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Wegener’s granulomatosis involving prostate
CASE REPORT
WEGENER’S GRANULOMATOSIS INVOLVING PROSTATE K. A. GABER, N. G. RYLEY, JAMES P. MACDERMOTT,
AND
J. M. GOLDMAN
ABSTRACT We report a rare case of Wegener’s granulomatosis involving the prostate in a 28-year-old man. The initial medical treatment was effective, but surgical intervention was required to manage late complications. UROLOGY 66: 195e.26–195e.27, 2005. © 2005 Elsevier Inc.
W
egener’s granulomatosis (WG) is a necrotizing vasculitis of unknown etiology. It usually involves the upper airway, lung, and kidney and has an incidence rate of 3:100,000, divided equally between men and women.1 The first-line treatment of WG is medical; however, occasionally surgical management is indicated. We report a rare case of WG involving the prostate that required surgical intervention. CASE REPORT A 28-year-old man presented in February 1995 with cavitating lung lesions, hematuria, peripheral neuropathy, and a collapsed nasal septum. Antineutrophil cytoplasmic antibody was positive, and a nasal biopsy was consistent with vasculitis, confirming a diagnosis of WG. He was initially treated with prednisolone and cyclophosphamide, followed by azathioprine, with good clinical and biochemical responses. Treatment was ceased after 42 months, with the patient in remission. In August 2000, he presented with lethargy, dysuria, and marked perineal pain, with the subsequent development of urinary retention. On examination, he was febrile with suprapubic tenderness, and digital rectal examination revealed an enlarged, acutely tender prostate. Investigations showed a white blood cell count of 10.6 ⫻ 109 From the Department of Respiratory Medicine, Bristol Royal Infirmary, Bristol, United Kingdom; Departments of Histopathology and Urology, Torbay Hospital, Torquay, United Kingdom; and Heart and Lung Unit, Torbay Hospital, Torquay, United Kingdom Address for correspondence: K. A. Gaber, M.R.C.P., Department of Respiratory Medicine, Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW, United Kingdom. E-mail: khalidgbr@ doctors.org.uk Submitted: July 14, 2004, accepted (with revisions): January 24, 2005 © 2005 ELSEVIER INC. 195.e26
ALL RIGHTS RESERVED
cells/L (normal 4 to 10) and a raised C-reactive protein (49 mg/L, normal less than 3) and erythrocyte sedimentation rate (36 mm/hr, normal 1 to 5). His chest radiograph, urea and electrolyte tests, prostate-specific antigen level, and blood and urine cultures were normal. Transrectal ultrasound scans showed an enlarged prostate (about 70 g) and multiple hypoechoic lesions, consistent with prostatic abscesses. Magnetic resonance imaging of the pelvis confirmed prostatic enlargement, but revealed no other pathologic features. Transurethral drainage of the abscesses was performed, and ciprofloxacin 500 mg twice daily was started. The prostatic tissue was negative on conventional bacteriologic culture, Ziehl-Neelsen stain, and culture for acid-fast bacilli. The histologic examination revealed marked inflammation, with areas of eosinophilic necrosis surrounded by neutrophils, histiocytes, and scattered multinucleated giant cells. Vasculitis affecting vessels of variable type and caliber was present. These features were considered consistent with WG involving the prostate gland (Fig. 1). Immunologic investigation confirmed a positive antineutrophil cytoplasmic antibody with positive anti-proteinase 3 activity, which is known to occur in 75% to 90% of patients with active WG.2 The patient was treated with oral cyclophosphamide and prednisolone, with rapid improvement in his symptoms. Three months later, the antineutrophil cytoplasmic antibody test was negative, and azathioprine was substituted for cyclophosphamide. Six months later, the size of his prostate had decreased to 25 g, but new obstructive urinary symptoms had developed. Measurements of the urinary flow rate suggested marked obstruction, with a peak flow of 4.9 mL/s for a volume of 150 mL. Cystoscopy revealed a stricture in the bulbar urethra, and internal urethrotomy and dilation 0090-4295/05/$30.00 doi:10.1016/j.urology.2005.01.045
FIGURE 1. Prostate biopsy showing vessels (arrows) displaying fibrinoid change and neutrophil infiltration of their walls.
were performed. The stricture recurred, and he eventually required membranous urethroplasty. COMMENT Ninety percent of patients with WG develop upper and lower respiratory tract involvement, and 80% develop renal disease.2 It is uncommon for the skin, central nervous system, eyes, and the heart to be involved in WG and rare for the genitourinary tract to be involved. In 1987, Stillwell et al.3 reported that the prostate was involved in only 4 (2.3%) of 174 patients with WG reviewed. The presentation of WG involving the prostate is varied. In a review of 26 patients with prostatic involvement in WG, 8 (31%) had chronic obstructive urinary symptoms, 7 (27%) were asymptomatic, 5 (19%) presented with acute urinary retention, 5 (19%) had hematuria, and 1 (4%) had recurrent urinary tract infection.4 In that series, surgical intervention was unusual, and only 4 of the 26 patients underwent transurethral resection of the prostate.5 In patients with WG, the clinical examination may reveal an enlarged or indurated prostate. The cys-
UROLOGY 66 (1), 2005
toscopic appearances are of a diffusely abnormal prostatic channel with shaggy-looking mucosa. Urethral stricture has not been described in WG. In our patient, the stricture was probably due to prior instrumentation, although some uncertainty remains because urethral biopsies were not performed. The initial treatment of choice for WG of the prostate is medical. One report has indicated that trimethoprim-sulfamethoxazole was effective as the initial treatment of a case of WG involving the prostate; however, recurrence was noted during the 15th month of treatment.6 Cyclophosphamide and prednisolone are the usual first-line medical treatments for active WG, including those with prostatic disease. Surgical treatment can be indicated, and in one published series, transurethral resection of the prostate was performed in 15% of cases of WG with prostatic involvement.5 Patients with WG who develop prostatic symptoms should undergo prostate biopsy to exclude other pathologic findings. Individually tailored combinations of medical and surgical treatment are indicated under the supervision of a physician and urologist with appropriate experience. REFERENCES 1. Cotch MF, Hoffman GS, Yerg DE, et al: The epidemiology of Wegener’s granulomatosis: estimates of the five years period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum 39: 87–92, 1996. 2. Hoffman GS, Kerr GS, Leavitt RY, et al: Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 116: 488 – 498, 1992. 3. Stillwell TJ, De Remee RA, McDonald TJ, et al: Prostatic involvement in Wegener’s granulomatosis. J Urol 138: 1251– 1253, 1987. 4. Middleton G, Krap D, Lee E, et al: Wegener’s granulomatosis presenting as a lower back pain with prostatitis and urethral obstruction. J Rheumatol 21: 566 –569, 1994. 5. Khattak AQ, Nair M, Haqqani MT, et al: Wegener granulomatosis: prostatic involvement and recurrent urinary tract infections. BJU Int 84: 531–532, 1999. 6. Bray VJ, and Hasbargen JA: Prostatic involvement in Wegener’s granulomatosis. Am J Kidney Dis 17: 578 –580, 1991.
195.e27
WEGENER’S GRANULOMATOSIS INVOLVING PROSTATE K. A. GABER, N. G. RYLEY, JAMES P. MACDERMOTT,
AND
J. M. GOLDMAN
ABSTRACT We report a rare case of Wegener’s granulomatosis involving the prostate in a 28-year-old man. The initial medical treatment was effective, but surgical intervention was required to manage late complications. UROLOGY 66: 195e.26–195e.27, 2005. © 2005 Elsevier Inc.
W
egener’s granulomatosis (WG) is a necrotizing vasculitis of unknown etiology. It usually involves the upper airway, lung, and kidney and has an incidence rate of 3:100,000, divided equally between men and women.1 The first-line treatment of WG is medical; however, occasionally surgical management is indicated. We report a rare case of WG involving the prostate that required surgical intervention. CASE REPORT A 28-year-old man presented in February 1995 with cavitating lung lesions, hematuria, peripheral neuropathy, and a collapsed nasal septum. Antineutrophil cytoplasmic antibody was positive, and a nasal biopsy was consistent with vasculitis, confirming a diagnosis of WG. He was initially treated with prednisolone and cyclophosphamide, followed by azathioprine, with good clinical and biochemical responses. Treatment was ceased after 42 months, with the patient in remission. In August 2000, he presented with lethargy, dysuria, and marked perineal pain, with the subsequent development of urinary retention. On examination, he was febrile with suprapubic tenderness, and digital rectal examination revealed an enlarged, acutely tender prostate. Investigations showed a white blood cell count of 10.6 ⫻ 109 From the Department of Respiratory Medicine, Bristol Royal Infirmary, Bristol, United Kingdom; Departments of Histopathology and Urology, Torbay Hospital, Torquay, United Kingdom; and Heart and Lung Unit, Torbay Hospital, Torquay, United Kingdom Address for correspondence: K. A. Gaber, M.R.C.P., Department of Respiratory Medicine, Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW, United Kingdom. E-mail: khalidgbr@ doctors.org.uk Submitted: July 14, 2004, accepted (with revisions): January 24, 2005 © 2005 ELSEVIER INC. 195.e26
ALL RIGHTS RESERVED
cells/L (normal 4 to 10) and a raised C-reactive protein (49 mg/L, normal less than 3) and erythrocyte sedimentation rate (36 mm/hr, normal 1 to 5). His chest radiograph, urea and electrolyte tests, prostate-specific antigen level, and blood and urine cultures were normal. Transrectal ultrasound scans showed an enlarged prostate (about 70 g) and multiple hypoechoic lesions, consistent with prostatic abscesses. Magnetic resonance imaging of the pelvis confirmed prostatic enlargement, but revealed no other pathologic features. Transurethral drainage of the abscesses was performed, and ciprofloxacin 500 mg twice daily was started. The prostatic tissue was negative on conventional bacteriologic culture, Ziehl-Neelsen stain, and culture for acid-fast bacilli. The histologic examination revealed marked inflammation, with areas of eosinophilic necrosis surrounded by neutrophils, histiocytes, and scattered multinucleated giant cells. Vasculitis affecting vessels of variable type and caliber was present. These features were considered consistent with WG involving the prostate gland (Fig. 1). Immunologic investigation confirmed a positive antineutrophil cytoplasmic antibody with positive anti-proteinase 3 activity, which is known to occur in 75% to 90% of patients with active WG.2 The patient was treated with oral cyclophosphamide and prednisolone, with rapid improvement in his symptoms. Three months later, the antineutrophil cytoplasmic antibody test was negative, and azathioprine was substituted for cyclophosphamide. Six months later, the size of his prostate had decreased to 25 g, but new obstructive urinary symptoms had developed. Measurements of the urinary flow rate suggested marked obstruction, with a peak flow of 4.9 mL/s for a volume of 150 mL. Cystoscopy revealed a stricture in the bulbar urethra, and internal urethrotomy and dilation 0090-4295/05/$30.00 doi:10.1016/j.urology.2005.01.045
FIGURE 1. Prostate biopsy showing vessels (arrows) displaying fibrinoid change and neutrophil infiltration of their walls.
were performed. The stricture recurred, and he eventually required membranous urethroplasty. COMMENT Ninety percent of patients with WG develop upper and lower respiratory tract involvement, and 80% develop renal disease.2 It is uncommon for the skin, central nervous system, eyes, and the heart to be involved in WG and rare for the genitourinary tract to be involved. In 1987, Stillwell et al.3 reported that the prostate was involved in only 4 (2.3%) of 174 patients with WG reviewed. The presentation of WG involving the prostate is varied. In a review of 26 patients with prostatic involvement in WG, 8 (31%) had chronic obstructive urinary symptoms, 7 (27%) were asymptomatic, 5 (19%) presented with acute urinary retention, 5 (19%) had hematuria, and 1 (4%) had recurrent urinary tract infection.4 In that series, surgical intervention was unusual, and only 4 of the 26 patients underwent transurethral resection of the prostate.5 In patients with WG, the clinical examination may reveal an enlarged or indurated prostate. The cys-
UROLOGY 66 (1), 2005
toscopic appearances are of a diffusely abnormal prostatic channel with shaggy-looking mucosa. Urethral stricture has not been described in WG. In our patient, the stricture was probably due to prior instrumentation, although some uncertainty remains because urethral biopsies were not performed. The initial treatment of choice for WG of the prostate is medical. One report has indicated that trimethoprim-sulfamethoxazole was effective as the initial treatment of a case of WG involving the prostate; however, recurrence was noted during the 15th month of treatment.6 Cyclophosphamide and prednisolone are the usual first-line medical treatments for active WG, including those with prostatic disease. Surgical treatment can be indicated, and in one published series, transurethral resection of the prostate was performed in 15% of cases of WG with prostatic involvement.5 Patients with WG who develop prostatic symptoms should undergo prostate biopsy to exclude other pathologic findings. Individually tailored combinations of medical and surgical treatment are indicated under the supervision of a physician and urologist with appropriate experience. REFERENCES 1. Cotch MF, Hoffman GS, Yerg DE, et al: The epidemiology of Wegener’s granulomatosis: estimates of the five years period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum 39: 87–92, 1996. 2. Hoffman GS, Kerr GS, Leavitt RY, et al: Wegener’s granulomatosis: an analysis of 158 patients. Ann Intern Med 116: 488 – 498, 1992. 3. Stillwell TJ, De Remee RA, McDonald TJ, et al: Prostatic involvement in Wegener’s granulomatosis. J Urol 138: 1251– 1253, 1987. 4. Middleton G, Krap D, Lee E, et al: Wegener’s granulomatosis presenting as a lower back pain with prostatitis and urethral obstruction. J Rheumatol 21: 566 –569, 1994. 5. Khattak AQ, Nair M, Haqqani MT, et al: Wegener granulomatosis: prostatic involvement and recurrent urinary tract infections. BJU Int 84: 531–532, 1999. 6. Bray VJ, and Hasbargen JA: Prostatic involvement in Wegener’s granulomatosis. Am J Kidney Dis 17: 578 –580, 1991.
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