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Wegener’s Granulomatosis Involving the Prostate Gland: Report of a Case
THE JOURNAL OF UROLOGY
Vol. 96, Nov. Printed in U.S.A.
Copyright © 1966 by The Williams & Wilkins Co.
WEGENER'S GRANULOMATOSIS INVOLVING THE PROSTATE GLAND: REPORT OF A CASE PHILIP A. YALOWITZ, LAURENCE F. GREENE, SHELDON G. SHEPS M. RICHARD CARLIN
AND
From the Sections of Urology and Medicine, Mayo Clinic and Mayo Foundation and Mayo Graduate School of Medicine, University of Minnesota, Rochester, Minnesota; and the St. Joseph's Hospital, St. Louis, Missouri
Wegener's granulomatosis is an uncommon disease characterized by intractable rhinitis and sinusitis, nodular pulmonary lesions, generalized vasculitis and terminal uremia. Many organs may be involved.1 - 3 It is usually fatal within 6 months from the onset of the initial symptoms but may run a protracted course of 2 years or more. The pathologic changes include necrotizing granulomatous lesions of the upper, lower, or both parts of the respiratory tract, generalized focal necrotizing vasculitis involving arteries and veins and focal glomerulitis. Male and female patients of all ages are affected, but the disease occurs most commonly in the fourth or fifth decade of life. 4 Involvement of the prostate gland by this disease process is not unusual5 but massive disease of the organ heralding the onset of the fatal illness is uncommon. This paper reports a case in which the prostatic symptoms were dominant. CASE REPORT
A 45-year-old man came to the Mayo Clinic in December 1963 complaining of urinary obstructive symptoms and purulent nasal discharge with stuffiness, crusting and recent collapse of Accepted for publication December 2, 1965. Read at annual meeting of North Central Section, American Urological Association, Inc., Minneapolis, Minnesota, September 15-18, 1965. 1 Fahey, J. L., Leonard, E., Churg, J. and Godman, G. C.: Wegener's granulomatosis. Amer. J. Med., 17: 168-179, 1954. 2 McDonald, J.B. and Edwards, R. W.: "Wegener's granulomatosis"-a triad. J.A.M.A., 173: 1205-1209, 1960.
3 Harrison, T. R., Adams, R. D., Bennett, I. L., Jr., Resnik, W. H., Thorn, G. W. and Wintrobe, M. M.: Principles of Internal Medicine. New York: McGraw-Hill Book Company, Inc., 1962, edit. 4, vol. 2, pp. 1900--1901. 4 Godman, G. and Churg, J.: Wegener's granulomatosis: Pathology and review of the literature. Arch. Path., 58: 533-553, 1954. 6 Walton, E. W.: Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Brit. Med. J., 2: 265-270, 1958.
the bridge of the nose. He had noticed anorexia, malaise, lethargy, fever, and weight loss of 40 pounds. His illness began in July 1963 when he noticed a watery nasal discharge followed within a few days by severe, progressive, obstructive and irritative urinary symptoms. His physician reported enlargement, induration and tenderness of the prostate. In September 1963, transurethral prostatic resection was done and the urinary symptoms improved considerably but recurred within 6 weeks; it was necessary to resort to urethral catheter drainage. In the interim, a draining ulcerated area had developed in the right axilla, and the watery rhinorrhea progressed to a sanguinopurulent material with crusting, almost complete nasal obstruction and collapse of the bridge of the nose. Physical examination revealed a saddle deformity of the nose, which internally was filled with dark crusts, pus and a granulomatous mass. The nasal septum was entirely destroyed. Digital rectal examination disclosed a movable, boggy mass overlying the prostate gland which felt granular, firm and without perceptible borders. Hemoglobin concentration was 9.2 gm. per 100 ml. blood; the leukocyte count was 13,000/mm3 with a normal differential count. Results of several urinalyses were: grade 1 to 2 proteinuria (on basis of 1 to 4), grade 1 erythrocyturia (on basis of 1 to 5), grade 3 to 4 leukocyturia (on basis of 1 to 4), culture, Proteus. The thoracic roentgenogram showed a 1.5 cm. nodule in the right pulmonary base. The upper part of the urinary tract appeared to be normal on excretory urography. A retrograde urethrogram showed irregular dilation of the prostatic urethra (fig. 1). At cystoscopy, the major portion of the prostatic urethra was occupied by a large cavity lined with a considerable amount of necrotic material. A transurethral biopsy was interpreted as necrotizing prostatitis. Cultures for tuberculosis organisms and fungi were negative.
801
802
YALOWITZ AND ASSOCIATES
The diagnosis of W egener's granulomatosis was made; the patient returned home and died 3 weeks later. Shortly before death, his blood urea nitrogen concentration was 86 mg. per 100 ml. A short course of steroid therapy had resulted in marked relief of pain. Pathology. Antemortem: sections from the nasal cavity showed a marked inflammatory
reaction with a few poorly formed granulomas, vasculitis and fibrosis. Prostatic sections of a specimen taken in November 1963 contained necrotizing granuloma with vasculitis (fig. 2). Postmortem: there was necrosis of the mucous membrane of the hard and soft palates, complete destruction of the nasal septum, moderate pulmonary edema and a single crescentic nodule in
Fw. 1. Retrograde urethrogram; 2 views demonstrating irregular dilation of prostatic urethra
Fw. 2. Pros ta tic biopsy shows necrotizing granuloma. H & E X 120
WEGENER'S GRANULOMATOSIS IN PROSTATE
the right lower lobe, which contained creamy, yellowish white material in a central cavity. The parenchyma of the spleen was dark reddish black, necrotic, and semifluid. The kidneys were swollen with extensive gray and yellow necrotic areas. There were multiple, ,:onfluent, recent, gray infarcts and widespread thrombonecrosis of the renal parenchymal vessels. The prostate was represented by a thin, necrotic shell. Microscopic exarnination of the prostate revealed necrotizing suppuration ,vith a few areas of granuloma, extension to the bladder trigone and a focus of granulation tissue replacing the mucosa. The kidney sections showed massive fibrinoid necrosis of the arteries and arterioles, widespread infarction and proliferative glomerulitis. The pulmonary nodule contained a necrotic purulent exuProstatic involvement In Wegener's granulomatosis Case
1*· 2'
6
31 41 .5' (j8
7' 810 9"
101, 11 Present report
(yr)
27 34 50 38
G3 47 29 58 82 60 45
Urinary Symptoms (Clinical)
Gross hema1.uria None None "Non-specific prostatitis" Acute 11ri1rnry retention None None None Acute urinary retention None Prostat.ic obstruction and secondary infection
* This patient also had. granulomatous lesions of bladder and glomerulitis. Source of hematuria was not determined. 6 Fienberg, R.: N ecrotizing grarrnlomatosis and angiitis of the lnngs: With massive splenic necrosis and focal thrombotic granulomatous glomernlonephritis. Amer. J. Clin. Path., 23: 413-428, HJ53.
7 Fanger, H. and Hoffman, J\/L: Wegener's granulomatosis: A case report. J . U rol., 77: 78-81,
elate surrounded by granuloma with occasional giant cells. DISCUSSION
In 1958 vValt.on reported prostatic involvement in 7.4 per cent of cases of vVegener's gran. ulomatosis." V\Te have found a total of 11 caseR, including our own, in which the prostate was histologically involved. In 3 cases (ours in. eluded), the prostatic symptoms were striking; and were among the dominant features of the clinical picture (table). Involvement of other portions of the urogeni. tal traet has been reported. The seminal vesicle,; usually are involved when there are prostatic lesions i. G-s, 11 and the teste;; are affected about as frequently as the prostate. 1 , .s, 9 • rn, H \Ve found 3 cases in the literature wit.Ii involvement of the bladder wall, in addition to our own case in which the lesions extended into the tri· gone. 6 , 9 , 14 Vilanova and associates case in which there was incomplete nec:rosis of the prepuce and penis. 1 ·5 :\fassive involvement of the prostate gland a granulomatous disease process associated with severe lower urinary tract symptoms and cli.sseminated lesions is not unique to granulomatosis. The,,;e same features occur in allergic granulomatous prostatitis, described by Kelalis and associates. 16 This is uniformly fatal disease occurring in asthmatics The lesions found in the prostate consist of muJ. tiple stellate areas of fihrinoid necrosis surrounded by epithelioid cells and an intense eosinophilic cell infiltration. Similar lesions have been found in the lungs at postmortem exarnirw, .. tion. 16 The occurrence of disseminated lcsiorrn and the uniform fatality of allergic granuloma tous prostatitis provide striking similarities to the cases of vVegener's granulomatosis with massive involvement of the prostate gland. However, the two conditions are clearly different.
Atkins. J. P. and Eisman, S. IL Wegener's granulomatosis. Ann. Oto!., Hhin. & Laryng., 68:
13 Berman, D. A., Rydell, H.. E. and Eichenhoh, A.: Wegeuer's granuloma(.osis: A clinico .. pathologic study of fonr cases. Ann. Intern. Med., 59;
524-.547, 195\J. 9 Ring, C.
521-530, 19G3. 14 Tuhy, .J. K, Ma.mice, G. L. and Niles, N. R.. :
Hl.57. 8
Wegener's granulomatosis. Trans. OphthaL Soc. Zeal. 14: 87-101, 1962. 10 Beidleman, B.: Wegener's granulomatosis; prolonged therapy with large doses of steroids. J.A.M.A., 186: 827-830, 1963. 11 Friend, D. S.: Wegener's granulomatosis: A ease report, Arch. Intern. Med., 111: 703-709, 19G3. 12 Fisher, J. H.: Wegener's grannlomatosis: A review of three cases. Canad. l\1ed. Ass. J., 90: 10-14, 19(34.
Wegener's granulomatosis. Amer. J. J\led., 25:
G38-646, 1958. 15 Vilanova, X., Pifiol-Aguade, J. and Huecla, L. A.: Grannlome malin centro-facial avec lesions genitales et visc{,rales. Bull. Soc. fran~. dernrnt, et syph., 70: 462-470, 19G3. 16 Kelalis, P. P,, Harrison, E. CL, Jr. a.nd Greene, L. F.: Allerg;ic granulomas of the n,-no,...,,,n in asthmatics. J,A.M.A., 188: 963-\J67,
804
YALOWITZ AND ASSOCIATES
There is almost never a history of allergy in Wegener's granulomatosis, and microscopically the gram1lomas contain only an occasional eosinophil rather than a full field. The clinical triad of a necrotizing granulomatous process of the respiratory tract, generalized vasculitis involving arteries and veins and focal glomerulitis is quite distinct and characteristic of W egener's granulomatosis. The cause is unknown in both conditions. They both may be within a spectrum of related systemic diseases, but this remains to be resolved. Treatment of Wegener's granulomatosis has been ineffective. The use of antibiotics to control secondary infections, irradiation of localized lesions, and administration of nitrogen mustards have all been used but with little benefit. Beidleman reported apparent remissions and temporary arrest of the disease process with the administration of steroids10 and our patient had marked
relief of pain when steroids were given. However, the role of steroids in this unusual disease needs further evaluation. SUMMARY
We have reported a case of W egener's granulomatosis in which the prostatic symptoms were dominant. Massive involvement of the prostate gland by this unusual disease, heralding the onset of the illness, is extremely rare. ADDENDUM
After this paper was submitted, Mcllvanie reported 2 cases in which remissions of 12 and 22 months, respectively, were maintained by continuous administration of chlorambucil.17 17 Mcllvanie, S. K.: Wegener's granulomatosis. Successful treatment with chlorambucil. J .A.M.A., 197: 90-92, 1966.
Vol. 96, Nov. Printed in U.S.A.
Copyright © 1966 by The Williams & Wilkins Co.
WEGENER'S GRANULOMATOSIS INVOLVING THE PROSTATE GLAND: REPORT OF A CASE PHILIP A. YALOWITZ, LAURENCE F. GREENE, SHELDON G. SHEPS M. RICHARD CARLIN
AND
From the Sections of Urology and Medicine, Mayo Clinic and Mayo Foundation and Mayo Graduate School of Medicine, University of Minnesota, Rochester, Minnesota; and the St. Joseph's Hospital, St. Louis, Missouri
Wegener's granulomatosis is an uncommon disease characterized by intractable rhinitis and sinusitis, nodular pulmonary lesions, generalized vasculitis and terminal uremia. Many organs may be involved.1 - 3 It is usually fatal within 6 months from the onset of the initial symptoms but may run a protracted course of 2 years or more. The pathologic changes include necrotizing granulomatous lesions of the upper, lower, or both parts of the respiratory tract, generalized focal necrotizing vasculitis involving arteries and veins and focal glomerulitis. Male and female patients of all ages are affected, but the disease occurs most commonly in the fourth or fifth decade of life. 4 Involvement of the prostate gland by this disease process is not unusual5 but massive disease of the organ heralding the onset of the fatal illness is uncommon. This paper reports a case in which the prostatic symptoms were dominant. CASE REPORT
A 45-year-old man came to the Mayo Clinic in December 1963 complaining of urinary obstructive symptoms and purulent nasal discharge with stuffiness, crusting and recent collapse of Accepted for publication December 2, 1965. Read at annual meeting of North Central Section, American Urological Association, Inc., Minneapolis, Minnesota, September 15-18, 1965. 1 Fahey, J. L., Leonard, E., Churg, J. and Godman, G. C.: Wegener's granulomatosis. Amer. J. Med., 17: 168-179, 1954. 2 McDonald, J.B. and Edwards, R. W.: "Wegener's granulomatosis"-a triad. J.A.M.A., 173: 1205-1209, 1960.
3 Harrison, T. R., Adams, R. D., Bennett, I. L., Jr., Resnik, W. H., Thorn, G. W. and Wintrobe, M. M.: Principles of Internal Medicine. New York: McGraw-Hill Book Company, Inc., 1962, edit. 4, vol. 2, pp. 1900--1901. 4 Godman, G. and Churg, J.: Wegener's granulomatosis: Pathology and review of the literature. Arch. Path., 58: 533-553, 1954. 6 Walton, E. W.: Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Brit. Med. J., 2: 265-270, 1958.
the bridge of the nose. He had noticed anorexia, malaise, lethargy, fever, and weight loss of 40 pounds. His illness began in July 1963 when he noticed a watery nasal discharge followed within a few days by severe, progressive, obstructive and irritative urinary symptoms. His physician reported enlargement, induration and tenderness of the prostate. In September 1963, transurethral prostatic resection was done and the urinary symptoms improved considerably but recurred within 6 weeks; it was necessary to resort to urethral catheter drainage. In the interim, a draining ulcerated area had developed in the right axilla, and the watery rhinorrhea progressed to a sanguinopurulent material with crusting, almost complete nasal obstruction and collapse of the bridge of the nose. Physical examination revealed a saddle deformity of the nose, which internally was filled with dark crusts, pus and a granulomatous mass. The nasal septum was entirely destroyed. Digital rectal examination disclosed a movable, boggy mass overlying the prostate gland which felt granular, firm and without perceptible borders. Hemoglobin concentration was 9.2 gm. per 100 ml. blood; the leukocyte count was 13,000/mm3 with a normal differential count. Results of several urinalyses were: grade 1 to 2 proteinuria (on basis of 1 to 4), grade 1 erythrocyturia (on basis of 1 to 5), grade 3 to 4 leukocyturia (on basis of 1 to 4), culture, Proteus. The thoracic roentgenogram showed a 1.5 cm. nodule in the right pulmonary base. The upper part of the urinary tract appeared to be normal on excretory urography. A retrograde urethrogram showed irregular dilation of the prostatic urethra (fig. 1). At cystoscopy, the major portion of the prostatic urethra was occupied by a large cavity lined with a considerable amount of necrotic material. A transurethral biopsy was interpreted as necrotizing prostatitis. Cultures for tuberculosis organisms and fungi were negative.
801
802
YALOWITZ AND ASSOCIATES
The diagnosis of W egener's granulomatosis was made; the patient returned home and died 3 weeks later. Shortly before death, his blood urea nitrogen concentration was 86 mg. per 100 ml. A short course of steroid therapy had resulted in marked relief of pain. Pathology. Antemortem: sections from the nasal cavity showed a marked inflammatory
reaction with a few poorly formed granulomas, vasculitis and fibrosis. Prostatic sections of a specimen taken in November 1963 contained necrotizing granuloma with vasculitis (fig. 2). Postmortem: there was necrosis of the mucous membrane of the hard and soft palates, complete destruction of the nasal septum, moderate pulmonary edema and a single crescentic nodule in
Fw. 1. Retrograde urethrogram; 2 views demonstrating irregular dilation of prostatic urethra
Fw. 2. Pros ta tic biopsy shows necrotizing granuloma. H & E X 120
WEGENER'S GRANULOMATOSIS IN PROSTATE
the right lower lobe, which contained creamy, yellowish white material in a central cavity. The parenchyma of the spleen was dark reddish black, necrotic, and semifluid. The kidneys were swollen with extensive gray and yellow necrotic areas. There were multiple, ,:onfluent, recent, gray infarcts and widespread thrombonecrosis of the renal parenchymal vessels. The prostate was represented by a thin, necrotic shell. Microscopic exarnination of the prostate revealed necrotizing suppuration ,vith a few areas of granuloma, extension to the bladder trigone and a focus of granulation tissue replacing the mucosa. The kidney sections showed massive fibrinoid necrosis of the arteries and arterioles, widespread infarction and proliferative glomerulitis. The pulmonary nodule contained a necrotic purulent exuProstatic involvement In Wegener's granulomatosis Case
1*· 2'
6
31 41 .5' (j8
7' 810 9"
101, 11 Present report
(yr)
27 34 50 38
G3 47 29 58 82 60 45
Urinary Symptoms (Clinical)
Gross hema1.uria None None "Non-specific prostatitis" Acute 11ri1rnry retention None None None Acute urinary retention None Prostat.ic obstruction and secondary infection
* This patient also had. granulomatous lesions of bladder and glomerulitis. Source of hematuria was not determined. 6 Fienberg, R.: N ecrotizing grarrnlomatosis and angiitis of the lnngs: With massive splenic necrosis and focal thrombotic granulomatous glomernlonephritis. Amer. J. Clin. Path., 23: 413-428, HJ53.
7 Fanger, H. and Hoffman, J\/L: Wegener's granulomatosis: A case report. J . U rol., 77: 78-81,
elate surrounded by granuloma with occasional giant cells. DISCUSSION
In 1958 vValt.on reported prostatic involvement in 7.4 per cent of cases of vVegener's gran. ulomatosis." V\Te have found a total of 11 caseR, including our own, in which the prostate was histologically involved. In 3 cases (ours in. eluded), the prostatic symptoms were striking; and were among the dominant features of the clinical picture (table). Involvement of other portions of the urogeni. tal traet has been reported. The seminal vesicle,; usually are involved when there are prostatic lesions i. G-s, 11 and the teste;; are affected about as frequently as the prostate. 1 , .s, 9 • rn, H \Ve found 3 cases in the literature wit.Ii involvement of the bladder wall, in addition to our own case in which the lesions extended into the tri· gone. 6 , 9 , 14 Vilanova and associates case in which there was incomplete nec:rosis of the prepuce and penis. 1 ·5 :\fassive involvement of the prostate gland a granulomatous disease process associated with severe lower urinary tract symptoms and cli.sseminated lesions is not unique to granulomatosis. The,,;e same features occur in allergic granulomatous prostatitis, described by Kelalis and associates. 16 This is uniformly fatal disease occurring in asthmatics The lesions found in the prostate consist of muJ. tiple stellate areas of fihrinoid necrosis surrounded by epithelioid cells and an intense eosinophilic cell infiltration. Similar lesions have been found in the lungs at postmortem exarnirw, .. tion. 16 The occurrence of disseminated lcsiorrn and the uniform fatality of allergic granuloma tous prostatitis provide striking similarities to the cases of vVegener's granulomatosis with massive involvement of the prostate gland. However, the two conditions are clearly different.
Atkins. J. P. and Eisman, S. IL Wegener's granulomatosis. Ann. Oto!., Hhin. & Laryng., 68:
13 Berman, D. A., Rydell, H.. E. and Eichenhoh, A.: Wegeuer's granuloma(.osis: A clinico .. pathologic study of fonr cases. Ann. Intern. Med., 59;
524-.547, 195\J. 9 Ring, C.
521-530, 19G3. 14 Tuhy, .J. K, Ma.mice, G. L. and Niles, N. R.. :
Hl.57. 8
Wegener's granulomatosis. Trans. OphthaL Soc. Zeal. 14: 87-101, 1962. 10 Beidleman, B.: Wegener's granulomatosis; prolonged therapy with large doses of steroids. J.A.M.A., 186: 827-830, 1963. 11 Friend, D. S.: Wegener's granulomatosis: A ease report, Arch. Intern. Med., 111: 703-709, 19G3. 12 Fisher, J. H.: Wegener's grannlomatosis: A review of three cases. Canad. l\1ed. Ass. J., 90: 10-14, 19(34.
Wegener's granulomatosis. Amer. J. J\led., 25:
G38-646, 1958. 15 Vilanova, X., Pifiol-Aguade, J. and Huecla, L. A.: Grannlome malin centro-facial avec lesions genitales et visc{,rales. Bull. Soc. fran~. dernrnt, et syph., 70: 462-470, 19G3. 16 Kelalis, P. P,, Harrison, E. CL, Jr. a.nd Greene, L. F.: Allerg;ic granulomas of the n,-no,...,,,n in asthmatics. J,A.M.A., 188: 963-\J67,
804
YALOWITZ AND ASSOCIATES
There is almost never a history of allergy in Wegener's granulomatosis, and microscopically the gram1lomas contain only an occasional eosinophil rather than a full field. The clinical triad of a necrotizing granulomatous process of the respiratory tract, generalized vasculitis involving arteries and veins and focal glomerulitis is quite distinct and characteristic of W egener's granulomatosis. The cause is unknown in both conditions. They both may be within a spectrum of related systemic diseases, but this remains to be resolved. Treatment of Wegener's granulomatosis has been ineffective. The use of antibiotics to control secondary infections, irradiation of localized lesions, and administration of nitrogen mustards have all been used but with little benefit. Beidleman reported apparent remissions and temporary arrest of the disease process with the administration of steroids10 and our patient had marked
relief of pain when steroids were given. However, the role of steroids in this unusual disease needs further evaluation. SUMMARY
We have reported a case of W egener's granulomatosis in which the prostatic symptoms were dominant. Massive involvement of the prostate gland by this unusual disease, heralding the onset of the illness, is extremely rare. ADDENDUM
After this paper was submitted, Mcllvanie reported 2 cases in which remissions of 12 and 22 months, respectively, were maintained by continuous administration of chlorambucil.17 17 Mcllvanie, S. K.: Wegener's granulomatosis. Successful treatment with chlorambucil. J .A.M.A., 197: 90-92, 1966.