Cardiac involvement in Wegener's granulomatosis

Abstract

Wegener's granulomatosis is a systemic inflammatory disorder of unknown aetiology. The protean clinical presentations depend on the organ(s) involved and the degree of progression from a local to a systemic arteritis. The development of serological tests (antineutrophil cytoplasmic antibodies) allows easier diagnosis of a disease whose incidence is increasing. This is particularly helpful where the presentation is not classic-for example "overlap syndromes"-or where the disease presents early in a more localised form. This is true of cardiac involvement, which is traditionally believed to be rare, but may not be as uncommon as has hitherto been thought (≦44%). This involvement may be subclinical or the principal source of symptoms either in the form oflocalised disease or as part of a systemic illness. Pericarditis, arteritis, myocarditis, valvulitis, and arrhythmias are all recognised. Wegener's granulomatosis should therefore be considered in the differential diagnosis of any non-specific illness with cardiac involvement. This includes culture negative endocarditis, because Wegener's granulomatosis can produce systemic upset with mass lesions and vasculitis. Echocardiography and particularly transoesophageal echocardiography can easily identify and delineate cardiac and proximal aortic involvement and may also be used to assess response to treatment.