title: Celiac disease in patients with sporadic and inherited cardiomyopathies and in their relatives.
abstract: 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.
authors.affiliation: Department of Pediatrics, I.R.C.C.S. Burlo Garofolo, Trieste, Italy.
authors.forename: Tarcisio
authors.initials: T
authors.lastname: Not
authors.forename: Elena
authors.initials: E
authors.lastname: Faleschini
authors.forename: Alberto
authors.initials: A
authors.lastname: Tommasini
authors.forename: Alessandra
authors.initials: A
authors.lastname: Repetto
authors.forename: Michele
authors.initials: M
authors.lastname: Pasotti
authors.forename: Valentina
authors.initials: V
authors.lastname: Baldas
authors.forename: Andrea
authors.initials: A
authors.lastname: Spano
authors.forename: Daniele
authors.initials: D
authors.lastname: Sblattero
authors.forename: Roberto
authors.initials: R
authors.lastname: Marzari
authors.forename: Carlo
authors.initials: C
authors.lastname: Campana
authors.forename: Antonello
authors.initials: A
authors.lastname: Gavazzi
authors.forename: Luigi
authors.initials: L
authors.lastname: Tavazzi
authors.forename: Federico
authors.initials: F
authors.lastname: Biagi
authors.forename: Gino
authors.initials: G
authors.lastname: Roberto Corazza
authors.forename: Alessandro
authors.initials: A
authors.lastname: Ventura
authors.forename: Eloisa
authors.initials: E
authors.lastname: Arbustini
mesh_terms: 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*
chemical_list: D000906:Antibodies; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D011503:Transglutaminases