Clinical and histological features of autoantibody‐negative autoimmune hepatitis in <scp>C</scp>hinese patients: A single center experience

Wang, Qi Xia; Jiang, Wei; Miao, Qi; Xiao, Xiao; Zhang, Hai Yan; Huang, Shan; Shen, Lei; Hua, Jing; Li, Hai; Shen, Li; Qiu, De Kai; Ma, Xiong

doi:10.1111/1751-2980.12022

Cited by 17 publications

(19 citation statements)

References 16 publications

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“…These patients can be given glucocorticoid alone or combination treatment. Their responses to immunosuppressive treatments are usually similar to patients with typical AIH …”

Section: Treatment For Aih Of Special Clinical Phenotypesmentioning

confidence: 93%

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Chinese consensus on the diagnosis and management of autoimmune hepatitis (2015)

2017

J of Digest Diseases

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“…These patients can be given glucocorticoid alone or combination treatment. Their responses to immunosuppressive treatments are usually similar to patients with typical AIH …”

Section: Treatment For Aih Of Special Clinical Phenotypesmentioning

confidence: 93%

“…Their responses to immunosuppressive treatments are usually similar to patients with typical AIH. 42…”

Section: Autoantibody-negative Aihmentioning

confidence: 99%

Chinese consensus on the diagnosis and management of autoimmune hepatitis (2015)

2017

J of Digest Diseases

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“…For example, AIH patients without autoantibodies may not achieve "probable" or "definite" scores and require empiric immunosuppression to establish a diagnosis of AIH [52,58]. In a retrospective Chinese study, 17 (10.2%) of 167 patients were autoantibody negative [59]. Using RDC, 6 were "definite" and 11 "probable" scores for AIH.…”

Section: Diagnostic Criteriamentioning

confidence: 99%

Autoimmune hepatitis

Sahebjam

Vierling

2015

Front. Med.

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Autoimmune hepatitis is a chronic liver disease putatively caused by loss of tolerance to hepatocyte-specific autoantigens. It is characterized by female predilection, elevated aminotransferase levels, autoantibodies, increased γ-globulin or IgG levels and biopsy evidence of interface hepatitis. It is currently divided into types 1 and 2, based on expression of autoantibodies. Autoantigenic epitopes have been identified only for the less frequent type 2. Although autoimmune hepatitis occurs in childhood, this review focuses on disease in adults. In the absence of pathognomonic biomarkers, diagnosis requires consideration of clinical, biochemical, serological and histological features, which have been codified into validated diagnostic scoring systems. Since many features also occur in other chronic liver diseases, these scoring systems aid evaluation of the differential diagnosis. New practice guidelines have redefined criteria for remission to include complete biochemical and histological normalization on immunosuppressive therapy. Immunosuppression is most often successful using prednisone or prednisolone and azathioprine; however, the combination of budesonide and azathioprine for non-cirrhotic patients offers distinct advantages. Patients failing standard immunosuppression are candidates for alternative immunosuppressive regimens, yet none of the options has been studied in a randomized, controlled trial. Overlap syndromes with either primary sclerosing cholangitis or primary biliary cirrhosis occur in a minority. Liver transplantation represents a life-saving option for patients presenting with acute liver failure, severely decompensated cirrhosis or hepatocellular carcinoma. Transplant recipients are at risk for recurrent autoimmune hepatitis in the allograft, and de novo disease may occur in patients transplanted for other indications. Patients transplanted for AIH are also at risk for recurrent or de novo inflammatory bowel disease. Progress in our understanding of the immunopathogenesis should lead to identification of specific diagnostic and prognostic biomarkers and new therapeutic strategies.

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“…Antisoluble liver antigen/liver pancreas antigen (anti-SLP/LP) found both in type 1 and type 2 AIH may be helpful in diagnosing AIH without classical autoantibodies. Circulating autoantibodies are absent in about 10% AIH patients [7]. Patients with acute presentation (as in our case) autoantibodies and elevated IgG levels may be lacking and that autoantibodies may become positive only weeks or months later.…”

Section: Discussionmentioning

confidence: 77%

Seronegative Autoimmune Hepatitis A Clinically Challenging Difficult Diagnosis

Sherigar

Yavgeniy

Guss

et al. 2017

Case Reports in Medicine

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Autoimmune hepatitis (AIH) is a complex liver disease of unknown cause which results in immune-mediated liver injury with varied clinical presentations. Seronegative AIH follows a similar course to autoantibody-positive disease and diagnosis may be challenging. There are no single serologic tests of sufficient diagnostic specificity, and delay in appropriate treatment may lead to progression of the liver disease and liver failure. The revised conventional diagnostic criteria (RDC) scoring for AIH is complex and not routinely used in the clinical practice. The more recent simplified diagnostic criteria (SDC) scoring proposed by International Autoimmune Hepatitis Group in 2008 has wider application in routine practice facilitating the diagnosis of AIH with a specificity and sensitivity of ~90%. In this report, we describe a case of seronegative autoimmune hepatitis diagnosed using RDC. SDC score calculated in our case was 4 and was not diagnostic for AIH. We subsequently used the complex revised diagnostic criteria for definitive diagnosis. Some of the patients previously diagnosed as cryptogenic active hepatitis of unknown etiology probably had an unrecognized diagnosis of seronegative autoimmune hepatitis. SDC scoring may not be applicable in patients with seronegative autoimmune hepatitis. These patients should be reassessed by using RDC.

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Clinical and histological features of autoantibody‐negative autoimmune hepatitis in Chinese patients: A single center experience

Abstract: Autoantibody-negative AIH is not uncommon in Chinese AIH patients, and has a good response to immunosuppressive treatment comparable to classical patients.

Cited by 17 publications

References 16 publications

Chinese consensus on the diagnosis and management of autoimmune hepatitis (2015)

Chinese consensus on the diagnosis and management of autoimmune hepatitis (2015)

Autoimmune hepatitis

Seronegative Autoimmune Hepatitis A Clinically Challenging Difficult Diagnosis

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