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Seronegative autoimmune hepatitis

MedGen UID:
1804383
Concept ID:
C5680121
Disease or Syndrome
Synonyms: Autoantibody negative autoimmune hepatitis; Autoantibody-negative autoimmune hepatitis; autoantibody-negative autoimmune hepatitis; Seronegative AIH; seronegative autoimmune hepatitis
SNOMED CT: Autoantibody negative autoimmune hepatitis (1197704005)
 
Monarch Initiative: MONDO:0035400
Orphanet: ORPHA563589

Definition

A form of autoimmune hepatitis characterized by the features of classic autoimmune hepatitis (i. e. clinical presentation as acute or chronic cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aspartate aminotransferase and alanine aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, therapeutic response to corticosteroids) in the absence of serum autoantibodies. Clinical manifestations include fatigue, malaise, arthralgia, jaundice, at later stages also signs of advanced chronic liver disease, such as spider nevi, caput medusae, splenomegaly, ascites, and palmar erythema. Presence of concurrent autoimmune diseases is frequently observed. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSeronegative autoimmune hepatitis

Professional guidelines

PubMed

Maggiore G, Pietrobattista A, Liccardo D, Sciveres M, Nastasio S, Jacquemin E, Bernard O
J Pediatr Gastroenterol Nutr 2021 Aug 1;73(2):e48. doi: 10.1097/MPG.0000000000003165. PMID: 34016885

Recent clinical studies

Therapy

Schmutz M, Chartier S, Leblanc T, Mussini C, Gardin A, Gonzales E, Roque-Afonso AM, Le Cam S, Hery G, Neven B, Charbel R, Vartanian JP, Jacquemin E, Morelle G, Almes M
Front Immunol 2024;15:1445610. Epub 2024 Sep 11 doi: 10.3389/fimmu.2024.1445610. PMID: 39328418Free PMC Article
Gonnot M, Neumann F, Huet F, Maudinas R, Leblanc T, Lacaille F
J Pediatr Gastroenterol Nutr 2022 Nov 1;75(5):553-555. Epub 2022 Sep 6 doi: 10.1097/MPG.0000000000003603. PMID: 36070526
Maggiore G, Pietrobattista A, Liccardo D, Sciveres M, Nastasio S, Jacquemin E, Bernard O
J Pediatr Gastroenterol Nutr 2021 Aug 1;73(2):e48. doi: 10.1097/MPG.0000000000003165. PMID: 34016885
Maggiore G, Socie G, Sciveres M, Roque-Afonso AM, Nastasio S, Johanet C, Gottrand F, Fournier-Favre S, Jacquemin E, Bernard O
Dig Liver Dis 2016 Jul;48(7):785-91. Epub 2016 Mar 28 doi: 10.1016/j.dld.2016.03.015. PMID: 27079745
Ozaslan E, Samsar U, Erden E
Hepatogastroenterology 2010 Mar-Apr;57(98):334-5. PMID: 20583438

Prognosis

Schmutz M, Chartier S, Leblanc T, Mussini C, Gardin A, Gonzales E, Roque-Afonso AM, Le Cam S, Hery G, Neven B, Charbel R, Vartanian JP, Jacquemin E, Morelle G, Almes M
Front Immunol 2024;15:1445610. Epub 2024 Sep 11 doi: 10.3389/fimmu.2024.1445610. PMID: 39328418Free PMC Article
Islek A, Keskin H
Acta Gastroenterol Belg 2021 Apr-Jun;84(2):305-310. doi: 10.51821/84.2.305. PMID: 34217180
Quail MA, Russell RK, Bellamy C, Mieli-Vergani G, Gillett PM
Eur J Gastroenterol Hepatol 2009 May;21(5):576-9. doi: 10.1097/MEG.0b013e3282fa1400. PMID: 19318969
Gassert DJ, Garcia H, Tanaka K, Reinus JF
Dig Dis Sci 2007 Sep;52(9):2433-7. Epub 2007 Apr 12 doi: 10.1007/s10620-006-9665-4. PMID: 17429719

Clinical prediction guides

Schmutz M, Chartier S, Leblanc T, Mussini C, Gardin A, Gonzales E, Roque-Afonso AM, Le Cam S, Hery G, Neven B, Charbel R, Vartanian JP, Jacquemin E, Morelle G, Almes M
Front Immunol 2024;15:1445610. Epub 2024 Sep 11 doi: 10.3389/fimmu.2024.1445610. PMID: 39328418Free PMC Article
Islek A, Keskin H
Acta Gastroenterol Belg 2021 Apr-Jun;84(2):305-310. doi: 10.51821/84.2.305. PMID: 34217180
Heringlake S, Schütte A, Flemming P, Schmiegel W, Manns MP, Tillmann HL
Z Gastroenterol 2009 May;47(5):417-23. Epub 2009 May 5 doi: 10.1055/s-0028-1109146. PMID: 19418409

Supplemental Content

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    Clinical resources

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