title: Current insights into the pathogenesis, diagnosis and therapy of inflammatory cardiomyopathy.
abstract: Persistence of cardiotropic viruses (enterovirus, adenovirus) and anticardiac autoimmunity constitute the predominant etiopathogenic pathways of dilated cardiomyopathy (DCM). The diagnosis of inflammatory cardiomyopathy (InfCM) imposes sensitivity and specificity requirements, which are not fulfilled by the histological Dallas Criteria. The immunohistological quantification and characterization of immunocompetent infiltrates and cell adhesion molecule (CAM) expression has endorsed a new entity of secondary cardiomyopathies acknowledged by the World Health Organization (WHO), InfCM, in approximately 50% of DCM patients. In the absence of viral persistence, InfCM patients benefit from immunosuppressive treatment. Enteroviral and adenoviral genomes have been detected in a significant proportion of DCM patients. Enteroviral persistence is associated with an adverse prognosis. The induction of the coxsackie-adenovirus receptor (CAR) exclusively in 63% of DCM patients, but not in other cardiomyopathies, might constitute a key molecular determinant for cardiotropic viral infections in DCM. In InfCM patients with enterovirus or adenoviral persistence, interferon-beta administration leads to viral elimination and cessation of the intramyocardial inflammation, paralleled by a significant improvement of left ventricular systolic function and heart failure symptoms. The biopsy-guided etiopathogenic differentiation of DCM has endorsed specific treatment strategies: immunosuppressive regimens are favorable in autoimmune InfCM, whereas patients with viral persistence benefit from antiviral immunomodulation.
authors.affiliation: Department of Cardiology and Pneumonology, University Hospital, Benjamin Franklin Free University of Berlin, Hindenburgdamm 30, D-12200 Berlin, Germany. noutsias@zedat.fu-berlin.de
authors.forename: Michel
authors.initials: M
authors.lastname: Noutsias
authors.forename: Matthias
authors.initials: M
authors.lastname: Pauschinger
authors.forename: Wolfgang-Christian
authors.initials: WC
authors.lastname: Poller
authors.forename: Heinz-Peter
authors.initials: HP
authors.lastname: Schultheiss
authors.forename: Uwe
authors.initials: U
authors.lastname: Kühl
mesh_terms: D002311:Cardiomyopathy, Dilated / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D009205:Myocarditis / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy