Copyright © 2003 by the European Society of Cardiology.
Regular Articles
Celiac disease in patients with sporadic and inherited cardiomyopathies and in their relatives
a Department of Pediatrics, I.R.C.C.S. Burlo Garofolo, Trieste, Italy
b Department of Cardiology, I.R.C.C.S. Policlinico San Matteo, Pavia, Italy
c Department of Biology, University of Trieste, Trieste, Italy
d Department of Cardiology, Ospedali Riuniti, Bergamo, Italy
e Gastroenterology, University Unit I.R.C.C.S. Policlinico San Matteo, Pavia, Italy
f Molecular Diagnostics, Cardiovascular and Transplant Pathology Laboratory, Transplant Research Area, I.R.C.C.S. Policlinico San Matteo, Pavia, Italy
* Correspondence to: Eloisa Arbustini, MD, Molecular Diagnostic, Cardiovascular and Transplant Pathology Laboratory, Transplant Research Area, I.R.C.C.S. Policlinico San Matteo, Via Forlanini 16, 27100 Pavia, Italy. Tel: +39 0382 503829; fax: +39 0382 525866
E-mail address: e.arbustini{at}smatteo.pv.it
Received 12 December 2002; revised 15 May 2003; accepted 21 May 2003
Aims To investigate celiac disease (CD) and related co-morbidity in patients with familial and sporadic cardiomyopathy and in their relatives.
Methods and 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.
Key Words: Celiac disease • Dilated cardiomyopathy • Tissue-transglutaminase