Diagnostic Criteria for Autoimmune Hepatitis: Scores and More

The diagnosis of autoimmune hepatitis is a clinical diagnosis that combines the patient's history, clinical examination, laboratory and serological markers and the results of a liver biopsy. As the clinical spectrum of autoimmune hepatitis is very wide, making the diagnosis can sometimes be difficult, especially in non-expert hands. Diagnostic scores can help in making the diagnosis, and the simplified diagnostic score of the International Autoimmune Hepatitis Group has a sensitivity and specificity of around 90% in the different populations that have been studied. Therefore, it can be very helpful in everyday use, but nonetheless for some patients the score is not good enough. Limitations are patients with very acute presentations as well as atypical cases. In such cases, a trial of monotherapy with steroids and quick tapering of the steroids is recommended. If the disease responds well to treatment, but recurs after tapering the steroids, the diagnosis of autoimmune hepatitis is confirmed. In addition to its clinical use, diagnostic scores can also be helpful in defining the unified criteria in order to make scientific studies comparable.

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Seronegative Autoimmune Hepatitis A Clinically Challenging Difficult Diagnosis

Case Reports in Medicine ◽

10.1155/2017/3516234 ◽

2017 ◽

Vol 2017 ◽

pp. 1-3 ◽

Cited By ~ 3

Author(s):

Jagannath M. Sherigar ◽

Arefiev Yavgeniy ◽

Debra Guss ◽

Nhu Ngo ◽

Smruti Mohanty

Keyword(s):

Liver Disease ◽

Autoimmune Hepatitis ◽

Diagnostic Criteria ◽

Unknown Etiology ◽

Routine Practice ◽

Specificity And Sensitivity ◽

Appropriate Treatment ◽

Immune Mediated ◽

Clinical Presentations ◽

International Autoimmune Hepatitis Group

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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Diagnostic criteria for autoimmune hepatitis

Best Practice & Research Clinical Gastroenterology ◽

10.1016/j.bpg.2011.10.004 ◽

2011 ◽

Vol 25 (6) ◽

pp. 665-671 ◽

Cited By ~ 17

Author(s):

Ansgar W. Lohse ◽

Christiane Wiegard

Keyword(s):

Autoimmune Hepatitis ◽

Diagnostic Criteria

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Impact of new diagnostic criteria for diabetes on different populations

Diabetes Care ◽

10.2337/diacare.22.5.762 ◽

1999 ◽

Vol 22 (5) ◽

pp. 762-766 ◽

Cited By ~ 73

Author(s):

J. E. Shaw ◽

M. de Courten ◽

E. J. Boyko ◽

P. Z. Zimmet

Keyword(s):

Diagnostic Criteria ◽

Different Populations

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Leveraging Social Networking Sites for an Autoimmune Hepatitis Genetic Repository: Pilot Study to Evaluate Feasibility (Preprint)

10.2196/preprints.7683 ◽

2017 ◽

Author(s):

Megan Comerford ◽

Rachel Fogel ◽

James Robert Bailey ◽

Prianka Chilukuri ◽

Naga Chalasani ◽

...

Keyword(s):

Pilot Study ◽

Liver Biopsy ◽

Social Networking ◽

Autoimmune Hepatitis ◽

Rare Disease ◽

Social Networking Sites ◽

Participant Recruitment ◽

Study Materials ◽

Immunosuppressant Medications ◽

Oriented Research

BACKGROUND Conventional approaches to participant recruitment are often inadequate in rare disease investigation. Social networking sites such as Facebook may provide a vehicle to circumvent common research limitations and pitfalls. We report our preliminary experience with Facebook-based methodology for participant recruitment and participation into an ongoing study of autoimmune hepatitis (AIH). OBJECTIVE The goal of our research was to conduct a pilot study to assess whether a Facebook-based methodology is capable of recruiting geographically widespread participants into AIH patient-oriented research and obtaining quality phenotypic data. METHODS We established a Facebook community, the Autoimmune Hepatitis Research Network (AHRN), in 2014 to provide a secure and reputable distillation of current literature and AIH research opportunities. Quarterly advertisements for our ongoing observational AIH study were posted on the AHRN over 2 years. Interested and self-reported AIH participants were subsequently enrolled after review of study materials and completion of an informed consent by our study coordinator. Participants returned completed study materials, including epidemiologic questionnaires and genetic material, to our facility via mail. Outside medical records were obtained and reviewed by a study physician. RESULTS We successfully obtained all study materials from 29 participants with self-reported AIH within 2 years from 20 different states. Liver biopsy results were available for 90% (26/29) of participants, of which 81% (21/29) had findings consistent with AIH, 15% (4/29) were suggestive of AIH with features of primary biliary cholangitis (PBC), and 4% (1/29) had PBC alone. A total of 83% (24/29) had at least 2 of 3 proposed criteria: positive autoimmune markers, consistent histologic findings of AIH on liver biopsy, and reported treatment with immunosuppressant medications. Self-reported and physician records were discrepant for immunosuppressant medications or for AIH/PBC diagnoses in 4 patients. CONCLUSIONS Facebook can be an effective ancillary tool for facilitating patient-oriented research in rare diseases. A social media-based approach transcends established limitations in rare disease research and can further develop research communities.

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Factors Predicting Relapse and Poor Outcome in Type I Autoimmune Hepatitis: Role of Cirrhosis Development, Patterns of Transaminases During Remission and Plasma Cell Activity in the Liver Biopsy

The American Journal of Gastroenterology ◽

10.1111/j.1572-0241.2004.30457.x ◽

2004 ◽

Vol 99 (8) ◽

pp. 1510-1516 ◽

Cited By ~ 119

Author(s):

Sumita Verma ◽

Basuki Gunuwan ◽

Michel Mendler ◽

Sugantha Govindrajan ◽

Allan Redeker

Keyword(s):

Liver Biopsy ◽

Autoimmune Hepatitis ◽

Plasma Cell ◽

Cell Activity ◽

Type I ◽

Development Patterns ◽

Poor Outcome

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Autoimmune Hepatitis with Concomitant Idiopathic Thrombocytopenic Purpura Diagnosed by Transjugular Liver Biopsy

Case Reports in Hepatology ◽

10.1155/2018/5305691 ◽

2018 ◽

Vol 2018 ◽

pp. 1-6

Author(s):

Hiromi Fukuda ◽

Kazuhide Takata ◽

Takanori Kitaguchi ◽

Ryo Yamauchi ◽

Hideo Kunimoto ◽

...

Keyword(s):

Platelet Count ◽

Liver Biopsy ◽

Autoimmune Hepatitis ◽

Histological Examination ◽

Idiopathic Thrombocytopenic Purpura ◽

Liver Dysfunction ◽

Severe Thrombocytopenia ◽

Percutaneous Liver Biopsy ◽

Thrombocytopenic Purpura ◽

Transjugular Liver Biopsy

Patients with autoimmune hepatitis (AIH) may sometimes have concomitant idiopathic thrombocytopenic purpura (ITP). Severe thrombocytopenia in ITP interferes with percutaneous liver biopsy for pathological diagnosis of AIH. Here, we report a case of AIH with ITP in a 63-year-old woman. The patient presented to our hospital with liver dysfunction and thrombocytopenia. For histological examination, transjugular liver biopsy (TJLB) was performed, leading to a diagnosis of AIH. Corticosteroids treatment led to an improvement in her liver enzyme levels and platelet count. In conclusion, patients with AIH may sometimes have concomitant ITP. TJLB was effective for making the diagnosis of AIH with severe thrombocytopenia due to ITP.

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