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Polyarteritis Nodosa Presenting as Acute Orchitis: A Case Report and Review of the Literature
0022-5347/93/1495-1139$03.00/0 Vol. 149,
THE JOURNAL O? UROLOGY
Copyright ©
1993
by AMERICAN
UROLOGICAL ASSOCIATION, INC.
POLYARTERITIS NODOSA PRESENTING AS ACUTE ORCHITIS: A CASE REPORT AND REVIEW OF THE LITERATURE JOEL M. H. TEICHMAN, ROBERT F. MATTREY, ALAN M. DEMBY
AND
JOSEPH D. SCHMIDT
From the Division of Urology and Department of Radiology, University of California, San Diego Medical Center, San Diego, California
ABSTRACT
We report a case of polyarteritis nodosa initially presenting as acute orchitis and review 4 similar reports in the literature. Of the cases 2 were diagnosed preoperatively. In 2 cases in which immunological markers were measured abnormal values were obtained. All 5 cases required biopsy for diagnosis. KEY WORDS: testicular neoplasms, orchitis, polyarteritis nodosa
The lesions of polyarteritis nodosa are widespread throughout the body. Early manifestations of polyarteritis nodosa are typically nonspecific, including fever, weakness, anorexia, weight loss, myalgias and arthralgias. 1 Specific organ systems involved early are gastrointestinal and renal. 2 We present an unusual case in which the initial manifestations of polyarteritis nodosa simulated a febrile episode of epididymo-orchitis. CASE REPORT
A 55-year-old man presented to the emergency room with a 4-day history of spiking temperatures to 104F and left hemiscrotal aching. He denied any voiding complaints. Medical history was significant for previous right hydrocele repair, transurethral resection of the prostate for benign prostatic hyperplasia and right nephrectomy 5 years ago for stage A (T2) renal cell carcinoma. There was no recurrent disease at followup. He also had had keratoconjunctivitis sicca (primary Sjogren's syndrome), which had resolved 10 years ago. No subsequent immunological sequela ensued. Physical examination showed a temperature of 102F. Bilateral inguinal lymph nodes were mildly tender. The penis was circumcised without discharge. The right hemiscrotum was unremarkable. The left hemiscrotum was warm, with mild tenderness of the superior pole of the testis and the globus major of the epididymis. This tenderness seemed to exceed the scant physical findings. The prostate was unremarkable. Urine dipstick and microscopy studies showed 1+ proteinuria and 5 to 10 red blood cells per high power field, with scant white blood cells and no bacteria present. Urine and blood cultures were negative. Serum white blood count was 8,100/ mm. 3 (normal 4,800 to 10,800) with no left shift. Hematocrit was 38.5% (normal 42 to 52%). Expressed prostatic secretions were unremarkable. Abdominal computerized tomography with intravenous contrast medium done 2 days earlier for surveillance of renal cell carcinoma, after the onset of the acute illness, was unchanged from baseline. Scrotal ultrasound showed a large left hydrocele and mild enlargement of the left testis relative to the right testicle with normal intratesticular architecture,3 Since the left hemiscrotum was the only apparent infectious focus, the patient was hospitalized for presumptive epididymo-orchitis. Intravenous ampicillin and gentamicin were started. The temperature continued to spike to 104F so intravenous ceftazidine was added. There was no change after 5 days. Scrotal magnetic resonance imaging showed several poorly
defined, low signal foci consistent with infarction. 3 Testicular vasculitis was considered and immunological markers were measured. Erythrocyte sedimentation rate was 120 mm. per hour (normal less than 20), complement 3 was 168 mg. % (normal 127 to 156) and complement 4 was 52 mg. % (normal 15 to 45). A trans scrotal biopsy of the testis was performed. Grossly, the testis appeared bluish and some calcifications were noted on the tunica albuginea. Histopathological examination demonstrated polyarteritis nodosa. The nephrectomy specimen from 5 years previously was reviewed and no vasculitis was present. Antibiotics were discontinued and corticosteroids were begun. The pain resolved rapidly, and the patient was maintained and tapered off corticosteroids after 6 months. He remained asymptomatic at 22 months. DISCUSSION
The pathological entity of polyarteritis nodosa was first recognized by von Rokitansky in 1852 but Kussmaul and Maier coined the term periarteritis nodosa in 1866. 1 Ferrari noted that the pathological changes affect all 3 arterial layers and appropriately suggested the terminology polyarteritis nodosa in 1903. Fauci and Leavitt classified polyarteritis nodosa as a variant of systemic necrotizing vasculitis that itself falls under the broader heading of vasculitis. 2 Polyarteritis nodosa is a necrotizing vasculitis of medium and sometimes small-sized muscular arteries. 4 Because the media and internal elastic lamella are predominantly affected, aneurysm formation is frequent.5 Arterial bifurcations are often affected. The histopathological findings may be seen in any 1 or combination of 4 stages: degeneration, acute inflammation, granulation and end stage or scar. This multisystem disease has a predilection for renal and visceral involvement with pulmonary sparing. 6 Autopsy studies demonstrate that the kid-
Accepted for publication September 4, 1992. Read at annual meeting of Western Section, American Urological Association, Vancouver, British Columbia, Canada, July 28-August 1, 1991. Supported by National Institutes of Health Grant 1R01DK39239 and the Medical Research Service of the Veterans Administration, Urological Research and Education Foundation. 1139
Clinical profile and manifestations in patients with classical polyarteritis nodosa Clinical Parameter General considerations: Mean age (yrs.) Male:female ratio Fever (%) Wt.loss (%) Organ involvement (%): Kidney Musculoskeletal system Hypertension Peripheral nerves Gastrointestinal tract Skin Heart Central nervous system Data obtained from Cupps and Fauci. 9
Value 45 2.5:1 71 54 70 64 54 51 44 43
36 23
1140
TEICHMAN AND ASSOCIATES
neys are the most frequently involved site. 5 ,6 The male-tofemale ratio is 2.5:1. Men in the fifth decade are most commonly affected. 7,8 Typical presenting symptoms are nonspecific (see table).9 Genitourinary involvement is the rule. Renal parenchymal, vascular and hypertensive disease may be secondary to polyarteritis nodosa. Unilateral or bilateral ureteral involvement and obstruction from polyarteritis nodosa have been described. lo-13 Testicular mass suspicious for tumor has proved to be polyarteritis nodosa.1 4,15 Epididymitis has also been found secondary to polyarteritis nodosa.1 6-l8 Autopsy series demonstrate testicular polyarteritis nodosa in 38 to 86% of cases.6,19 However, the testis is less commonly symptomatic. 20 Even less common, the testis is the initial site of polyarteritis nodosa. To our knowledge, our case represents only the fifth report of histologically verified polyarteritis nodosa manifesting initially as acute orchitis without previous or concomitant disease. 2l,22 These 5 cases are reviewed and 2 were considered to be bacterial infectious processes. All 5 cases had negative cultures. One patient was treated unsuccessfully with antibiotics. In 2 of the 5 cases the correct diagnosis was considered preoperatively. Abnormal serum immunological markers were confirmed in 2 of 2 cases. Histopathological examination was required for diagnostic confirmation in all 5 cases. The most common cause of death from polyarteritis nodosa is renal disease, accounting for 45% of the attributable mortality rate. Therefore it is essential that polyarteritis nodosa be diagnosed so that appropriate treatment may be started. In multisystem polyarteritis nodosa the 5-year survival rate for untreated patients is 13% or less. 23 ,24 With corticosteroids survival improves to 48% and up to 80% with cytotoxic agents combined with corticosteroids. 9,20 It is interesting that our patient had a remote history of another autoimmune disorder, that is Sjogren's syndrome. Sjogren's syndrome is associated with numerous clinical problems besides sicca complex. 25 Approximately one-quarter of the patients have hyper-a-globulinemia, which may lead to glomerulonephritis, nephrocalcinosis, nephrogenic diabetes insipidus or renal tubular disorders.26 However, testis involvement is not a feature of Sjogren's syndrome. Another autoimmune disorder to consider is HenochSchonlein's purpura. Henoch-Schonlein's purpura is a hypersensitivity vasculitis that commonly affects the skin, gastrointestinal tract, joints and kidneys usually in children. 2 Rarely Henoch-Schonlein's purpura involves the testis and may be difficult to distinguish from testicular torsion. 27 The histopathology of testicular Henoch-Schonlein's purpura is diffuse interstitial hemorrhage with marked inflammatory reaction, focal necrosis and leukocytic infiltration. 28 ,29 HenochSchonlein's purpura is diagnosed easily on the basis of obvious skin purpura. Since Henoch -Schonlein's purpura resolves spontaneously, a conservative approach to the acute scrotum in Henoch-Schonlein's purpura seems to be warranted. 30 In conclusion, testicular vasculitis is not uncommon, although rarely symptomatic. The diagnosis should be considered in any patient with known polyarteritis nodosa or in any patient with testicular features considered atypical for an infectious process. Since the mortality rate for untreated polyarteritis nodosa is high, it is mandatory that a testis biopsy be performed for diagnosis and to guide therapy. REFERENCES
1. Kussmaul, A. and Maier, R.: Uber eine bisher neiht beschriebene eigenthumliche Arterienerkrankung die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellahmung einheght. Deutch. Arch. Klin, Med., 1: 484, 1866. 2. Fauci, A. S. and Leavitt, R. Y.: Vasculitis. In: Arthritis and Allied
3. 4. 5. 6. 7.
8. 9. 10. 11. 12. 13. 14. 15.
16. 17. 18. 19. 20. 21. 22. 23. 24. 25.
26.
27. 28. 29. 30.
Conditions: A Textbook of Rheumatology, 11th ed. Edited by D. J. McCarty. Philadelphia: Lea & Febiger, chapt. 74, pp. 1166, 1989. Hayward, I., Trambert, M. A., Mattrey, R. F., Saltzstein, S. L. and Demby, A. M.: Imaging of vasculitis of the testis. J. Comput. Assist. Tomogr., 15: 502, 1991. Arkin, A.: A clinical and pathological study of periarteritis nodosa. Arner. J. Path., 6: 401, 1930. Damjanov, I.: Pathology quiz. Res. Staff Phys., 36: 79, November 1990. Patalano, V. J. and Sommers, S. C.: Biopsy diagnosis of periarteritis nodosa. Arch. Path., 72: 1, 1961. Fauci, A. S.: The vasculitis syndromes. In: Harrison's Principles of Internal Medicine, 11th ed. Edited by E. Braunwald, K. I. Isselbacher, R. G. Petersdorf, J. D. Wilson, J. B. Martin and A. S. Fauci. New York: McGraw-Hill Book Co., part 8, chapt. 269, pp. 1438-1444, 1987. Travers, R. L., Allison, D. J., Brettie, R. P. and Hughes, G. R.: Polyarteritis nodosa: a clinical and angiographic analysis of 17 cases. Semin. Arthr. Rheum., 8: 184, 1979. Cupps, T. R. and Fauci, A. S.: The Vasculitides. Philadelphia: W. B. Saunders Co., 1981. Samellas, W., Bellonias, E. and Papacharalampous, N.: Polyarteritis nodosa: ureteral involvement. J. Urol., 105: 186, 1971. Glanz, I. and Griinebaum, M.: Ureteral changes in polyarteritis nodosa as seen during excretory urography. J. Urol., 116: 731, 1976. Fisher, R. S. and Howard, H. H.: Unusual ureterograms in a case of periarteritis nodosa. J. Urol., 60: 398, 1948. Melin, J. P., Lemaire, P., Birembaut, P., Aubert, L., Cohen, J., Lardennois, B. and Chanard, J.: Polyarteritis nodosa with bilateral ureteric involvement. Nephron, 32: 87, 1982. Belville, W. D., Insalaco, S. J., Dresner, M. L. and Buck, A. S.: Benign testis tumors. J. Urol., 128: 1198, 1982. Huisman, T. K., Collins, W. T., Jr. and Voulgarakis, G. R.: Polyarteritis nodosa masquerading as a primary testicular neoplasm: a case report and review of the literature. J. Urol., 144: 1236, 1990. Wright, L. F. and Bicknell, S. L.: Systemic necrotizing vasculitis presenting as epididymitis. J. Urol., 136: 1094, 1986. McLean, N. R. and Burnett, R. A.: Polyarteritis nodosa of epididymis. Urology, 21: 70, 1983. Rov, J. B. Hamblin, D. W. and Brown, C. H.: Periarteritis nodosa of epididymis. Urology, 10: 62, 1977. Monckeberg, J. G.: Uber periarteritis nodosa. Beitr. Path. Anat., 38: 101, 1905. Frohnert, P. P. and Sheps, S. G.: Long-term follow-up study of periarteritis nodosa. Amer. J. Med., 43: 8, 1967. Lee, L. M., Moloney, P. J., Wong, H. C. G., Magil, A. B. and McLoughlin, M. G.: Testicular pain: an unusual presentation of polyarteritis nodosa. J. Urol., 129: 1243, 1983. Shurbaji, M. S. and Epstein, J.I.: Testicular vasculitis: implications for systemic disease. Hum. Path., 19: 186,1988. Rose, G. A. and Spencer, H.: Polyarteritis nodosa. Quart. J. Med., 26: 43, 1957. Rose, G. A.: The natural history of polyarteritis. Brit. Med. J., 2: 1148,1957. Talal N.: Sjogren's syndrome and connective tissue diseases associated with other immunologic disorders. In: Arthritis and Allied Conditions: A Textbook of Rheumatology, 11th ed. Edited by D. J. McCarty. Philadelphia: Lea & Febiger, chapt. 76, pp. 11971213,1989. Whaley, K. and Alspaugh, M. A.: Sjogren's syndrome. In: Textbook of Rheumatology, 3rd ed. Edited by W. N. Kelley, E. D. Harris, Jr., S. Ruddy and C. B. Sledge. Philadelphia: W. B. Saunders Co., chapt. 58, pp. 999-1019, 1989. Sahn, D. J. and Schwartz, A. D.: SchOnlein-Henoch syndrome: observations on some atypical clinical presentations. Pediatrics, 49: 614, 1972. O'Regan, S. and Robitaille, P.: Orchitis mimicking testicular torsion in Henoch-Schonlein's purpura. J. Urol., 126: 834, 1981. Mikuz, G., Hofstiidter, F. and Hager, J.: Testis involvement in Schlonlein-Henoch purpura. Path. Res. Pract., 165: 323, 1979. Khan, A. U., Williams, T. H. and Malek, R. S.: Acute scrotal swelling in Henoch-SchOnlein syndrome. Urology, 10: 139, 1977.
THE JOURNAL O? UROLOGY
Copyright ©
1993
by AMERICAN
UROLOGICAL ASSOCIATION, INC.
POLYARTERITIS NODOSA PRESENTING AS ACUTE ORCHITIS: A CASE REPORT AND REVIEW OF THE LITERATURE JOEL M. H. TEICHMAN, ROBERT F. MATTREY, ALAN M. DEMBY
AND
JOSEPH D. SCHMIDT
From the Division of Urology and Department of Radiology, University of California, San Diego Medical Center, San Diego, California
ABSTRACT
We report a case of polyarteritis nodosa initially presenting as acute orchitis and review 4 similar reports in the literature. Of the cases 2 were diagnosed preoperatively. In 2 cases in which immunological markers were measured abnormal values were obtained. All 5 cases required biopsy for diagnosis. KEY WORDS: testicular neoplasms, orchitis, polyarteritis nodosa
The lesions of polyarteritis nodosa are widespread throughout the body. Early manifestations of polyarteritis nodosa are typically nonspecific, including fever, weakness, anorexia, weight loss, myalgias and arthralgias. 1 Specific organ systems involved early are gastrointestinal and renal. 2 We present an unusual case in which the initial manifestations of polyarteritis nodosa simulated a febrile episode of epididymo-orchitis. CASE REPORT
A 55-year-old man presented to the emergency room with a 4-day history of spiking temperatures to 104F and left hemiscrotal aching. He denied any voiding complaints. Medical history was significant for previous right hydrocele repair, transurethral resection of the prostate for benign prostatic hyperplasia and right nephrectomy 5 years ago for stage A (T2) renal cell carcinoma. There was no recurrent disease at followup. He also had had keratoconjunctivitis sicca (primary Sjogren's syndrome), which had resolved 10 years ago. No subsequent immunological sequela ensued. Physical examination showed a temperature of 102F. Bilateral inguinal lymph nodes were mildly tender. The penis was circumcised without discharge. The right hemiscrotum was unremarkable. The left hemiscrotum was warm, with mild tenderness of the superior pole of the testis and the globus major of the epididymis. This tenderness seemed to exceed the scant physical findings. The prostate was unremarkable. Urine dipstick and microscopy studies showed 1+ proteinuria and 5 to 10 red blood cells per high power field, with scant white blood cells and no bacteria present. Urine and blood cultures were negative. Serum white blood count was 8,100/ mm. 3 (normal 4,800 to 10,800) with no left shift. Hematocrit was 38.5% (normal 42 to 52%). Expressed prostatic secretions were unremarkable. Abdominal computerized tomography with intravenous contrast medium done 2 days earlier for surveillance of renal cell carcinoma, after the onset of the acute illness, was unchanged from baseline. Scrotal ultrasound showed a large left hydrocele and mild enlargement of the left testis relative to the right testicle with normal intratesticular architecture,3 Since the left hemiscrotum was the only apparent infectious focus, the patient was hospitalized for presumptive epididymo-orchitis. Intravenous ampicillin and gentamicin were started. The temperature continued to spike to 104F so intravenous ceftazidine was added. There was no change after 5 days. Scrotal magnetic resonance imaging showed several poorly
defined, low signal foci consistent with infarction. 3 Testicular vasculitis was considered and immunological markers were measured. Erythrocyte sedimentation rate was 120 mm. per hour (normal less than 20), complement 3 was 168 mg. % (normal 127 to 156) and complement 4 was 52 mg. % (normal 15 to 45). A trans scrotal biopsy of the testis was performed. Grossly, the testis appeared bluish and some calcifications were noted on the tunica albuginea. Histopathological examination demonstrated polyarteritis nodosa. The nephrectomy specimen from 5 years previously was reviewed and no vasculitis was present. Antibiotics were discontinued and corticosteroids were begun. The pain resolved rapidly, and the patient was maintained and tapered off corticosteroids after 6 months. He remained asymptomatic at 22 months. DISCUSSION
The pathological entity of polyarteritis nodosa was first recognized by von Rokitansky in 1852 but Kussmaul and Maier coined the term periarteritis nodosa in 1866. 1 Ferrari noted that the pathological changes affect all 3 arterial layers and appropriately suggested the terminology polyarteritis nodosa in 1903. Fauci and Leavitt classified polyarteritis nodosa as a variant of systemic necrotizing vasculitis that itself falls under the broader heading of vasculitis. 2 Polyarteritis nodosa is a necrotizing vasculitis of medium and sometimes small-sized muscular arteries. 4 Because the media and internal elastic lamella are predominantly affected, aneurysm formation is frequent.5 Arterial bifurcations are often affected. The histopathological findings may be seen in any 1 or combination of 4 stages: degeneration, acute inflammation, granulation and end stage or scar. This multisystem disease has a predilection for renal and visceral involvement with pulmonary sparing. 6 Autopsy studies demonstrate that the kid-
Accepted for publication September 4, 1992. Read at annual meeting of Western Section, American Urological Association, Vancouver, British Columbia, Canada, July 28-August 1, 1991. Supported by National Institutes of Health Grant 1R01DK39239 and the Medical Research Service of the Veterans Administration, Urological Research and Education Foundation. 1139
Clinical profile and manifestations in patients with classical polyarteritis nodosa Clinical Parameter General considerations: Mean age (yrs.) Male:female ratio Fever (%) Wt.loss (%) Organ involvement (%): Kidney Musculoskeletal system Hypertension Peripheral nerves Gastrointestinal tract Skin Heart Central nervous system Data obtained from Cupps and Fauci. 9
Value 45 2.5:1 71 54 70 64 54 51 44 43
36 23
1140
TEICHMAN AND ASSOCIATES
neys are the most frequently involved site. 5 ,6 The male-tofemale ratio is 2.5:1. Men in the fifth decade are most commonly affected. 7,8 Typical presenting symptoms are nonspecific (see table).9 Genitourinary involvement is the rule. Renal parenchymal, vascular and hypertensive disease may be secondary to polyarteritis nodosa. Unilateral or bilateral ureteral involvement and obstruction from polyarteritis nodosa have been described. lo-13 Testicular mass suspicious for tumor has proved to be polyarteritis nodosa.1 4,15 Epididymitis has also been found secondary to polyarteritis nodosa.1 6-l8 Autopsy series demonstrate testicular polyarteritis nodosa in 38 to 86% of cases.6,19 However, the testis is less commonly symptomatic. 20 Even less common, the testis is the initial site of polyarteritis nodosa. To our knowledge, our case represents only the fifth report of histologically verified polyarteritis nodosa manifesting initially as acute orchitis without previous or concomitant disease. 2l,22 These 5 cases are reviewed and 2 were considered to be bacterial infectious processes. All 5 cases had negative cultures. One patient was treated unsuccessfully with antibiotics. In 2 of the 5 cases the correct diagnosis was considered preoperatively. Abnormal serum immunological markers were confirmed in 2 of 2 cases. Histopathological examination was required for diagnostic confirmation in all 5 cases. The most common cause of death from polyarteritis nodosa is renal disease, accounting for 45% of the attributable mortality rate. Therefore it is essential that polyarteritis nodosa be diagnosed so that appropriate treatment may be started. In multisystem polyarteritis nodosa the 5-year survival rate for untreated patients is 13% or less. 23 ,24 With corticosteroids survival improves to 48% and up to 80% with cytotoxic agents combined with corticosteroids. 9,20 It is interesting that our patient had a remote history of another autoimmune disorder, that is Sjogren's syndrome. Sjogren's syndrome is associated with numerous clinical problems besides sicca complex. 25 Approximately one-quarter of the patients have hyper-a-globulinemia, which may lead to glomerulonephritis, nephrocalcinosis, nephrogenic diabetes insipidus or renal tubular disorders.26 However, testis involvement is not a feature of Sjogren's syndrome. Another autoimmune disorder to consider is HenochSchonlein's purpura. Henoch-Schonlein's purpura is a hypersensitivity vasculitis that commonly affects the skin, gastrointestinal tract, joints and kidneys usually in children. 2 Rarely Henoch-Schonlein's purpura involves the testis and may be difficult to distinguish from testicular torsion. 27 The histopathology of testicular Henoch-Schonlein's purpura is diffuse interstitial hemorrhage with marked inflammatory reaction, focal necrosis and leukocytic infiltration. 28 ,29 HenochSchonlein's purpura is diagnosed easily on the basis of obvious skin purpura. Since Henoch -Schonlein's purpura resolves spontaneously, a conservative approach to the acute scrotum in Henoch-Schonlein's purpura seems to be warranted. 30 In conclusion, testicular vasculitis is not uncommon, although rarely symptomatic. The diagnosis should be considered in any patient with known polyarteritis nodosa or in any patient with testicular features considered atypical for an infectious process. Since the mortality rate for untreated polyarteritis nodosa is high, it is mandatory that a testis biopsy be performed for diagnosis and to guide therapy. REFERENCES
1. Kussmaul, A. and Maier, R.: Uber eine bisher neiht beschriebene eigenthumliche Arterienerkrankung die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellahmung einheght. Deutch. Arch. Klin, Med., 1: 484, 1866. 2. Fauci, A. S. and Leavitt, R. Y.: Vasculitis. In: Arthritis and Allied
3. 4. 5. 6. 7.
8. 9. 10. 11. 12. 13. 14. 15.
16. 17. 18. 19. 20. 21. 22. 23. 24. 25.
26.
27. 28. 29. 30.
Conditions: A Textbook of Rheumatology, 11th ed. Edited by D. J. McCarty. Philadelphia: Lea & Febiger, chapt. 74, pp. 1166, 1989. Hayward, I., Trambert, M. A., Mattrey, R. F., Saltzstein, S. L. and Demby, A. M.: Imaging of vasculitis of the testis. J. Comput. Assist. Tomogr., 15: 502, 1991. Arkin, A.: A clinical and pathological study of periarteritis nodosa. Arner. J. Path., 6: 401, 1930. Damjanov, I.: Pathology quiz. Res. Staff Phys., 36: 79, November 1990. Patalano, V. J. and Sommers, S. C.: Biopsy diagnosis of periarteritis nodosa. Arch. Path., 72: 1, 1961. Fauci, A. S.: The vasculitis syndromes. In: Harrison's Principles of Internal Medicine, 11th ed. Edited by E. Braunwald, K. I. Isselbacher, R. G. Petersdorf, J. D. Wilson, J. B. Martin and A. S. Fauci. New York: McGraw-Hill Book Co., part 8, chapt. 269, pp. 1438-1444, 1987. Travers, R. L., Allison, D. J., Brettie, R. P. and Hughes, G. R.: Polyarteritis nodosa: a clinical and angiographic analysis of 17 cases. Semin. Arthr. Rheum., 8: 184, 1979. Cupps, T. R. and Fauci, A. S.: The Vasculitides. Philadelphia: W. B. Saunders Co., 1981. Samellas, W., Bellonias, E. and Papacharalampous, N.: Polyarteritis nodosa: ureteral involvement. J. Urol., 105: 186, 1971. Glanz, I. and Griinebaum, M.: Ureteral changes in polyarteritis nodosa as seen during excretory urography. J. Urol., 116: 731, 1976. Fisher, R. S. and Howard, H. H.: Unusual ureterograms in a case of periarteritis nodosa. J. Urol., 60: 398, 1948. Melin, J. P., Lemaire, P., Birembaut, P., Aubert, L., Cohen, J., Lardennois, B. and Chanard, J.: Polyarteritis nodosa with bilateral ureteric involvement. Nephron, 32: 87, 1982. Belville, W. D., Insalaco, S. J., Dresner, M. L. and Buck, A. S.: Benign testis tumors. J. Urol., 128: 1198, 1982. Huisman, T. K., Collins, W. T., Jr. and Voulgarakis, G. R.: Polyarteritis nodosa masquerading as a primary testicular neoplasm: a case report and review of the literature. J. Urol., 144: 1236, 1990. Wright, L. F. and Bicknell, S. L.: Systemic necrotizing vasculitis presenting as epididymitis. J. Urol., 136: 1094, 1986. McLean, N. R. and Burnett, R. A.: Polyarteritis nodosa of epididymis. Urology, 21: 70, 1983. Rov, J. B. Hamblin, D. W. and Brown, C. H.: Periarteritis nodosa of epididymis. Urology, 10: 62, 1977. Monckeberg, J. G.: Uber periarteritis nodosa. Beitr. Path. Anat., 38: 101, 1905. Frohnert, P. P. and Sheps, S. G.: Long-term follow-up study of periarteritis nodosa. Amer. J. Med., 43: 8, 1967. Lee, L. M., Moloney, P. J., Wong, H. C. G., Magil, A. B. and McLoughlin, M. G.: Testicular pain: an unusual presentation of polyarteritis nodosa. J. Urol., 129: 1243, 1983. Shurbaji, M. S. and Epstein, J.I.: Testicular vasculitis: implications for systemic disease. Hum. Path., 19: 186,1988. Rose, G. A. and Spencer, H.: Polyarteritis nodosa. Quart. J. Med., 26: 43, 1957. Rose, G. A.: The natural history of polyarteritis. Brit. Med. J., 2: 1148,1957. Talal N.: Sjogren's syndrome and connective tissue diseases associated with other immunologic disorders. In: Arthritis and Allied Conditions: A Textbook of Rheumatology, 11th ed. Edited by D. J. McCarty. Philadelphia: Lea & Febiger, chapt. 76, pp. 11971213,1989. Whaley, K. and Alspaugh, M. A.: Sjogren's syndrome. In: Textbook of Rheumatology, 3rd ed. Edited by W. N. Kelley, E. D. Harris, Jr., S. Ruddy and C. B. Sledge. Philadelphia: W. B. Saunders Co., chapt. 58, pp. 999-1019, 1989. Sahn, D. J. and Schwartz, A. D.: SchOnlein-Henoch syndrome: observations on some atypical clinical presentations. Pediatrics, 49: 614, 1972. O'Regan, S. and Robitaille, P.: Orchitis mimicking testicular torsion in Henoch-Schonlein's purpura. J. Urol., 126: 834, 1981. Mikuz, G., Hofstiidter, F. and Hager, J.: Testis involvement in Schlonlein-Henoch purpura. Path. Res. Pract., 165: 323, 1979. Khan, A. U., Williams, T. H. and Malek, R. S.: Acute scrotal swelling in Henoch-SchOnlein syndrome. Urology, 10: 139, 1977.