scholarly journals Seronegative Autoimmune Hepatitis A Clinically Challenging Difficult Diagnosis

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
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.

scholarly journals Sudden Sensorioneural Hearing Loss and Autoimmune Systemic Diseases

2016 ◽  
Vol 21 (03) ◽  
pp. 213-223 ◽  
Author(s):  
Bruno Rossini ◽  
Norma Penido ◽  
Mario Munhoz ◽  
Eduardo Bogaz ◽  
Renata Curi
Keyword(s):  
Hearing Loss ◽  
Response To Treatment ◽  
Unknown Etiology ◽  
Profound Hearing Loss ◽  
Appropriate Treatment ◽  
Immune Mediated ◽  
Clinical Presentations ◽  
Unilateral Involvement ◽  
The Relationship ◽  
Probable Diagnosis

Introduction Several authors have demonstrated the relationship between sudden sensorineural hearing loss (SNHL) and systemic autoimmune diseases (SAD). Immune-mediated SNHL can rarely present as unilateral sudden SNHL and manifests itself in the contralateral ear only after years. It presents clinical relevance for being one of the few SNHL that may be reversible given that early and appropriate treatment is applied. Objective The objective of this study is to describe the clinical presentations and audiological findings from patients with idiopathic sudden SNHL and SAD associated with a probable diagnosis of immune-mediated SNHL. Furthermore, we strive to estimate the prevalence of SAD in patients with sudden SNHL. Methods This is an observational retrospective cohort. We have selected and studied patients with SAD. Revision of available literature on scientific repositories. Results We evaluated 339 patients with sudden SNHL. Among them, 13 (3.83%) patients suffered from SAD. Three patients had bilateral involvement, a total of 16 ears. We evaluate and describe various clinical, epidemiological, and audiological aspects of this sample. Conclusion In our sample of patients with sudden SNHL, the prevalence of SAD was found relevant. The majority had tinnitus and dizziness concomitant hearing loss, unilateral involvement and had experienced profound hearing loss at the time of the installation. In spite of instituted treatment, most cases showed no improvement in audiometric thresholds. Apparently, patients with sudden SNHL and SAD have a more severe initial impairment, higher percentage of bilateral, lower response to treatment, and worse prognosis than patients with sudden SNHL of unknown etiology.

Diagnostic Criteria for Autoimmune Hepatitis: Scores and More

2015 ◽  
Vol 33 (Suppl. 2) ◽  
pp. 47-52 ◽  
Author(s):  
Ansgar W. Lohse
Keyword(s):  
Liver Biopsy ◽  
Autoimmune Hepatitis ◽  
Diagnostic Criteria ◽  
Clinical Use ◽  
Serological Markers ◽  
Diagnostic Score ◽  
Different Populations ◽  
International Autoimmune Hepatitis Group ◽  
Patient’S History ◽  
Diagnostic Scores

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.

scholarly journals Scar sarcoidosis: additional involvement of de novo sites and update on immunopathogenesis

2019 ◽  
Vol 5 (4) ◽  
pp. 898
Author(s):  
Vasudha A. Belgaumkar ◽  
Ravindranath B. Chavan ◽  
Prernaa R. Suryataley ◽  
Pallavi P. Patil ◽  
Vijay V. Raut
Keyword(s):  
Developing Countries ◽  
Inflammatory Disease ◽  
De Novo ◽  
Unknown Etiology ◽  
Cutaneous Sarcoidosis ◽  
Diverse Range ◽  
Noncaseating Granuloma ◽  
Immune Mediated ◽  
Clinical Presentations ◽  
Scar Sarcoidosis

Cutaneous sarcoidosis is an immune-mediated multisystem granulomatous inflammatory disease of unknown etiology. Noncaseating granuloma in pre-existing scars is an uncommon but specific cutaneous manifestation of sarcoidosis. This great imitator may be an under-diagnosed disease in developing countries like India probably because of the diverse range of clinical presentations. Herein we present a 45-year old female with cutaneous sarcoidosis involving scars in addition to de-novo sites.

scholarly journals Autoimmune Hepatitis: Clinical Manifestations and Diagnostic Criteria

2001 ◽  
Vol 15 (2) ◽  
pp. 107-113 ◽  
Author(s):  
Ian G Mcfarlane
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Scoring System ◽  
Clinical Manifestations ◽  
Chronic Viral Hepatitis ◽  
Published Data ◽  
Group A ◽  
Diagnostic Modalities ◽  
Circulating Autoantibodies ◽  
International Autoimmune Hepatitis Group

In 1998, the International Autoimmune Hepatitis Group - a panel of 40 hepatologists and hepatopathologists from 17 countries who have a particular interest in autoimmune hepatitis (AIH) - undertook a review, in light of subsequent experience, of the descriptive criteria and diagnostic scoring system that it had proposed in 1993 for the diagnosis of AIH. This review (published in 1999) noted that the original descriptive criteria appeared to be quite robust and required only relatively minor modifications to bring them up to date with developments and experience in diagnostic modalities for liver disease in general. Analysis of published data on the application of the original criteria in nearly 1000 patients revealed that the diagnostic scoring system had an overall diagnostic accuracy of 89.8%, with a sensitivity of 98.0%. Specificity for excluding definite AIH in patients with chronic viral hepatitis and circulating autoantibodies or patients with overlapping cholestatic syndromes was 98% to 100%, but specificity for excluding probable AIH in these disorders ranged from only 60% to 80%. Modifications, including adjustments to the weightings against biochemical and histological cholestatic features, have been made to the scoring system to improve its specificity.

scholarly journals Occurrence of autoimmune chronic hepatitis in siblings

2005 ◽  
Vol 62 (7-8) ◽  
pp. 591-594 ◽  
Author(s):  
Darko Nozic ◽  
Jovan Dimitrijevic ◽  
Slavica Knezevic-Usaj
Keyword(s):  
Chronic Hepatitis ◽  
Case Report ◽  
Autoimmune Hepatitis ◽  
Chronic Inflammatory Disease ◽  
Hepatic Tissue ◽  
Unknown Etiology ◽  
Hepatic Parenchyma ◽  
Family Case ◽  
Loss Of Tolerance ◽  
International Autoimmune Hepatitis Group

Background. Autoimmune hepatitis is a chronic inflammatory disease of the liver of unknown etiology, characterized by the loss of tolerance against hepatic tissue, leading to the destruction of hepatic parenchyma. It predominantly affects females, and rarely occurs in the same family. Case report. In this paper we presented brother and sister with autoimmune hepatitis according to the criteria of the International Autoimmune Hepatitis Group. Conclusion. Because of a possible genetic predisposition to autoimmune hepatitis, the occurrence of the disease in a family member suggested the need to examine other family members.

scholarly journals Autoimmune Hepatitis as a Unique Form of an Autoimmune Liver Disease: Immunological Aspects and Clinical Overview

2012 ◽  
Vol 2012 ◽  
pp. 1-17 ◽  
Author(s):  
Hind I. Fallatah ◽  
Hisham O. Akbar
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Response To Treatment ◽  
Unique Form ◽  
Decompensated Liver Cirrhosis ◽  
Aggressive Disease ◽  
Immune Mediated ◽  
Acute Liver Disease ◽  
Progressive Liver Disease ◽  
Clinical Overview

Autoimmune hepatitis (AIH) is a unique form of immune-mediated disease that attacks the liver through a variety of immune mechanisms. The outcomes of AIH are either acute liver disease, which can be fatal, or, more commonly, chronic progressive liver disease, which can lead to decompensated liver cirrhosis if left untreated. AIH has characteristic immunological, and pathological, features that are important for the establishment of the diagnosis. More importantly, most patients with AIH have a favorable response to treatment with prednisolone and azathioprine, although some patients with refractory AIH or more aggressive disease require more potent immune-suppressant agents, such as cyclosporine or Mycophenolate Mofetil. In this paper, we discuss the immunological, pathological and clinical features of AIH, as well as the standard and alternative treatments for AIH.

scholarly journals Autoimmune hepatitis and complexities in management

2018 ◽  
Vol 10 (1) ◽  
pp. 77-87 ◽  
Author(s):  
Ashnila Janmohamed ◽  
Gideon M Hirschfield
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Standard Therapy ◽  
Effective Therapy ◽  
Adult Patients ◽  
Therapeutic Options ◽  
Second Line ◽  
Immune Mediated ◽  
Vast Variation ◽  
Case Based

Autoimmune hepatitis (AIH) is a rare heterogenous immune-mediated liver disease that for the majority has effective therapy, usually resulting in excellent prognosis. Treatment is based on immunosuppression using standard therapy with corticosteroids and azathioprine. Second-line therapeutic options exist for those who are non-responders (‘difficult to treat AIH’) or intolerant to standard therapy; however, their use is not standardised, and in addition, there is vast variation in practice and efficacy. Given the rarity of AIH, expertise in its management can be limited to large referral programmes. In this case-based review, we aim to discuss common clinical dilemmas encountered by clinicians managing adult patients with AIH and address the related competencies in the 2010 Gastroenterology curriculum.

scholarly journals CONCEPT OF AUTOIMMUNE HEPATITIS IN AYURVEDA PERSPECTIVE – A CONCEPTUAL STUDY

2020 ◽  
pp. 1-2
Author(s):  
Arti Jain* ◽  
Rajesh Uikey ◽  
Naresh Jain
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Chronic Liver Disease ◽  
Diagnostic Criteria ◽  
Environmental Parameters ◽  
Main Site ◽  
Digestion Process ◽  
Literature Overview ◽  
Conceptual Study ◽  
Almost All

Autoimmune hepatitis is a chronic inammatory disorder characterized by high levels of aminotransferase and auto antibodies, hypergammaglobulinemia and interface hepatitis. The etiology of AIH has not been completely elucidated, but immunogenetic background and environmental parameters may contribute to itsdevelopment. Autoimmune hepatitis does not have a pathgnomonic features, and its laboratory,serologic, andhistologic manifestations are found in acute and chronic liver disease of diverse causes.Autoimmune Hepatitis can be compared to mandya Bhutangni in Yakrit. According to Ayurveda,Yakrut is the main site of Bhutangni and one of the sub types of pitta namely Ranjakapitta. This Bhutangni helps in the digestion process as well as Ranjakapitta imparts colour to the blood. According to Charkathe digestion of food by jatharangi results in the breakdown of food in to ve distinct physiochemical groups. Jatharangi ignites the Agni fraction present in each of ve groups. This Bhutangni then converts the respective portion in to assimilable form. If the function of Bhutangni is not proper, at this stage Ama would be produced is called Bhutangni Manda janya Ama. If Agni improperly processed during digestion and metabolism of food it disturbs the process leading formation of Ama. Which is responsible for the manifestation of almost all disease especially autoimmune hepatitis compared with Bhutangni giving a detailed literature overview of Bhutangni, Immunopathogenesis (Ama production) and diagnostic criteria.

Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group (IAIHG) criteria in acute and chronic liver disease

Hepatology ◽  
2009 ◽  
Vol 50 (2) ◽  
pp. 538-545 ◽  
Author(s):  
Andrew D. Yeoman ◽  
Rachel H. Westbrook ◽  
Thawab Al-Chalabi ◽  
Ivana Carey ◽  
Nigel D. Heaton ◽  
...  
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Chronic Liver Disease ◽  
Diagnostic Value ◽  
Chronic Liver ◽  
International Autoimmune Hepatitis Group
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