Abstract
Background: Granulomatosis with polyangiitis (Wegener’s) is a systemic necrotizing
vasculitis. Cardiac involvement in Wegener’s has rarely been reported. In this
study the echocardiographic findings of granulomatosis with polyangiitis (GPA)
in a patient is described.
Case Presentation: The case was a 45 years old man admitted to the hospital with a 3
months history of persistent fever and sinusitis. Mild left and right ventricular enlargements
as well as three small masses in the right ventricular cavity were identified
in echocardiography. One mass was attached to the tricuspid valve and the
other two masses were attached to the right septum. Pulmonary artery hypertension
(45mmHg) was also reported. The masses responded dramatically to plasma
exchanges in combination with steroid therapy, followed by oral cyclophosphamid
and low-dose steroid therapy. In the kidney biopsy, 8 out of 11 golomeruli
contained fibrous crescents along with sclerotic lesions. Spleen has 140 mm diameter
with multiple echo-free lesions and coarse parenchyma in abdominal ultrasound.
Serum C-ANCA=671, P-ANCA=1.7 (normal= up to 15U/mL). The
diagnosis of granulomatosis with polyangiitis (Wegener’s) was established.
Conclusions: Presence of three small masses in right ventricular cavity and pulmonary
artery hypertension in association with the spleen lesions were an uncommon
presentation of GPA. GPA should be considered in the differential diagnosis
of any nonspecific illnesses with cardiac involvement.