www.nephropathol.com Journal of Nephropathology, Vol 1, No 1, April 2012 Wegener’s and ventricular masses… 49 Granulomatosis with polyangiitis (Wegener’s) presenting as the right ventricular masses: A case report and review of the literature. Mojgan Mortazavi„, Hamid Nasri 2,* Case Report Implication for health policy/practice/research/medical education: Granulomatosis with polyangiitis (Wegeners) is a systemic necrotizing vasculitis, rarely presented with ven- tricular masses and also multiple echo-free lesions in the spleen. Cardiac masses and spleen lesions completely resolved after corticosteroid pulse therapy. GPA should be considered in the differential diagnosis of any nonspeci!c illnesses with cardiac involvement. Please cite this paper as: Mortazavi M. Nasri H. Granulomatosis with polyangiitis (Wegeners) presenting as the right ventricular masses: A case report and review of the literature. J Nephro Pathol. 2012; 1(1): 49-56. DOI: 10.5812/jnp.9 *Corresponding author: Prof. Hamid Nasri, Department of Nephrology, Division of Nephropathology, Isfahan University of Medical Sciences, Isfahan, Iran. Email: hamidnasri@med.mui.ac.ir „ Isfahan Kidney Disease Research Center, Isfahan University of Medical Sciences, Isfahan, Iran. ² Department of Nephrology, Division of Nephropathology, Isfahan University of Medical Sciences, Isfahan, Iran. www.nephropathol.com J Nephro Pathol. 2012; 1(1): 49-56 Journal of Nephropathology ABSTRACT Background: Granulomatosis with polyangiitis (Wegeners) is a systemic necrotiz- ing vasculitis. Cardiac involvement in Wegeners has rarely been reported. In this study the echocardiographic !ndings 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 en- largements as well as three small masses in the right ventricular cavity were identi- !ed in echocardiography. One mass was attached to the tricuspid valve and the other two masses were attached to the right septum. Pulmonary artery hyperten- sion (45mmHg) was also reported. The masses responded dramatically to plasma exchanges in combination with steroid therapy, followed by oral cyclophospha- mid and low-dose steroid therapy. In the kidney biopsy, 8 out of 11 golomeruli contained !brous crescents along with sclerotic lesions. Spleen has 140 mm di- ameter with multiple echo-free lesions and coarse parenchyma in abdominal ul- trasound. 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 pulmo- nary artery hypertension in association with the spleen lesions were an uncom- mon presentation of GPA. GPA should be considered in the differential diagno- sis of any nonspeci!c illnesses with cardiac involvement. ARTICLE INFO Article type: Case Report Article history: Received: 1 Nov 2011 Revised: 10 Nov 2011 Accepted: 20 Dec 2011 Published online:18 Mar 2012 DOI: 10.5812/jnp.9 Keywords: Granulomatosis Polyangiitis Wegeners Vasculitis Ventricular mass