding and management of autoimmune hepatitis. Alterations in the composition of the intestinal microflora(dysbiosis) can disrupt intestinal and systemic immune tolerances for commensal bacteria. Toll-like receptors within the intestine can recognize microbe-associated molecular patterns and shape subsets of T helper lymphocytes that may cross-react with host antigens(molecular mimicry). Activated gutderived lymphocytes can migrate to lymph nodes, and gut-derived microbial antigens can translocate to extra-intestinal sites. Inflammasomes can form within hepatocytes and hepatic stellate cells, and they can drive the pro-inflammatory, immune-mediated, and fibrotic responses. Diet, designer probiotics, vitamin supplements, re-colonization methods, antibiotics, drugs that decrease intestinal permeability, and molecular interventions that block signaling pathways may emerge as adjunctive regimens that complement conventional immunosuppressive management. In conclusion, investigations of the intestinal microbiome are warranted in autoimmune hepatitis and promise to clarify pathogenic mechanisms and suggest alternative management strategies.

Fulminant hepatic failure in a case of autoimmune hepatitis in hepatitis C during peg-interferon-alpha 2b plus ribavirin treatment

A 27-year-old Caucasian female with hepatitis C virus (HCV) infection treated with interferon (IFN) who developed severe autoimmune hepatitis (AIH) is described. The infecting viral strain was of genotype Ib and the pre-treatment HCV viral load was at a high level. The patient was treated with pegylated IFN-alpha 2b and ribavirin,and her HCV-RNA became negative at wk 12,but after that she developed fulminant hepatic failure. The patient recovered after steroid pulse therapy consisting of methylprednisolone 1000 mg/d for three days which was administered twice. A needle liver biopsy revealed the typical pathological findings of AIH.

IgG4-related sclerosing disease

Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prosrate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AlP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4- related sclerosing diseases. This disease includes AlP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AlP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.

Hepatocellular carcinoma in patients with autoimmune hepatitis

AIM: To evaluate and confirm the low incidence of hepatocellular carcinoma (HCC) in patients with autoimmune hepatitis (AIH). At present only very few cases of HCC in patients with AIH and definite exclusion of chronic viral hepatitis have been published, suggesting that HCC due to AIH is rare. METHODS: In order to further investigate the incidence of HCC in patients with AIH, we reviewed our large cohort of 278 patients with AIH. RESULTS: Eighty-nine patients (32%) were diagnosed with liver cirrhosis, a preneoplastic condition for HCC. We studied a total of 431 patient years of cirrhosis in these patients, an average 4.8 years per patient. During this period none of the patients of our own study cohort developed HCC. However, three patients with HCC due to AIH associated liver cirrhosis were referred to our department for further treatment of HCC. In all three patients chronic viral hepatitis was excluded. CONCLUSION: We conclude that HCC may under rare circumstances develop due to chronic AIH dependent liver cirrhosis. Compared to other causes of liver cirrhosis such as chronic viral hepatitis, alcohol, or hemochromatosis, the incidence of HCC is significantly lower. Pathophysiological differences between AIH and chronic viral hepatitis responsible for differences in the incidence of HCC are yet to be further characterized and may lead to new therapeutic concepts in prevention and treatment of liver cancer.

Usefulness of liver infiltrating CD86-positive mononuclear cells for diagnosis of autoimmune hepatitis

AIM： Although the pathogenic mechanism underlying autoimmune hepatitis （AIH） remains unclear, the immune system is thought to be critical for the progression of the disease. Cellular immune responses may be linked to the hepatocellular damage in AIH. Recently, much attention has been focused on the critical functions of costimulatory molecules expressed on mononuclear cells in the generation of effective T cell-mediated immune responses. Analysis of costimulatory molecule expressed on mononuclear cells from the patients with AIH may give us insight into the pathogenic mechanism of hepatocellular damage in AIH. METHODS： Peripheral blood mononuclear cells （PBMC） were taken from the patients with AIH （34 cases） and healthy controls （25 cases）. Uver infiltrating mononuclear cells （LIMCs） were taken from the patients with AIH （18 cases）, the patient with chronic hepatitis C （CH-C） （13 cases） and the patients with fatty liver （2 cases）. Using flow cytometry, the cells were analyzed for the expression of costimulatory molecules, such as CD80, CD86, and CD152 （CTLA-4）. The results were compared with clinical data such as the level of gammaglobulin, histological grade, presence or absence of corticosteroids administration and the response to corticosteroids. RESULTS： The levels of CD80＋, CD86＋ and CD152＋ PBMC were significantly reduced in the patients with AIH as compared with healthy controls. By contrast, those cells were significantly higher in LIMC than in PBMC of the patients with AIH. Especially, the level of CD86＋ LIMC showed a marked increase irrespective of the degree of disease activity in the patients with AIH,although CD86＋ cells were rarely present in PBMC. The levels of CD86＋ cells were present in significantly higher frequency in patients with AIH than in the patients with CH-C. Furthermore, the patients with AIH with high levels of CD86＋ LIMC showed good responses to corticosteroids, whereas 2 cases of AIH with low levels of CD86＋ LIMC did not respond well. CONCLUSION： These results suggest that LIMC overexpressing costimulatory molecules such as CD80 and CD86 appears to play a role in the pathogenesis of AIH. Especially, CD86 molecule expressed on the LIMC may be useful for the diagnosis of AIH and for the prediction of the therapeutic effects of corticosteroids on AIH.