Celiac disease in patients with sporadic and inherited cardiomyopathies and in their relatives.
OBJECTIVE
To investigate celiac disease (CD) and related co-morbidity in patients with familial and sporadic cardiomyopathy and in their relatives.


RESULTS
We screened anti-human-tissue-transglutaminase (IgA and IgG anti-h-tTG) and anti-endomysial antibodies (AEAs) in 238 consecutive adult patients with inherited or sporadic dilated cardiomyopathy (DCM), 418 relatives, and 2000 healthy blood donors. HLADQ2-DQ8 was tested in tTG-positive subjects. The IgA-tTG-positive patients with cardiomyopathy underwent duodenal biopsy. Twenty-six subjects were tTG-positive: five DCM patients (2.1%), two of 28 (7.1%) and three of 390 (0.7%) relatives with and without echocardiographic abnormalities respectively, and 16 controls (0.8%). Twenty-two of 26 subjects were AEA-positive, and 25 HLA-positive. Of the five patients with cardiomyopathy and biopsy-proven CD, four suffered iron-deficiency anaemia. Two CD-positive DCM patients and two tTG-positive relatives were from families with inherited disease in which CD did not co-segregate with DCM. CONCLUSIONS; The higher prevalence of CD in patients with sporadic or inherited DCM, and of tTG-positive serology in relatives with echocardiographic abnormalities, suggests that immune-mediated mechanisms are active in subsets of patients/families. However, gluten intolerance cannot be considered causative since CD seems to be associated but not co-segregated with DCM in familial cases.
Not
Tarcisio
T
Department of Pediatrics, I.R.C.C.S. Burlo Garofolo, Trieste, Italy.
Faleschini
Elena
E
Tommasini
Alberto
A
Repetto
Alessandra
A
Pasotti
Michele
M
Baldas
Valentina
V
Spano
Andrea
A
Sblattero
Daniele
D
Marzari
Roberto
R
Campana
Carlo
C
Gavazzi
Antonello
A
Tavazzi
Luigi
L
Biagi
Federico
F
Roberto Corazza
Gino
G
Ventura
Alessandro
A
Arbustini
Eloisa
E
D000293:Adolescent; D000328:Adult; D000368:Aged; D000906:Antibodies / Q000097:blood; D009202:Cardiomyopathies / Q000235:genetics*; D002446:Celiac Disease / Q000235:genetics*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011503:Transglutaminases / Q000276:immunology*
D000906:Antibodies; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D011503:Transglutaminases