Simultaneous testicle and epididymis vasculitis revealing granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatous vasculitis, which predominantly affects small-sized blood vessels. We aimed to report a case of GPA involving testicles and epididymis taken for malignancy. A 75-year-old patient was admitted for a painful left testicular mass. There was no extra urogenital manifestations on examination and the workup was unremarkable. Histological findings after orchidectomy revealed granulomatous inflammation of the testis and epididymis with fibrinoid necrosis and necrotic vasculitis consisting with the diagnosis of GPA. Further investigations regarding ear, nose, throat, pulmonary, and renal involvement were negative. Proteinase 3 antineutrophil cytoplasmic antibodies (PR3-ANCA) test was negative. Furthermore, infectious diseases especially tuberculosis were ruled out. Based on histopathological findings, limited GPA was diagnosed. The patient was treated with methotrexate and prednisone with good outcome. There was no relapse after 1 year of follow-up. Isolated urogenital involvement may occur at the onset of GPA and can be taken for malignancy. Histopathological findings are the gold standard for the diagnosis. Treatment is based on steroids and immunosuppressive drugs.