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POLYARTERITIS NODOSA PRESENTING AS HEMATURIA AND A TESTICULAR MASS
0022-5347/01/1662-0624/0 THE JOURNAL OF UROLOGY® Copyright © 2001 by AMERICAN UROLOGICAL ASSOCIATION, INC.®
Vol. 166, 624, August 2001 Printed in U.S.A.
POLYARTERITIS NODOSA PRESENTING AS HEMATURIA AND A TESTICULAR MASS KARYN S. EILBER, STEPHEN J. FREEDLAND
AND
JACOB RAJFER*
From the Department of Urology, University of California Los Angeles, Los Angeles, California KEY WORDS: polyarteritis nodosa, hematuria, abnormalities
Polyarteritis nodosa is a rare, necrotizing vasculitis of small and medium arteries that may involve any organ. Testicular and renal involvement is the rule. Diagnosis is often difficult and delayed. CASE REPORT
A 43-year-old white man presented with a 1-week history of fever, myalgia and gross hematuria. The patient denied any dysuria or flank pain. History was significant for renal insufficiency presumed secondary to essential hypertension. Physical examination revealed a tender extratesticular mass superior to the left testis and a soft atrophic right testicle. Urinalysis was remarkable for 652 red blood cells per high power field without evidence of infection. Serum blood urea nitrogen and creatinine were 60 mg./dl. (normal 10 to 20) and 2.6 mg./dl. (normal 0.5 to 1.2), respectively. Erythrocyte sedimentation rate was 74 mm. per hour (normal 0 to 15) and antinuclear antibody screen was positive. Complete blood count, liver function tests and testicular tumor markers were normal. Blood and urine cultures were negative. An echocardiogram was negative for valvar vegetations. Computerized tomography of the abdomen and pelvis was unremarkable. Scrotal ultrasound confirmed a simple, left epididymal cyst and detected a suspicious right, heterogeneous intratesticular lesion. Cystoscopy and urine cytology were unremarkable. The patient underwent right radical orchiectomy for a presumed testicular tumor. Pathological examination was remarkable for polyarteritis nodosa (see figure). Fever, myalgia and hematuria resolved on postoperative day 1.
polyarteritis nodosa cases remains unclear. Associations with multiple viruses, including hepatitis B virus and HIV, have been described.1 The clinical manifestations of polyarteritis nodosa vary considerably. The majority of patients present with constitutional symptoms, that is malaise, fever and weight loss, and they appear acutely ill. Invariably polyarteritis involves the genitourinary system with testicular pathology and renal insufficiency included in the diagnostic criteria for polyarteritis nodosa.1 Reported testicular signs and symptoms include pain, tenderness, mass and small size.2, 3 Glomerular nephropathy and/or rupture of microaneurysms may cause hematuria, and hypertension may result from renal artery involvement.1 Definitive diagnosis is based on histology. Arterial lesions of polyarteritis nodosa are characterized by infarction, necrosis, hemorrhage, microaneurysms and leukocytic infiltration (see figure).2 Tissues frequently biopsied include skeletal muscle, sural nerve, kidney, testis, liver and rectum. When tissue diagnosis is equivocal, angiography may be considered to detect microaneurysms.1 The prognosis for polyarteritis nodosa has been greatly improved with the use of immunosuppressive drugs. Corticosteroids have increased the 5-year survival rate from 10% for untreated patients to more than 50%, and as high as 82% with the addition of cyclophosphamide.1 To our knowledge we report the first case of polyarteritis nodosa presenting simultaneously with hematuria and a testicular lesion. Polyarteritis must be considered in the differential diagnosis of renal insufficiency and atypical testicular lesions. REFERENCES
DISCUSSION
Polyarteritis, also referred to as periarteritis and polyarteritis nodosa, is a clinical and pathological disease of small and medium arteries. It is an uncommon disease with an annual incidence of 0.7/100,000, occurring mainly in males between the fourth and sixth decades of life. Although considered an immunopathogenic disease, the etiology of most Accepted for publication March 16, 2001. *Financial interest and/or other relationship with Pfizer and Bayer.
1. Lhote, F. and Guillevin, L.: Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment. Rheum Dis Clin North Am, 21: 911, 1995 2. Dahl, E. V., Baggenstoss, A. H. and DeWeerd, J. H.: Testicular lesions of periarteritis nodosa, with special reference to diagnosis. Am J Med, 28: 222, 1960 3. Huisman, T. K., Collins, W. T. 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
Polyarteritis nodosa. A, inflammatory infiltrate and thrombosis in vessel lumina. Reduced from ⫻ 10. B, necrosis of intimal layer. Reduced from ⫻40. 624
Vol. 166, 624, August 2001 Printed in U.S.A.
POLYARTERITIS NODOSA PRESENTING AS HEMATURIA AND A TESTICULAR MASS KARYN S. EILBER, STEPHEN J. FREEDLAND
AND
JACOB RAJFER*
From the Department of Urology, University of California Los Angeles, Los Angeles, California KEY WORDS: polyarteritis nodosa, hematuria, abnormalities
Polyarteritis nodosa is a rare, necrotizing vasculitis of small and medium arteries that may involve any organ. Testicular and renal involvement is the rule. Diagnosis is often difficult and delayed. CASE REPORT
A 43-year-old white man presented with a 1-week history of fever, myalgia and gross hematuria. The patient denied any dysuria or flank pain. History was significant for renal insufficiency presumed secondary to essential hypertension. Physical examination revealed a tender extratesticular mass superior to the left testis and a soft atrophic right testicle. Urinalysis was remarkable for 652 red blood cells per high power field without evidence of infection. Serum blood urea nitrogen and creatinine were 60 mg./dl. (normal 10 to 20) and 2.6 mg./dl. (normal 0.5 to 1.2), respectively. Erythrocyte sedimentation rate was 74 mm. per hour (normal 0 to 15) and antinuclear antibody screen was positive. Complete blood count, liver function tests and testicular tumor markers were normal. Blood and urine cultures were negative. An echocardiogram was negative for valvar vegetations. Computerized tomography of the abdomen and pelvis was unremarkable. Scrotal ultrasound confirmed a simple, left epididymal cyst and detected a suspicious right, heterogeneous intratesticular lesion. Cystoscopy and urine cytology were unremarkable. The patient underwent right radical orchiectomy for a presumed testicular tumor. Pathological examination was remarkable for polyarteritis nodosa (see figure). Fever, myalgia and hematuria resolved on postoperative day 1.
polyarteritis nodosa cases remains unclear. Associations with multiple viruses, including hepatitis B virus and HIV, have been described.1 The clinical manifestations of polyarteritis nodosa vary considerably. The majority of patients present with constitutional symptoms, that is malaise, fever and weight loss, and they appear acutely ill. Invariably polyarteritis involves the genitourinary system with testicular pathology and renal insufficiency included in the diagnostic criteria for polyarteritis nodosa.1 Reported testicular signs and symptoms include pain, tenderness, mass and small size.2, 3 Glomerular nephropathy and/or rupture of microaneurysms may cause hematuria, and hypertension may result from renal artery involvement.1 Definitive diagnosis is based on histology. Arterial lesions of polyarteritis nodosa are characterized by infarction, necrosis, hemorrhage, microaneurysms and leukocytic infiltration (see figure).2 Tissues frequently biopsied include skeletal muscle, sural nerve, kidney, testis, liver and rectum. When tissue diagnosis is equivocal, angiography may be considered to detect microaneurysms.1 The prognosis for polyarteritis nodosa has been greatly improved with the use of immunosuppressive drugs. Corticosteroids have increased the 5-year survival rate from 10% for untreated patients to more than 50%, and as high as 82% with the addition of cyclophosphamide.1 To our knowledge we report the first case of polyarteritis nodosa presenting simultaneously with hematuria and a testicular lesion. Polyarteritis must be considered in the differential diagnosis of renal insufficiency and atypical testicular lesions. REFERENCES
DISCUSSION
Polyarteritis, also referred to as periarteritis and polyarteritis nodosa, is a clinical and pathological disease of small and medium arteries. It is an uncommon disease with an annual incidence of 0.7/100,000, occurring mainly in males between the fourth and sixth decades of life. Although considered an immunopathogenic disease, the etiology of most Accepted for publication March 16, 2001. *Financial interest and/or other relationship with Pfizer and Bayer.
1. Lhote, F. and Guillevin, L.: Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment. Rheum Dis Clin North Am, 21: 911, 1995 2. Dahl, E. V., Baggenstoss, A. H. and DeWeerd, J. H.: Testicular lesions of periarteritis nodosa, with special reference to diagnosis. Am J Med, 28: 222, 1960 3. Huisman, T. K., Collins, W. T. 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
Polyarteritis nodosa. A, inflammatory infiltrate and thrombosis in vessel lumina. Reduced from ⫻ 10. B, necrosis of intimal layer. Reduced from ⫻40. 624