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(Circulation. 2002;105:2611.)
© 2002 American Heart Association, Inc.

Clinical Investigation and Reports

Celiac Disease Associated With Autoimmune Myocarditis

Andrea Frustaci, MD; Lucio Cuoco, MD; Cristina Chimenti, MD; Maurizio Pieroni, MD; Giuseppina Fioravanti, CTER; Nicola Gentiloni, MD; Attilio Maseri, MD; Giovanni Gasbarrini, MD

From the Departments of Cardiology (A.F., C.C., M.P., A.M.), Internal Medicine (L.C., N.G., G.G.), and Transplant Surgery (F.G.), Catholic University, Rome, Italy.

Correspondence to Andrea Frustaci, MD, Cardiology Department, Catholic University, Largo Gemelli 8, 00168 Rome, Italy. E-mail biocard{at}rm.unicatt.it

Background— Both celiac disease (CD) and myocarditis can be associated with systemic autoimmune disorders; however, the coexistence of the 2 entities has never been investigated, although its identification may have a clinical impact.

Methods and Results— We screened the serum of 187 consecutive patients with myocarditis (118 males and 69 females, mean age 41.7±14.3 years) for the presence of cardiac autoantibodies, anti–tissue transglutaminase (IgA-tTG), and anti-endomysial antibodies (AEAs). IgA-tTG–positive and AEA-positive patients underwent duodenal endoscopy and biopsy and HLA analysis. Thirteen of the 187 patients were positive for IgA-tTG, and 9 (4.4%) of them were positive for AEA. These 9 patients had iron-deficient anemia and exhibited duodenal endoscopic and histological evidence of CD. CD was observed in 1 (0.3%) of 306 normal controls (P<0.003). In CD patients, myocarditis was associated with heart failure in 5 patients and with ventricular arrhythmias (Lown class III-IVa) in 4 patients. From histological examination, a lymphocytic infiltrate was determined to be present in 8 patients, and giant cell myocarditis was found in 1 patient; circulating cardiac autoantibodies were positive and myocardial viral genomes were negative in all patients. HLA of the patients with CD and myocarditis was DQ2-DR3 in 8 patients and DQ2-DR5(11)/DR7 in 1 patient. The 5 patients with myocarditis and heart failure received immunosuppression and a gluten-free diet, which elicited recovery of cardiac volumes and function. The 4 patients with arrhythmia, after being put on a gluten-free diet alone, showed improvement in the arrhythmia (Lown class I).

Conclusions— A common autoimmune process toward antigenic components of the myocardium and small bowel can be found in >4% of the patients with myocarditis. In these patients, immunosuppression and a gluten-free diet can be effective therapeutic options.

Key Words: celiac disease • myocarditis • immune system

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