LETTER TO THE EDITOR
Immunosuppressive treatment in familial dilated cardiomyopathy with biopsy-proven intramyocardial inflammation?
Michel Noutsias, MD*,*,
Matthias Pauschinger, MD*,
Uwe Kühl, PhD* and
Heinz-Peter Schultheiss, MD*
* Department of Cardiology and Pneumonology, University Hospital Benjamin Franklin, Hindenburgdamm 30, D-12200 Berlin, Germany
* Reprint requests and correspondence: Dr. Michel Noutsias, Department of Cardiology and Pneumonology, University Hospital Benjamin Franklin, Hindenburgdamm 30, D-12200 Berlin, Germany.
noutsias{at}zedat.fu-berlin.de
In a recent issue of the Journal, Mahon et al. (1) added substantially to the pathogenesis of familial dilated cardiomyopathy (DCM), elucidating that intramyocardial inflammation, as assessed by immunohistochemical quantification of CD3+ T-lymphocytes and abundance of endothelial cell adhesion molecule expression (ICAM-1 and HLA-DR), is significantly present in asymptomatic individuals with left ventricular enlargement and who are relatives of patients with familial DCM. Their report confirms previous findings on anticardiac autoimmunity in familial DCM, such as autoantibodies (2) and the HLA type DR4-linked predisposition (3). The first successful immunosuppressive study in DCM, demonstrating beneficial long-term hemodynamic effects over a two-year follow-up period, was based on the immunohistochemical diagnosis of inflammatory cardiomyopathy (i.e., HLA abundance) (4). In contrast, preliminary data by Chimenti et al. (5) elucidated that only patients with biopsy-proven absence of viral persistence will benefit from such immunosuppressive treatment.
Given the reported absence of enteroviral, adenoviral, and cytomegaloviral genome in familial DCM (6), would the investigators consider immunosuppressive treatment to prevent disease progression in patients with established familial DCM and in their asymptomatic relatives who have left ventricular enlargement?
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1. Mahon NG, Madden BP, Caforio ALP, et al. Immunohistologic evidence of myocardial disease in apparently healthy relatives of patients with dilated cardiomyopathy. J Am Coll Cardiol. 2002;39:455–462[Abstract/Free Full Text]
2. Caforio ALP, Keeling PJ, Zachara E, et al. Evidence from family studies for autoimmunity in dilated cardiomyopathy. Lancet. 1994;344:773–777[CrossRef][Medline]
3. McKenna CJ, Codd MB, McCann HA, Sugrue DD. Idiopathic dilated cardiomyopathy: familial prevalence and HLA distribution. Heart. 1997;77:549–552[Abstract/Free Full Text]
4. Wojnicz R, Nowalany-Kozielska E, Wojciechowska C, et al. Randomized, placebo-controlled study for immunosuppressive treatment of inflammatory dilated cardiomyopathy: two-year follow-up results. Circulation. 2001;104:39–45[Abstract/Free Full Text]
5. Chimenti C, Calabrese F, Pieroni M, et al. Active lymphocytic myocarditis: virologic and immunologic profile of responders versus non-responders to immunosuppressive therapy. (abstr)Circulation. 2001;104:II559
6. Mahon NG, Zal B, Arno G, et al. Absence of viral nucleic acids in early and late dilated cardiomyopathy. Heart. 2001;86:687–692[Abstract/Free Full Text]
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