Constrictive pericarditis as first presentation of IgG4-related disease: pitfalls and pearls.

Abstract

IgG-4 related disease (IgG4-RD) is an under recognised multisystem inflammatory disorder that has several typical manifestations. Cardiac manifestations of IgG4-RD are well documented however do not feature in the definition or diagnosis of IgG4-RD according to a recent consensus statement. The most well recognised cardiac manifestation of IgG4-RD, pericardial disease, is outlined in this case report as the initial presenting pathology. In the present case the diagnosis was delayed due to the relative obscurity of cardiac manifestations of IgG4-RD and exposed the patient to the risks of pericardiectomy.