Autoantibodies against liver mitochondria, indicating primary biliary cholangitis
Anti-mitochondrial antibodies
(
AMA
) are
autoantibodies
, consisting of
immunoglobulins
formed against
mitochondria
,
[1]
primarily the mitochondria in
cells
of the
liver
.
The presence of AMA in the
blood
or
serum
of a person may be indicative of the presence of, or the potential to develop, the
autoimmune disease
primary biliary cholangitis
(PBC; previously known as
primary biliary cirrhosis
). PBC causes scarring of liver tissue, confined primarily to the bile duct drainage system. AMA is present in about 95% of cases.
[2]
PBC is seen primarily in middle-aged women, and in those afflicted with other autoimmune diseases.
Antigens
[
edit
]
Several of the antigens associated with anti-mitochondrial antibodies have been identified.
[3]
Disease associations
[
edit
]
Antibodies to these specific antigens have been associated with a number of conditions:
[4]
anti M2, M4, M8, and M9 are associated with primary biliary cholangitis; M2 ? autoimmune hepatitis; M1 ?
syphilis
; M3 ? drug-induced lupus erythematosus; M6 ? drug-induced
hepatitis
; M7 ?
cardiomyopathy
,
myocarditis
; M5 ?
systemic lupus erythematosus
and undifferentiated collagenosis, autoimmune haemolytic anaemia.
[5]
These associations are not completely specific and should not be relied upon solely for diagnosis.
Antimitochondrial antibodies can also be detected in
Sjogren's syndrome
,
systemic sclerosis
, asymptomatic recurrent
bacteriuria
in women, pulmonary
tuberculosis
, and
leprosy
.
[4]
Anti-cardiolipin antibodies
are another type of AMA, and
cardiolipin
is found on the
inner mitochondrial membrane
.
Development
[
edit
]
A cause of AMA has been postulated to be that xenobiotic-induced and/or oxidative modification of mitochondrial autoantigens is a critical step leading to loss of tolerance. In
acute liver failure
AMA are found against all major liver antigens.
[6]
Around 40.5% of acute liver failure patients were found to have elevated AMA, although a larger proportion (56.9%) had
anti-transglutaminase antibodies
, usually associated with
coeliac disease
.
[6]
See also
[
edit
]
References
[
edit
]
- ^
MedlinePlus Encyclopedia
:
003529
- ^
Oertelt S, Rieger R, Selmi C, Invernizzi P, Ansari A, Coppel R, Podda M, Leung P, Gershwin M (2007).
"A sensitive bead assay for antimitochondrial antibodies: Chipping away at AMA-negative primary biliary cirrhosis"
.
Hepatology
.
45
(3): 659?65.
doi
:
10.1002/hep.21583
.
PMID
17326160
.
- ^
Berg PA, Klein R (1992) Antimitochondrial antibodies in primary biliary cirrhosis and other disorders: definition and clinical relevance: Dig Dis 10(2):85-101
- ^
a
b
Berg PA, Klein R (1986) Mitochondrial antigens and autoantibodies: from anti-M1 to anti-M9. Klin Wochenschr 64(19):897-909
- ^
Labro MT, Andrieu MC, Weber M, Homberg JC (1976) A new pattern of non-organ- and non-species-specific anti-organelle antibody detected by immunofluorescence: the mitochondrial antibody number 5. Clin Exp Immunol 31(3):357-366
- ^
a
b
Leung PS, Rossaro L, Davis PA, et al. (2007).
"Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis"
.
Hepatology
.
46
(5): 1436?42.
doi
:
10.1002/hep.21828
.
PMC
3731127
.
PMID
17657817
.