Polyangiitis with granulomatosis, previously known as Wegener’s granulomatosis,
is a systemic necrotising granulomatous vasculitis. It predominantly affects the
upper and lower respiratory tracts and the kidneys, but can potentially affect any
organ system. It is diagnosed by clinical features, immunology (anti-neutrophil
cytoplasmic antibodies) and histology. Cardiac involvement occurs in 6 to 44% of
cases. We present a case of polyangiitis with granulomatosis and constrictive
pericarditis, which occurred despite vigorous immunosuppression and which
required surgical pericardectomy.
