Very little has been published about single-organ vasculitis of the testicle in the radiological literature. Consequently, it is a diagnosis that is unfamiliar to most radiologists. This case report describes the sonographic, pathologic, and laboratory findings of testicular vasculitis and reviews the available literature with regard to this subject.
Systemic vasculitides can often involve the testes; however, isolated vasculitis of the testes is uncommon [
An 84-year-old male presented to the emergency department with testicular pain worsening over a 24-hour period. The patient was otherwise healthy with no significant medical concerns and no other symptoms. A testicular ultrasound was arranged on an urgent basis with the differential diagnosis consisting of epididymoorchitis versus torsion.
Sonographic evaluation revealed a heterogeneous appearance of both testicles with diminished parenchymal Doppler flow (Figure
Multiple axial and longitudinal sonographic images (a–c) of the left testicle with Doppler color demonstrating a heterogeneous appearance, with multiple hypoechoic mass-like areas, and lack of Doppler flow within the majority of the testis. The right testis (image not provided) had a similar appearance.
The specimen was submitted for pathologic evaluation, which revealed a medium vessel vasculitis with associated hemorrhagic infarction of much of the testicular parenchyma (Figures
Histological section (H&E stain) of the left testicle viewed at medium (a) and high (b) power demonstrating a proliferation of neutrophils as well as T and B lymphocytes surrounding one of the intratesticular vessels consistent with vasculitis. No granulomas are present.
Histological section (H&E stain) of the left testicle viewed at medium power demonstrating hemorrhagic infarction of seminiferous tubules secondary to underlying vasculitis.
Given the pathological findings, additional blood work to assess an underlying vasculitis was obtained. The antinuclear antibody (ANA) screen was negative. The anti-neutrophil cytoplasmic antibody (ANCA) indirect immunofluorescence (IIF) was positive with a perinuclear pattern (p-ANCA). Anti-proteinase 3 (PR3-ANCA) and anti-myeloperoxidase (MPO-ANCA) antibody testing by ELISA (INOVA Diagnostics Inc.) were both negative. Protein electrophoresis revealed decreased albumin and beta 1 (LDL and transferrin) and beta 2 (C3) globulins. Midstream urinalysis was unremarkable. CRP was elevated at 72 mg/L (reference range < 10 mg/L). The patient was hepatitis B and hepatitis C negative. Liver function tests were normal. HIV status was not determined, but the patient had no known risk factors. Given the medium vessel involvement demonstrated on pathological assessment, as well as the blood work, a diagnosis of nongranulomatous testicular vasculitis was made. Clinical workup for the presence of systemic vasculitis was negative and inflammatory markers returned to normal values following orchiectomy and medical management.
Unfortunately, the patient was lost to follow-up following discharge which we acknowledge is a limitation of this case report.
Various forms of vasculitis can involve the testes. While PAN is the most common form to affect the testicle, granulomatosis with polyangiitis (GPA), Henoch-Schonlein purpura, giant cell arteritis, and vasculitis associated with some autoimmune connective tissue disorders such as Systemic Lupus Erythematosus (SLE) can also involve the testes [
PAN was first described by Kussmaul and Maier in 1866 and commonly affects multiple organs in a patient such as the skin, kidneys, gastrointestinal tract, and peripheral and central nervous systems [
In 2012, the Chapel Hill consensus for nomenclature of vasculitides added “single-organ vasculitis” as a new category to differentiate PAN which is reserved for the primary systemic form of this medium-sized vessel vasculitis [
Laboratory results, in addition to pathological findings, are crucial in establishing a specific vasculitis as the causative factor for the testicular findings seen on ultrasound. ANA positivity is suggestive of a diagnosis of SLE or other connective tissue diseases. ANCA positivity is helpful in identifying certain small vessel vasculitides. A cytoplasmic pattern (c-ANCA) by IIF and PR-3 positivity by ELISA are suggestive of GPA. A p-ANCA by IIF and MPO positivity by ELISA are suggestive of microscopic polyangiitis (MPA). Our patient had a positive p-ANCA by IIF and a negative PR3 and MPO by ELISA, supporting a diagnosis of PAN-type rather than GPA or MPA. It should be noted that PAN is not classically associated with ANCA [
There is no consensus regarding the treatment of TV, although it is postulated that the excision of the affected organ is curative [
As mentioned previously, the sonographic diagnosis of SOV affecting the testicle is a difficult one. Little has been published about the sonographic appearances of testicular vasculitis in the radiological literature [
Testicular vasculitis is a great mimic [
It should be noted that a limitation of this study was the lack of follow-up of the patient. It is true that, in any medium-sized vessel vasculitis, P-ANCA positivity at IIF (even in the absence of MPO specificity by ELISA) should alert the clinician for the possible systemic extent (even subclinical), which can also evolve to a generalized disease over time.
The presented case provides an important teaching point with regard to the differential diagnosis of nonspecific testicular sonographic findings in patients with testicular pain. Due to a relative lack of literature addressing testicular vasculitis, it may be a diagnosis that is overlooked. In the right clinical setting, even given the lack of specific ultrasound findings, testicular vasculitis is an entity that should be considered.
The authors declare that they have no competing interests.