U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Antimitochondrial antibody positivity

MedGen UID:
866704
•Concept ID:
C4021051
•
Finding
Synonym: Serum antimitochrondrial antibodies
 
HPO: HP:0030167

Definition

The presence of autoantibodies (immunoglobulins) in the serum that react against mitochondria. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAntimitochondrial antibody positivity

Conditions with this feature

Reynolds syndrome
MedGen UID:
450547
•Concept ID:
C0748397
•
Disease or Syndrome
An autoimmune disorder characterized by the association of primary biliary cirrhosis with limited cutaneous systemic sclerosis. Onset occurs between 30-65 years. Occurs sporadically, but rare familial cases with an unknown inheritance pattern have been observed. There is no cure and management is mainly supportive.
See: Condition Record
Immunodeficiency 25
MedGen UID:
346666
•Concept ID:
C1857798
•
Disease or Syndrome
Any severe combined immunodeficiency in which the cause of the disease is a mutation in the CD247 gene.
See: Condition Record
Immunodeficiency 25
Reynolds syndrome

Professional guidelines

PubMed

Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history.
Murillo Perez CF, Goet JC, Lammers WJ, Gulamhusein A, van Buuren HR, Ponsioen CY, Carbone M, Mason A, Corpechot C, Invernizzi P, Mayo MJ, Battezzati PM, Floreani A, Pares A, Nevens F, Kowdley KV, Bruns T, Dalekos GN, Thorburn D, Hirschfield G, LaRusso NF, Lindor KD, Zachou K, Poupon R, Trivedi PJ, Verhelst X, Janssen HLA, Hansen BE; GLOBAL PBC Study Group
Hepatology 2018 May;67(5):1920-1930. Epub 2018 Apr 6 doi: 10.1002/hep.29717. PMID: 29220537
Primary biliary cirrhosis and chronic active hepatitis: an examination of clinical, biochemical, and histopathological features in differential diagnosis.
Williamson JM, Chalmers DM, Clayden AD, Dixon MF, Ruddell WS, Losowsky MS
J Clin Pathol 1985 Sep;38(9):1007-12. doi: 10.1136/jcp.38.9.1007. PMID: 2413079Free PMC Article

Recent clinical studies

Etiology

Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history.
Murillo Perez CF, Goet JC, Lammers WJ, Gulamhusein A, van Buuren HR, Ponsioen CY, Carbone M, Mason A, Corpechot C, Invernizzi P, Mayo MJ, Battezzati PM, Floreani A, Pares A, Nevens F, Kowdley KV, Bruns T, Dalekos GN, Thorburn D, Hirschfield G, LaRusso NF, Lindor KD, Zachou K, Poupon R, Trivedi PJ, Verhelst X, Janssen HLA, Hansen BE; GLOBAL PBC Study Group
Hepatology 2018 May;67(5):1920-1930. Epub 2018 Apr 6 doi: 10.1002/hep.29717. PMID: 29220537
Hispanics with primary biliary cirrhosis are more likely to have features of autoimmune hepatitis and reduced response to ursodeoxycholic acid than non-Hispanics.
Levy C, Naik J, Giordano C, Mandalia A, O'Brien C, Bhamidimarri KR, Schiff ER, Martin P
Clin Gastroenterol Hepatol 2014 Aug;12(8):1398-405. Epub 2013 Dec 17 doi: 10.1016/j.cgh.2013.12.010. PMID: 24361417
Primary biliary cirrhosis is more severe in overweight patients.
Híndi M, Levy C, Couto CA, Bejarano P, Mendes F
J Clin Gastroenterol 2013 Mar;47(3):e28-32. doi: 10.1097/MCG.0b013e318261e659. PMID: 23059407
Immunoreactivity to M2 proteins in antimitochondrial antibody-negative patients with primary biliary cirrhosis.
Kitami N, Ishii H, Shimizu H, Adachi H, Komada T, Mikami H, Yokoi Y, Sato N
J Gastroenterol Hepatol 1994 Jan-Feb;9(1):7-12. doi: 10.1111/j.1440-1746.1994.tb01208.x. PMID: 8155871
Primary biliary cirrhosis and chronic active hepatitis: an examination of clinical, biochemical, and histopathological features in differential diagnosis.
Williamson JM, Chalmers DM, Clayden AD, Dixon MF, Ruddell WS, Losowsky MS
J Clin Pathol 1985 Sep;38(9):1007-12. doi: 10.1136/jcp.38.9.1007. PMID: 2413079Free PMC Article

Diagnosis

Histology and clinical correlations in autoimmune hepatitis, primary biliary cholangitis, and autoimmune hepatitis-primary biliary cholangitis overlap syndrome.
Liang Y, Khandakar B, Hao Y, Xiong Y, Liu BL, Zhang X
Ann Diagn Pathol 2023 Dec;67:152178. Epub 2023 Jul 12 doi: 10.1016/j.anndiagpath.2023.152178. PMID: 37468373
Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history.
Murillo Perez CF, Goet JC, Lammers WJ, Gulamhusein A, van Buuren HR, Ponsioen CY, Carbone M, Mason A, Corpechot C, Invernizzi P, Mayo MJ, Battezzati PM, Floreani A, Pares A, Nevens F, Kowdley KV, Bruns T, Dalekos GN, Thorburn D, Hirschfield G, LaRusso NF, Lindor KD, Zachou K, Poupon R, Trivedi PJ, Verhelst X, Janssen HLA, Hansen BE; GLOBAL PBC Study Group
Hepatology 2018 May;67(5):1920-1930. Epub 2018 Apr 6 doi: 10.1002/hep.29717. PMID: 29220537
Primary biliary cirrhosis is more severe in overweight patients.
Híndi M, Levy C, Couto CA, Bejarano P, Mendes F
J Clin Gastroenterol 2013 Mar;47(3):e28-32. doi: 10.1097/MCG.0b013e318261e659. PMID: 23059407
Validation of coding algorithms for the identification of patients with primary biliary cirrhosis using administrative data.
Myers RP, Shaheen AA, Fong A, Wan AF, Swain MG, Hilsden RJ, Sutherland L, Quan H
Can J Gastroenterol 2010 Mar;24(3):175-82. doi: 10.1155/2010/237860. PMID: 20352146Free PMC Article
Primary biliary cirrhosis and chronic active hepatitis: an examination of clinical, biochemical, and histopathological features in differential diagnosis.
Williamson JM, Chalmers DM, Clayden AD, Dixon MF, Ruddell WS, Losowsky MS
J Clin Pathol 1985 Sep;38(9):1007-12. doi: 10.1136/jcp.38.9.1007. PMID: 2413079Free PMC Article

Therapy

Histology and clinical correlations in autoimmune hepatitis, primary biliary cholangitis, and autoimmune hepatitis-primary biliary cholangitis overlap syndrome.
Liang Y, Khandakar B, Hao Y, Xiong Y, Liu BL, Zhang X
Ann Diagn Pathol 2023 Dec;67:152178. Epub 2023 Jul 12 doi: 10.1016/j.anndiagpath.2023.152178. PMID: 37468373
Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history.
Murillo Perez CF, Goet JC, Lammers WJ, Gulamhusein A, van Buuren HR, Ponsioen CY, Carbone M, Mason A, Corpechot C, Invernizzi P, Mayo MJ, Battezzati PM, Floreani A, Pares A, Nevens F, Kowdley KV, Bruns T, Dalekos GN, Thorburn D, Hirschfield G, LaRusso NF, Lindor KD, Zachou K, Poupon R, Trivedi PJ, Verhelst X, Janssen HLA, Hansen BE; GLOBAL PBC Study Group
Hepatology 2018 May;67(5):1920-1930. Epub 2018 Apr 6 doi: 10.1002/hep.29717. PMID: 29220537
The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis.
Floreani A, Motta R, Cazzagon N, Franceschet I, Roncalli M, Del Ross T, Rosina F, Lleo A, Mescoli C, Colloredo G, Invernizzi P
Dig Liver Dis 2015 May;47(5):432-5. Epub 2015 Feb 11 doi: 10.1016/j.dld.2015.02.002. PMID: 25747115
Hispanics with primary biliary cirrhosis are more likely to have features of autoimmune hepatitis and reduced response to ursodeoxycholic acid than non-Hispanics.
Levy C, Naik J, Giordano C, Mandalia A, O'Brien C, Bhamidimarri KR, Schiff ER, Martin P
Clin Gastroenterol Hepatol 2014 Aug;12(8):1398-405. Epub 2013 Dec 17 doi: 10.1016/j.cgh.2013.12.010. PMID: 24361417

Prognosis

Histology and clinical correlations in autoimmune hepatitis, primary biliary cholangitis, and autoimmune hepatitis-primary biliary cholangitis overlap syndrome.
Liang Y, Khandakar B, Hao Y, Xiong Y, Liu BL, Zhang X
Ann Diagn Pathol 2023 Dec;67:152178. Epub 2023 Jul 12 doi: 10.1016/j.anndiagpath.2023.152178. PMID: 37468373
Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history.
Murillo Perez CF, Goet JC, Lammers WJ, Gulamhusein A, van Buuren HR, Ponsioen CY, Carbone M, Mason A, Corpechot C, Invernizzi P, Mayo MJ, Battezzati PM, Floreani A, Pares A, Nevens F, Kowdley KV, Bruns T, Dalekos GN, Thorburn D, Hirschfield G, LaRusso NF, Lindor KD, Zachou K, Poupon R, Trivedi PJ, Verhelst X, Janssen HLA, Hansen BE; GLOBAL PBC Study Group
Hepatology 2018 May;67(5):1920-1930. Epub 2018 Apr 6 doi: 10.1002/hep.29717. PMID: 29220537
The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis.
Floreani A, Motta R, Cazzagon N, Franceschet I, Roncalli M, Del Ross T, Rosina F, Lleo A, Mescoli C, Colloredo G, Invernizzi P
Dig Liver Dis 2015 May;47(5):432-5. Epub 2015 Feb 11 doi: 10.1016/j.dld.2015.02.002. PMID: 25747115
Primary biliary cirrhosis is more severe in overweight patients.
Híndi M, Levy C, Couto CA, Bejarano P, Mendes F
J Clin Gastroenterol 2013 Mar;47(3):e28-32. doi: 10.1097/MCG.0b013e318261e659. PMID: 23059407
Validation of coding algorithms for the identification of patients with primary biliary cirrhosis using administrative data.
Myers RP, Shaheen AA, Fong A, Wan AF, Swain MG, Hilsden RJ, Sutherland L, Quan H
Can J Gastroenterol 2010 Mar;24(3):175-82. doi: 10.1155/2010/237860. PMID: 20352146Free PMC Article

Clinical prediction guides

Histology and clinical correlations in autoimmune hepatitis, primary biliary cholangitis, and autoimmune hepatitis-primary biliary cholangitis overlap syndrome.
Liang Y, Khandakar B, Hao Y, Xiong Y, Liu BL, Zhang X
Ann Diagn Pathol 2023 Dec;67:152178. Epub 2023 Jul 12 doi: 10.1016/j.anndiagpath.2023.152178. PMID: 37468373
Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history.
Murillo Perez CF, Goet JC, Lammers WJ, Gulamhusein A, van Buuren HR, Ponsioen CY, Carbone M, Mason A, Corpechot C, Invernizzi P, Mayo MJ, Battezzati PM, Floreani A, Pares A, Nevens F, Kowdley KV, Bruns T, Dalekos GN, Thorburn D, Hirschfield G, LaRusso NF, Lindor KD, Zachou K, Poupon R, Trivedi PJ, Verhelst X, Janssen HLA, Hansen BE; GLOBAL PBC Study Group
Hepatology 2018 May;67(5):1920-1930. Epub 2018 Apr 6 doi: 10.1002/hep.29717. PMID: 29220537
Primary biliary cirrhosis is more severe in overweight patients.
Híndi M, Levy C, Couto CA, Bejarano P, Mendes F
J Clin Gastroenterol 2013 Mar;47(3):e28-32. doi: 10.1097/MCG.0b013e318261e659. PMID: 23059407
Validation of coding algorithms for the identification of patients with primary biliary cirrhosis using administrative data.
Myers RP, Shaheen AA, Fong A, Wan AF, Swain MG, Hilsden RJ, Sutherland L, Quan H
Can J Gastroenterol 2010 Mar;24(3):175-82. doi: 10.1155/2010/237860. PMID: 20352146Free PMC Article
Primary biliary cirrhosis and chronic active hepatitis: an examination of clinical, biochemical, and histopathological features in differential diagnosis.
Williamson JM, Chalmers DM, Clayden AD, Dixon MF, Ruddell WS, Losowsky MS
J Clin Pathol 1985 Sep;38(9):1007-12. doi: 10.1136/jcp.38.9.1007. PMID: 2413079Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...