Concurrent Autoimmune Diseases in Patients With Autoimmune Hepatitis

Teufel, Andreas; Weinmann, Arndt; Kahaly, George J.; Centner, Catherine; Piendl, Anja; Wörns, Marcus A.; Lohse, Ansgar W.; Galle, Peter R.; Kanzler, Stephan

doi:10.1097/mcg.0b013e3181c74e0d

Cited by 185 publications

(140 citation statements)

References 70 publications

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“…AIH has extrahepatic features of autoimmunity, such as high serum globulin concentrations and the presence of circulating autoantibodies (1). Patients with AIH occasionally have other autoimmune diseases, including hemolytic anemia, immune thrombocytopenia, type 1 diabetes mellitus, thyroiditis, celiac sprue, ulcerative colitis, rheumatoid arthritis, and systemic lupus erythematosus (SLE) (2).…”

Section: Introductionmentioning

confidence: 99%

Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus

Lim

Lee

Ahn

et al. 2016

Arthritis Care & Research

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Objective. Autoimmune hepatitis (AIH) is a chronic progressive liver disease characterized by circulating autoantibodies and hyperglobulinemia. This study was conducted to identify the features of AIH accompanied by systemic lupus erythematosus (SLE-AIH) that differ from those of primary AIH (P-AIH), and to evaluate factors that affect the outcome for SLE-AIH patients. Methods. From May 1995 to April 2014, clinical data (including liver pathology) from 164 patients with P-AIH and 23 patients with SLE-AIH were collected from an electronic database at a tertiary referral center. AIH was diagnosed according to the diagnostic scoring system for AIH (revised in 1999). SLE patients fulfilled at least 4 of the 1997 revised American College of Rheumatology criteria. Progression was defined as occurrence of cirrhosis, hepatocellular carcinoma, liver transplantation, or death from hepatic failure. Results. The age at the time of AIH diagnosis was lower and initial levels of serum IgG were higher in SLE-AIH than in P-AIH patients. Progression was more common in P-AIH, and hepatocellular carcinoma, liver transplantation, or death occurred only in P-AIH patients. Among 23 patients with SLE-AIH, 8 with cirrhosis had higher levels of serum IgG than 15 patients without cirrhosis (mean 6 SD 4,077.4 6 1,641.0 mg/dl and 2,560.7 6 932.2 mg/dl, respectively; P 5 0.017). Moreover, a serum IgG level more than 2-fold the upper normal limit was associated with a high risk of cirrhosis in SLE-AIH (odds ratio 11.00 [95% confidence interval 1.420-85.201]; P 5 0.026). Conclusion. Initially high levels of serum IgG are a poor prognostic factor for SLE-AIH. Additionally, the long-term outcome for SLE-AIH might be better than for P-AIH.

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Section: Introductionmentioning

confidence: 99%

Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus

Lim

Lee

Ahn

et al. 2016

Arthritis Care & Research

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“…Diseases involving polyclonal increases in IgM such as in this case include rheumatoid arthritis, Sjögren syndrome, acute hepatitis type A, and chronic nephritis, in addition to PBC, and PBC -AIH overlap. PBC is often associated with Sjögren's syndrome 6,7) , but this was not the situation in our case, which had no the symptoms of dryness and showed negative results for anti -SS -A and anti -SS -B antibodies. Chronic nephritis was also ruled out because renal function and urinary findings were normal.…”

Section: Discussionmentioning

confidence: 48%

A CASE OF OVERLAP SYNDROME OF PRIMARY BILIARY CIRRHOSIS AND AUTOIMMUNE HEPATITIS WITH MARKED ELEVATION OF IgM

Katsushima

Takahashi

Suzuki

et al. 2013

FJMS

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: A 60 -year -old woman was admitted to our hospital because of anorexia and vomiting. Hematology revealed elevated levels of hepatobiliary enzymes and positive results for antinuclear antibody (ANA) and antimitochondrial (AMA) M2 antibody. Immunoglobulin (Ig) G and IgM levels were extremely elevated, 3,379 mg/dl and 4,250 mg/dl, respectively. A diagnosis of primary biliary cirrhosis and autoimmune hepatitis (PBC -AIH) overlap syndrome was made. However, IgM levels as high as the level of 4,250 mg/dl in this case has not been reported previously. This case may be of value in studying elevated IgM levels with PBC -AIH overlap syndrome and relationships to the mechanism of onset.

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“…En un solo caso se demostró asociación a otra enfermedad autoinmune, como el LES, lo cual puede presentarse de hecho en asociación con cualquier enfermedad autoinmune, tales como tiroiditis autoinmune, enfermedad inflamatoria intestinal, artritis idiopática juvenil, vitiligo, y la enfermedad celíaca, (21)(22)(23)(24) más comúnmente . Los antecedentes familiares de enfermedad autoinmune se presentan en aproximadamente el 40% de los afectados con la HAI, sin embargo, en nuestra casuística no (25) encontramos antecedentes .…”

Section: Discussionunclassified

Hepatitis Autoinmune en niños en un Hospital de Tercer Nivel. Reporte de casos

Morinigo

Ortiz

Ayala

et al. 2015

Pediatr. (Asunción)

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Los autores declaran que no existen conflictos de interés en el presente estudio. RESUMEN Introducción:Casos Clínicos: Discusión: Palabras clave:La Hepatitis autoinmune (HAI) en niños se constituye en una enfermedad inflamatoria del hígado, caracterizada histológicamente por un denso infiltrado de células mononucleares en el sistema porta, y serológicamente por la presencia de auto-anticuerpos específicos del hígado y aumento de los niveles de transaminasas e IgG, en ausencia de una patología conocida que pueda afectar el hígado. Se presenta un total de 10 casos con diagnóstico de HAI, la media de edad es de 12 años, con 2 de sexo femenino (20%) y 8 masculino (80%); 4 casos fueron de tipo 1, un caso de tipo 2, y a 5 pacientes no se los pudo clasificar. La elevación de transaminasas (> x10 su valor normal) se observó en el 70% de los casos y la hipergammaglobulinemia en todos los casos. El tratamiento instaurado consistió en corticosteroides y azatioprina en todos los pacientes, con un tiempo medio de remisión de 4-6 semanas. Se observó abandono de tratamiento en 3 casos de los 10 casos, 2 de ellos con desenlace fatal.La HAI presenta manifestaciones clínicas generales y específicas en etapas tempranas de la enfermedad. El alto porcentaje de respuesta al tratamiento médico nos hace considerar que el diagnóstico y tratamiento precoz son muy importantes, con el objetivo de detener la evolución a la cirrosis.Hepatitis autoinmune, enfermedad hepática crónica, hipergammaglobulinemia. ABSTRACTIntroduction:

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Concurrent Autoimmune Diseases in Patients With Autoimmune Hepatitis

Abstract: Concurrent autoimmune diseases are common in patients with AIH and mirror the full range of known autoimmune diseases. Therefore, an extended diagnostic screening for accumulating autoimmune diseases, especially autoimmune thyroiditis, seems reasonable in patients with AIH.

Cited by 185 publications

References 70 publications

Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus

Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus

A CASE OF OVERLAP SYNDROME OF PRIMARY BILIARY CIRRHOSIS AND AUTOIMMUNE HEPATITIS WITH MARKED ELEVATION OF IgM

Hepatitis Autoinmune en niños en un Hospital de Tercer Nivel. Reporte de casos

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