THE WESTERN JOURNAL OF MEDICINE * NOVEMBER 1991

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13. Young RC, Bennett JE, Vogel CL, et al: Aspergillosis: The spectrum of the disease in 98 patients. Medicine (Baltimore) 1970; 49:147-173 14. Kher HL, Ahuja IM, Rastogi DS, Dua SP: Primary disseminated aspergillosis involving myocardium: Review ofliterature with a case report. J Assoc Physicians India 1977; 25:433-436 15. Meyer RD, Fox ML: Aspergillus endocarditis: Therapeutic failure of amphotericin B. Arch Intern Med 1973; 132:102-106

Bilateral Spontaneous Renal Hemorrhage Due to Polyarteritis Nodosa JOSEPH C. PRESTI, Jr, MD New York, New York PETER R. CARROLL, MD

San Francisco, California

POLYARTERITIS NODOSA iS a progressive, necrotizing, inflammatory disease of the medium and small muscular arteries. The kidneys are the most common organs involved (70% to 80% of cases). Although the cause is unknown, much evidence supports an autoimmune origin, and an interesting subgroup of patients shares a history of intravenous methamphetamine abuse. The case reported here shows the importance of the aggressive medical treatment of the disorder. Report of a Case The patient, a 27-year-old woman with a history of intravenous methamphetamine abuse, was evaluated at an outpatient clinic for fever of several days' duration, anorexia, arthralgia, and right upper quadrant pain. The results of liver function tests were elevated: alkaline phosphatase 805 IU per liter (normal 4 to 133); aspartate aminotransferase (previously SGOT) 81 IU per liter (normal 7 to 39); the hepatitis B surface antigen (HBsAg) test was positive; and tests for the human immunodeficiency virus were negative. Ultrasonography showed a normal gallbladder and biliary tree. The patient was treated expectantly for a presumptive diagnosis of hepatitis. Ten days later the patient was admitted to another hospital with fever and worsening upper quadrant pain. On physical examination, she had a temperature of 39.4°C (103°F) and right upper quadrant tenderness. The hematocrit was 0.30 (normal 0.36 to 0.46), leukocyte count 25 x 109 per liter (25,000 per IL; normal 3.4 to 10 x 109 per liter), but the serum creatinine value and results of a urinalysis were normal. An ultrasonogram showed a thickened gallbladder wall, and a regimen of intravenous antibiotics was begun for a presumptive diagnosis of acute cholecystitis. Two days later, increasing abdominal and left flank pain developed, along with abdominal guarding and rebound tenderness. The hematocrit dropped to 0.21, and the patient became hypertensive. At laparotomy, 1 liter of brown ascitic fluid was removed and the liver was noted to be firm with a fibrinous exudate. The gallbladder appeared normal. Biopsy

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specimens were taken from the liver and enlarged mesenteric lymph nodes. The retroperitoneum was edematous. The patient did well until the fourth postoperative day when she became acutely hypotensive and gross hematuria developed. The hematocrit was 0.08, and the serum creatinine value was 190 limol per liter (normal 40 to 120). She was resuscitated with the administration of crystalloid and 6 units of packed erythrocytes. Computed tomography showed bilaterally enlarged kidneys with irregular, hypodense infiltrates that, by densitometric analysis, were consistent with intrarenal hemorrhage (Figure 1). The patient was transferred to the University of California, San Francisco, Medical Center hemodynamically stable with a hematocrit of0.28. Renal ultrasonography showed the right kidney to be 13 cm and the left kidney 20 cm long. A review ofthe liver and lymph node biopsy specimens showed focal necrotizing arteritis consistent with polyarteritis nodosa. The patient remained hemodynamically stable and was given corticosteroids (3 grams of methylprednisolone sodium succinate over three days) and a pulse dose of cyclophosphamide (750 mg per m2). With therapy, the leukocyte count declined quickly from 51 x 109 per liter to a nadir of 1.7 x 109 per liter at day 16. Serum creatinine values peaked at 450 ,tmol per liter and stabilized at 240 limol per liter. The patient's hypertension eventually responded to medical therapy. Magnetic resonance imaging was done before discharge and showed large, bilateral perinephric fluid collections of intermediate signal intensity on TI-weighted images that increased in signal intensity on T2-weighted images-consistent with resolving perinephric hematoma (Figure 2). Twenty days after the initiation of therapy, she was discharged on a maintenance dose of corticosteroids and cyclophosphamide. A year later, the patient was taking antihypertensive medications, had a serum creatinine level of 280 itmol per liter, and had no evidence of active polyarteritis nodosa.

Comment Polyarteritis nodosa is a necrotizing, inflammatory disease ofboth medium and small muscular arteries. There is no age predilection, but it is more common in men than in women. Presenting symptoms include generalized constitutional symptoms as well as organ-specific signs and symptoms. Multiorgan involvement is common, with the kidneys being the most frequently involved. Indeed, renal disease

(Presti JC Jr, Carroll PR: Bilateral spontaneous renal hemorrhage due to polyarteritis nodosa. West J Med 1991 Nov; 155:527-528) From the Department of Urology, University of California, San Francisco, School of Medicine. Dr Presti is now with the Memorial Sloan-Kettering Cancer Center, New York, New York. Reprint requests to Peter R. Carroll, MD, Department of Urology, University of California, San Francisco, CA 94143-0738.

Figure 1.-A computed tomographic scan shows massive bilateral renal hemorrhage.

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Refractory cases may be successfully managed with angiographic embolization. If surgical intervention is necessary, partial nephrectomy should be attempted, if feasible, as renal failure accounts for much of the morbidity and mortality associated with the disease process.

Figure 2.-A magnetic resonance image (coronal plane) 10 days after the initiation of therapy shows persistent renal enlargement and hemorrhage.

occurs in as many as 70% to 80% of patients, manifested chiefly as hematuria or proteinuria. Hypertension occurs in 50% of cases. Gastrointestinal findings due to mesenteric artery involvement are noted in 60%. Advanced disease may involve coronary arteries, resulting in myocardial infarction. 1 Although the cause of polyarteritis nodosa is unknown, much evidence supports an autoimmune origin. As many as 30% of patients test positive for HBsAg. Hepatitis B antigen along with immunoglobulin G has been found in the walls of vessels with acute polyarteritic lesions.23 An association between polyarteritis nodosa and the Australia antigen2 as well as rheumatic fever and rheumatic heart disease4 has been reported. A type of vasculitis that is indistinguishable from polyarteritis nodosa has been recognized in a large number of intravenous drug abusers,5 and methamphetamine abuse appears to be a common denominator. It thus seems likely that a variety of antigens may, under appropriate circumstances, stimulate the immune system, resulting in polyarteritis nodosa. Pathologic findings in the kidneys of patients with the disorder may be related to glomerulonephritis, arteritis of medium and small vessels, or both. Antigen-antibody complex deposition along basement membranes initiates an inflammatory response. The subsequent necrotizing reaction destroys the elastic lamina of the vessels, thus weakening the walls and resulting in aneurysm formation, thrombosis, or rupture. The arcuate and interlobular arteries are the most commonly involved renal vessels.6', Aneurysm formation tends to occur at branch points and produces a characteristic "beading pattern" on angiography.8 Renal failure remains one of the major causes of death. Corticosteroids and cytotoxic agents are the cornerstones of medical therapy. Without treatment, half to two thirds of patients will die of renal failure or congestive heart failure within a year. With corticosteroid treatment, the five-year survival is about 40%.1 The use of cytotoxic agents may increase survival or benefit those in whom corticosteroid therapy has failed. Spontaneous renal hemorrhage or rupture has been reported previously in patients with polyarteritis nodosa and is typically unilateral.9'3 In many cases, emergency surgical treatment and nephrectomy are needed. Because of the diffuse nature of the disease process, attempts at maximal renal preservation are warranted. As in the present case, aggressive medical therapy may obviate surgical intervention.

REFERENCES 1. Fauci AS: The vasculitis syndromes, chap 269, In Braunwald E, Isselbacher KJ, Petersdorf RG, Wilson JD, Martin JB, Fauci AS (Eds): Harrison's Principles of Internal Medicine, 1 Ith Ed. New York, NY, McGraw-Hill, 1987, pp 1438-1445 2. Gocke EJ, Hsu K, Morgan C, et al: Association between polyarteritis and Australian antigen. Lancet 1970; 2:1149-1153 3. Michalak T: Immune complexes of hepatitis B surface antigen in the pathogenesis of periarteritis nodosa. Am J Pathol 1978; 90:619-628 4. Freidberg CK, Gross L: Periarteritis nodosa (necrotizing arteritis) associated with rheumatic heart disease, with a note on abdominal rheumatism. Arch Intern Med 1934; 54:170 5. Citron BP, Halpem M, McCarron M, et al: Necrotizing angiitis associated with drug abuse. N Engl J Med 1970; 283:1003-1011 6. Ralston DE, Kvale WF: The renal lesions of periarteritis nodosa. Mayo Clin Proc 1949; 24:18-27 7. Arkin A: A clinical and pathological study of periarteritis nodosa; report of five cases, one histologically healed. Am J Pathol 1930; 6:401-426 8. Peterson C Jr, Willerson JT, Doppman JL, et al: Polyarteritis nodosa with bilateral renal artery aneurysms and perirenal haematomas-Angiographic and nephrotomographic features. Br J Radiol 1970; 43:62-66 9. Cornfield JZ, Johnson ML, Dolehide J, et al: Massive renal hemorrhage owing to polyarteritis nodosa. J Urol 1988; 140:808-811 10. Tocci PE, Lankford RW, Lymme CM: Spontaneous rupture of the kidney secondary to polyarteritis nodosa. J Urol 1975; 1 13:860-863 11. Litvak AS, Lucas BA, McRoberts JW: Urologic manifestations of polyarteritis nodosa. J Urol 1976; 115:572-574 12. Henderson FW, Jordan WP: Spontaneous rupture of the kidney due to polyarteritis nodosa-A case report. J Urol 1967; 97:811-817 13. Ostrum BJ, Soder PD: Periarteritis nodosa complicated by spontaneous perinephric hematoma-Roentgenographic findings in three cases and a review of the literature. Am J Roentgenol 1960; 84:849-860

The Numb Chin in Metastatic Cancer CHERYL P. HARRIS, MD J. RICHARD BARINGER, MD Salt Lake City, Utah

THE MENTAL NERVE is one of the terminal branches of the third (mandibular) division of cranial nerve V, the trigeminal nerve. The mandibular division exits the base of the skull at the foramen ovale and branches until it reaches the mental foramen in the horizontal ramus of the mandible. The mental nerve has many small offshoots that supply the skin of the chin and lower lip and the mucous membranes on the buccal surface of the lower lip. 1 Many neurologic processes produce lesions of the trigeminal nerve inside the cranial vault, including vertebrobasilar vascular disease,2 intracranial tumors,3 and multiple sclerosis. Lesions of the mandibular division include compression by tumors, meningeal carcinomatosis, and basilar meningitis, among many other causes. Lesions affecting solely the mental nerve are less common and generally produce more localized symptoms than involvement of the mandibular division or of the entire trigeminal nerve. Also commonly classified under the term "mental neuropathy" are lesions of the inferior alveolar nerve, from which the mental nerve branches, occurring at the mandibular foramen. In addition to the numbness of the (Harris CP, Baringer JR: The numb chin in metastatic cancer. West J Med 1991 Nov; 155:528-531) From the Departments of Pathology (Dr Harris) and Neurology (Dr Baringer), University of Utah Medical Center, Salt Lake City. Reprint requests to Cheryl P. Harris, MD, Department of Pathology, University of Utah Medical Center, 50 N Medical Dr, Salt Lake City, UT 84132.

Bilateral spontaneous renal hemorrhage due to polyarteritis nodosa.

THE WESTERN JOURNAL OF MEDICINE * NOVEMBER 1991 * 155 13. Young RC, Bennett JE, Vogel CL, et al: Aspergillosis: The spectrum of the disease in 98 p...
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