Circulating heart autoantibodies in familial as compared with nonfamilial idiopathic dilated cardiomyopathy.
BACKGROUND
Idiopathic dilated cardiomyopathy (DCM) is a serious heart disease characterized by enlargement of one or both ventricles and ventricular dysfunction. Although most patients have sporadic disease, 20% have been found to have familial DCM when relatives are investigated by echocardiography. No other factors have been identified to date that consistently distinguish familial from nonfamilial DCM. Although some patients have a family history of DCM, a "negative" family history does not exclude familial DCM because affected family members may be presymptomatic or undiagnosed. Because some patients have life-threatening complications at the time of initial assessment of DCM, identifying a serum marker predictive of familial disease would help determine which families would most likely benefit from echocardiographic investigation.


OBJECTIVE
In this study, our objective was to determine whether antiheart autoantibodies could be used to distinguish familial from nonfamilial idiopathic DCM.


METHODS
We analyzed serum specimens for antiheart antibodies from 19 patients categorized as having familial DCM and 15 classified as having nonfamilial DCM on the basis of echocardiographic investigation of first-degree relatives. The mean duration of disease in these 34 patients was 50 months at the time the serum specimens were obtained.


RESULTS
Titers of antibodies against the adenine nucleotide translocator, branched-chain keto acid dehydrogenase, and cardiac myosin did not distinguish between familial and nonfamilial cases of DCM.
Michels
V V
VV
Department of Medical Genetics, Mayo Clinic Rochester, MN 55905.
Moll
P P
PP
Rodeheffer
R J
RJ
Miller
F A
FA
Tajik
A J
AJ
Burnett
J C
JC
Driscoll
D J
DJ
Thibodeau
S N
SN
Ansari
A A
AA
Herskowitz
A
A
D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D002311:Cardiomyopathy, Dilated / Q000235:genetics / Q000276:immunology*; D005190:Family; D005260:Female; D006801:Humans; D007658:Ketone Oxidoreductases / Q000276:immunology; D008297:Male; D008875:Middle Aged; D000226:Mitochondrial ADP, ATP Translocases / Q000276:immunology; D009097:Multienzyme Complexes / Q000276:immunology; D009206:Myocardium / Q000276:immunology*; D009218:Myosins / Q000276:immunology
D001323:Autoantibodies; D009097:Multienzyme Complexes; D000226:Mitochondrial ADP, ATP Translocases; D007658:Ketone Oxidoreductases; D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); D009218:Myosins