First described in 1936, Wegener’s granulomatosis (WG) is a clinical syndrome of unknown etiology characterized by necrotizing granulomatous vasculitis of the upper respiratory tract (74%), lungs (70%), and kidneys (46%). It affects all ages but is common in middle-aged men. Although renal involvement is common, lower urogenital involvement WG is rare.
A 31 year old man was admitted to the hospital with a 4-month history of night sweats, anorexia, and 30 lb weight loss and one-month history of hemoptysis and progressive dyspnea. One month prior, he had been diagnosed with necrotizing prostatitis by TURP after experiencing rectal pain, dysuria, and hematuria. After the TURP, he experienced transient blurred vision and hearing loss. He had emigrated to the US 12 years ago from Ecuador and had not traveled since then. At the time of admission, he was in hypoxemic respiratory failure and required support with mechanical ventilation. The initial examination of the chest was normal but rapidly diffuse crackles were appreciated. Lateral episcleritis was noted in both eyes. Admitting laboratory data revealed a hemoglobin of 8.2, ABGs on room air of pH 7.51, pCO2 28.5 and pO2 52.9. The BUN was 8, creatinine of 0.7, and the urinalysis showed “large” blood, “trace” protein, 40 RBCs, and 18 WBCs. A chest radiograph showed the development of bilateral upper lobe infiltrates, new from one week prior. The initial differential diagnosis included pneumonia and vasculitis. He was started on broad-spectrum antibiotics, antituberculous medications, and systemic corticosteroids. Bronchoalveolar lavage (BAL) revealed frank blood and a predominance of neutrophils. BAL bacterial, fungal, mycobacterial, and viral cultures, special stains and serum antinuclear, anti-GBM antibodies, and rheumatic factor were all negative. A PPD was negative. C-ANCA was positive. Review of the prostate tissue showed a leukocytoclastic vasculitis, fibrinoid necrosis, and giant cells consistent with WG. Cyclophosphamide was added and antimicrobials were discontinued. The patient improved rapidly over 14 days, was extubated, and ultimately discharged breathing on room air on a corticosteroid taper and continued cyclophosphamide.
Involvement of the lower urogenital tract in WG is rare. Prostatitis is the most common manifestation causing frequency, dysuria, hematuria, and urinary retention. To our knowledge, only 28 cases of symptomatic prostatitis due to WG have been reported in the literature. Walton reported granuloma involving the prostate in 7.4% of cases. Stillwell et al. reported in 1987 only 2.3% (four of 174 patients) cases with WG with prostatic involvement. We report a case of WG that initially presented with extrapulmonary manifestations including prostatitis, transient vision and hearing loss followed by fulminant hypoxemic respiratory failure secondary to diffuse alveolar hemorrhage. Tuberculosis was initially considered but felt excluded by the negative PPD and BAL findings. He responded well to corticosteroids and cytotoxic therapy.
WG is a multisystem disorder that is usually, but not exclusively, limited to the respiratory tract and kidneys. Symptomatic involvement of the lower genitourinary tract is rare but may be more frequent than previously thought. Symptoms of the urogenital system may precede the onset of pulmonary disease. Because tuberculosis, fungal and bacterial infections may cause a similar granulomatous inflammation and vasculitis, microscopic stains and cultures should be reviewed before initiating immunosuppressive therapy. Urogenital manifestations of WG seem highly sensitive to corticosteroid and cyclophosphamide therapy.
C.Q. See, None.