Case Presentation:
A 34‐year‐old man, originally from India, presented with 6 months of polyarthralgias, sinusitis, episcleritis, intermittent hearing loss, and microscopic hematuria. He had signs of acute on chronic sinusitis and bilateral ground‐glass iung opacities on CT. Infectious workup was negative. Serologic studies revealed high c‐ANCA titers with positive anti‐PR3 antibodies, suggestive of Wegener's granulomatosis. He was initiated on prednisone with rapid improvement, but he soon relapsed when prednisone was tapered to obtain a sinus biopsy, results of which were nonspecific Symptoms returned with fever, polyarthritis, purpuric lesions on the elbows and knuckles, and bilateral scrotal pain from orchitis. Ultrasound showed thickened epididymal heads and hypoechoic foci in the testicles, consistent with necrotizing vasculitic disease. Skin biopsy of the purpuric lesions demonstrated marked necrotizing vasculitis of small‐ and medium‐sized vessels in a nodular distribution, suggestive of Wegener's vasculitis. The patient's orchitis improved with IV methylprednisolone. Cyclophosphamide was considered but deferred to allow time for sperm banking. His subsequent course has included 2 additional hospitalizations for severe arthralgias, recurrent purpuric lesions, and new neurologic complaints including foot drop. In addition to motor weakness, he was found to have decreased sensation in the right S1 distribution. His deficits were most consistent with a vasculitic radiculopathy, although mononeuritis multiplex was also on the differential. He responded to high‐dose steroids and cyclophosphamide during each relapse and is currently doing well on outpatient immunosuppressive therapy.
Discussion:
Wegener's granulomatosis (WG) is a small‐ and medium‐vessel vasculitis disease classically associaled with upper and lower airway and renal disease. Interestingly, this patient had many other manifestations, including skin, eye, neurologic, and genitourinary findings. Involvement of the genitourinary tract in WG is rare, but case reports do exist, with prostatitis being the most common. This patient had clinical symptoms of orchitis and radiographic findings of necrotizing disease of the testicles. Orchitis has been described in other vasculitic diseases, particularly in polyarteritis nodosa. Genitourinary disease in WG generally responds well to high‐dose steroids and cyclophosphamide. The same is not true, unfortunately, for the overall course ofWG. Relapse is common in WG and more likely than with Us sister disease, microscopic polyangiitis. Cornerstones of Ireatment remain steroids and immunomodulators such as cyclophosphamide.
Conclusions:
This case demonstrates the systemic nature of Wegener's granulomatosis (WG) and its potential to affect any organ system, including less common sites such as the genitourinary tract. WG may be responsive to immunosuppressive treatment, but patients often suffer significant morbidity and relapse is fairly common.
Author Disclosure:
C. Parker, none; C. Lien, none.