Case report: Testicular manifestation of ANCA vasculitis

Abstract

ANCA-associated vasculitis is a rare autoimmune disorder affecting small to medium-sized vessels, often targeting the respiratory tract and kidneys. Testicular involvement is rare and can resemble malignancy, leading to unnecessary surgery. A 36-year-old male presented with painful fingers, oral and nasal ulcers, and knee arthritis. Elevated proteinase 3 (PR3) antibodies confirmed PR3 ANCA-associated vasculitis. During hospitalization, patient developed testicular pain, and an ultrasound raised suspicion of malignancy. An inguinal orchidectomy was performed, revealing inflammation consistent with vasculitis, but no malignancy. Testicular involvement in ANCA vasculitis can mimic cancer, and increased awareness may help prevent unnecessary surgical procedures.

1 Introduction

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic autoimmune disease characterized by inflammation and necrosis of small to medium-sized blood vessels, which can lead to multi-organ involvement ( bbb, ). While it commonly affects the respiratory tract and kidneys, genital manifestations are rare and often underrecognized, especially when they mimic more common malignancies. Testicular involvement in ANCA-associated vasculitis is particularly uncommon, with clinical presentations that can be challenging to distinguish from testicular cancer, often leading to radical surgical interventions. Here, we present a case of testicular manifestation of ANCA vasculitis that clinically resembled testicular cancer, resulting in an orchidectomy. This case report aims to raise awareness among medical specialists and addresses the importance of considering vasculitis in the differential diagnosis of testicular masses, potentially avoiding unnecessary surgical procedures in similar cases.

2 Case presentation

A 36-year old male patient with no significant prior medical history presented to the emergency department with painful, swollen and acrocyanotic fingers. Additionally, the patient exhibited oral and nasal ulcerative mucosal lesions, otitis media and arthritis of the knees. Internal medicine and rheumatology were consulted. Laboratory results revealed elevated proteinase 3 (PR3) antibodies (170 U/mL, normal range <3 U/mL), suggestive of PR3 ANCA-associated vasculitis, and the patient was admitted to the internal medicine department. There were no signs of respiratory or renal involvement.

With suspicion of PR3 ANCA-associated vasculitis, immunosuppressive treatment was initiated with methylprednisolone, rituximab and iloprost, alongside analgesics and nifedipine for vasodilation. During hospital admission, the patient reported mild pain in the right testicle and subsequently urology was consulted. At physical examination, a mass in the right testicle was palpated. Abdominal and pulmonal examination were without abnormalities. Serum markers, including alpha-fetoprotein, lactate dehydrogenase and human chorionic gonadotropin were within normal limits. Scrotal ultrasound revealed a focal hypoechoic lesion in the right testicle, measuring 1.9 × 1.4 cm, with no detectable blood flow ( Fig. 1 ). As ultrasound could not rule out malignancy, an inguinal orchidectomy of the right testicle was performed on the following day. A subsequent CT scan showed no evidence of metastasis.