URETHRAL DIVERTICULUM: AN UNUSUAL PRESENTATION OF WEGENER'S GRANULOMATOSIS

Wegener's granulomatosis is characterized by vasculitis and necrotizing granulomatous inflammation that classically affects the upper respiratory tract and lungs, and frequently involves the kidneys with pauci-immune glomerulonephritis.' Almost any organ may be involved, and eye, skin, nervous system and inner ear involvement are not uncommon. Involvement of the lower urinary tract is unusual.l.* To our knowledge we report the first case in which the initial presenting sign of Wegener's granulomatosis was a urethral diverticulum. CASE RE PORT A 56-year-old white woman presented with vaginal spotting. Full evaluation was unrevealing except for a nontender fluctuant area in the distal anterior vaginal wall, which had been incised transvaginally elsewhere. Some pus had been expressed and the incision was closed but the area soon became tender and indurated. The patient was placed on antibiotics and referred for further definitive evaluation and treatment. Urethroscopy revealed inflammatory changes as well as an ostium in the ventral distal urethra. Voiding cystourethrography confirmed a urethral diverticulum at the corresponding location (fig. 1). At surgery it was noted that the distal anterior vaginal wall had become much more indurated with pronounced inflammatory changes. Frozen sections of the distal anterior vaginal wall and tissue beneath it revealed no malignant elements. After meticulous dissection we removed a large doughnut-shaped urethral diverticulum circumferentially surrounding the distal half of the urethra with a portion of the anterior vaginal wall. A firm yellowish material was contained within the diverticulum. The anterior vaginal wall was reconstructed by advancing vaginal wall flaps to surround the urethra and cover the distal anterior vagina. Final pathological evaluation revealed a 4.5 x 3.8 x 1.4 cm. urethral diverticulum with a mass of necrotizing granulomatous inflammation (fig. 2). All tissue cultures and a purified protein derivative tuberculin test were negative. The patient did well until 1 month later, when a macular rash, arthralgia and chondritis of the right ear developed. Additional evaluations demonstrated superficial nasal septa1 ulceration, bilateral scleritis, nodules on the legs, fever of 101F. and paresthesia of the hands and feet. Chest x-ray showed a right lower lobe infiltrate and computerized tomography revealed 3 focal lung infiltrates consistent with local vasculitis. Bronchoscopy excluded infection. Antineutrophil cytoplasmic antibodies with anti-proteinase 3 specificity were present. Serum creatinine and urinalysis (catheterized sample) were normal. Based on the histological findings, laboratory data, constellation of symptoms and signs, and radiological findings Wegener's granulomatosis was diagnosed. A regimen of prednisone and methotrexate was initiated. During the ensuing 8 weeks symptoms resolved, the radiological findings disappeared and the anterior vaginal wall healed completely. Currently the patient is asymptomatic. Prednisone will be ta