Polyarteritis nodosa diagnosed by renal arteriography

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Brief clinical and laboratory observations

there was an accessory spleen. Blood cultures taken at autopsy were negative. Microscopic examination of the vegetation in the region of the pulmonary band showed thrombus formation without marked fibrosis. There was no evidence of bacteria with special stains. It was concluded that the vegetation represented thrombotic nonbacterial endoarteritis of recent onset. DISCUSSION Verrucous endocarditis has previously been r e p o r t e d in association with a p u l m o n a r y b a n d which h a d cut t h r o u g h the posterior wall of the artery. 4 However, occlusion of the stenotic orifice with a verrucous lesion has not been reported. This complication should be considered w h e n r a p i d deterioration with cyanosis occurs in a p a t i e n t with a p u l m o n a r y artery band. A l t h o u g h this is the only such case in 118 b a n d i n g procedures done in this hospital in the t5 years from 1951 through 1966, it m i g h t be a n t i c i p a t e d t h a t this late complication m a y a p p e a r m o r e frequently as more of these operations are performed. T h e question which remains unanswered is w h e t h e r the vegetations on the p u l m o n a r y artery b a n d represented bacterial or nonbacterial endoarteritis. T h e course of this child's illness was consistent with subacute

Polyarteritis nodosa diagnosed by renal arteriography L. G. McLaln, M.D., J. j. Bookste~n, M.D., and R. C. Kelsch, M.D., A n n Arbor, Mich.

From the Department of Pediatrics and Communicable Diseases, and the Department of Radiology, University of Michigan Medical Center. Reprint address: Department o] Pediatrics and Communicable Diseases, University o[ Michigan Medical Center, 1405 Ann St., Ann Arbor, Mich. 48104.

The ]ournat of Pediatrics June 19'72

bacterial endoarteritis. However, no bacteria were isolated from blood cultures taken preor post m o r t e m , a n d no bacterial colonies were seen in the vegetation with histologic stains. T h e penicillin t h e r a p y m i g h t account for the p o s t m o r t e m absence of bacteria. O n the other hand, the lesion m a y have been sterile from the outset. T h e vegetations were on the distal p o r t i o n of the b a n d , an a r e a of m a x i m a l turbulence. E i t h e r the turbulence or m i n i m a l erosion of the b a n d into the lumen m a y have led to disruption of the e n d o t h e l i u m in this region, predisposing to deposition of fibrin a n d thrombus formation.

REFERENCES 1. Muller, W. It., Jr., and Dammann, J. F., Jr.: The treatment of certain congenital malformations of the heart by the creation of pulmonic stenosis to reduce pulmonary hypertension and excessive pulmonary blood flow, Surg. Gynecol. Obstet. 95" 213, 1952. 2. Stark, J,, Aberdeen, E., Waterston, D. J., Bonham-Carter, R. E., and Tynan, M.: Pulmonary artery constriction (banding): A report of 146 cases, Surgery 65" 808, 1969. 3. Woda, J., and Iwa, T.: Two stage treatment of ventricular septal defect with pulmonary hypertension, Ann. Thorac. Surg. 8" 415, 1969. 4. Rohmer, J., Brom, A. G., and Nauta, J.: Bands inside the pulmonary artery--a complication of the Dammann-Muller procedure, Ann. Thorac. Surg. 3" 449, 1967.

T H E D I A G N O S I S O F polyarteritis is m a d e d u r i n g life in only a small n u m b e r of patients. Utilizing arteriography, 1-4 several authors have d e m o n s t r a t e d m u l t i p l e intrap a r e n c h y m a l aneurysms of the kidney, liver, a n d spleen in patients w i t h polyarteritis. T h e purpose of this p a p e r is to present a patient in w h o m a r t e r i o g r a p h y defined the subacute lesions of polyarteritis nodosa a n d their regression following the use of steroid therapy.

CASE REPORT The patient was a 16-year-old Negro boy who in May, 1970, began complaining of epigastric pain and intermittent episodes of vomiting. White .blood counts obtained during the next 30 days ranged between 18,000 and 21,000 but no apparent infection was found. Because of persistent

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Brief clinical and laboratory observations

symptoms, despite treatment with a l m u m i n u m hydroxide gel and methantheline, 1,200,000 units of penicillin (Bicillin C-R) was administered intranmscularly. Within two days he developed a fine pruritic papular rash and a low-grade fever which were interpreted to be the result of a penicillin reaction. No more penicillin was given but treatment with lincomycin was initiated. Two weeks later he was admitted to The University of Michigan Medical Center with persistent abdominal pain, fever, and a 24 pound weight loss. At the time of admission pertinent ancillary history included the statement by the patient that he had used "a kind of grass" on two occasions approximately one year prior to his illness and in one instance had sniffed cocaine; he denied the use of other drugs. General appearance was that of an acutely and chronically ill young male. Blood pressure was 135/90, respirations 24, and pulse rate 110. A diffuse, finely papular erythematous rash which covered the trunk, scalp, and extremities was beginning to desquamate. He had superficial tenderness and hyperesthesia over the right upper quadrant. There was bilateral flank tenderness and he complained of pain on palpation of his calf muscles. Laboratory findings were: hemoglobin, 12 Gm. per 100 ml.; white blood count, 16,000 (65 per cent polymorphonuclear leukocytes, 22 per cent lymphocytes, 8 per cent eosinophils); and platelet count, 385,000 per cubic millimeter. The urinalysis showed a specific gravity of 1,020, pH 5, sugar negative, and protein negative; the spun sediment contained 5 white blood cells per high-power field, but no bacteria were seen. The blood urea nitrogen was 11 rag. per 100 ml., serum creatinine 1.3 mg. per 100 ml., and creatinine clearance 161 liters per 24 hours per 1.73 M 2. The total serum protein was 10.0 Gm. per 100 ml., 44 per cent of which was gamma globulin. Immunodiffusion showed IgA of 325, IgM 235, and IgG 2,360 mg. per 100 ml. The antistreptolysin-O titer was 833 units, C-re.active protein 6+, antinnclear antibody negative, lupus erythematosus prep negative, and cold agglutinins positive 1:640. The latex fixation test was negative. The S G O T was 242 units and S G P T was 114 units. Electrocardiograms suggested the possibility of a posterior myocardial infarction. The patient's hospital course was characterized by anorexia and wasting with a further loss of 16 pounds by July. Calf pain persisted but was variable in intensity. The blood pressure began to rise and by the end of July had reached

10 3 3

Fig. 1. Initial left renal arteriogram. Note multiple 2 to 5 mm. aneurysms and stenoses and occlusions of distal interlobar arteries. 210/130. During his hospitalization, biopsy of the liver was read as showing focal disarray of hepatic plates with plasma cell infillrates, but preservation of the lobular architecture. There was a mononuclear infiltrate in the portal spaces and in the sinusoids; a diagnosis of reactive hepatosis was made. An inguinal lymph node biopsy was interpreted as dermatopathic lymphadenitis on the basis of a reticuloendothelial hyperpiasia with numerous histiocytes containing melanin. A biopsy of calf muscle was described as normal. Because of the development of hypertension, as well as a suspected diagnosis of polyarteritis nodosa, arteriography was performed. Bilateral selective renal arteriography demonstrated multiple aneurysms, 2 to 5 mm. in diameter, primarily near the bifurcation of interlobar arteries into arcuate vessels (Fig. 1). Some arterial stenoses and occlusions were also demonstrated in distal interlobar arteries; the nephrogram demonstrated cortical defects suggestive of infarcts. As judged by backflow of contrast medium into the aorta, there was diminished renal blood flow bilaterally. Celiac arteriography demonstrated multiple 2 to 3 mm. aneurysms in several arteries of the right lobe of the liver.

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The Journal of Pediatrics June 1972

Fig. 2. Renal arteriogram performed after a one-year interval. A. Early arterial phase demonstrates complete disappearance of all aneurysms. Only an occasional residual stenosis was evident on the original films. B, Nephrogram phase demonstrating multiple cortical defects due to previous renal infarction.

Following the arteriography the patient was given 10 rag. of prednisone twice a day for five months and then 20 rag. of prednisone once every other day for approximately six months. All medication was discontinued after one year. Following the onset of prednisone therapy there was striking improvement in the patient's general well being, almost complete relief of muscle pain, increase in appetite, and improvement in general attitude. Eight months after the initiation of therapy the patient had regained his lost weight (40 pounds). His blood pressure was 130/85 while taking guanethedine 25 rag. per day. One year after the Onset of his illness a repeat arteriogram revealed complete resolution of the aneurysms previously noted (Fig. 2, A) but areas of multiple infarction were still present (Fig. 2, B). The aneurysms of the hepatic artery had also disappeared without residual stenoses. DISCUSSION This patient had the clinical findings of polyarteritis nodosa but we were unable to make a histologic diagnosis of the disease by muscle, liver, or lymph node biopsy. Percutaneous renal biopsy was not done since it is difficult to make a definitive diagnosis of

polyarteritis unless a medium-sized artery is obtained in the specimen, ~ a particularly undesirable occurrence in the presence of serious hypertension. T h e renal arteriogram demonstrated the classic s, 7 form of polyarteritis with multiple aneurysms of the interlobar arteries and infarctions of the cortical parenchyma. T h e patient improved dramatically following the onset of low-dose steroid therapy. We believe that this represents the first case report of a patient with polyarteritis nodosa diagnosed by arteriography in w h o m follow-up arteriographic studies demonstrated repair of the aneurysms but persistent evidence of infarction, It is hoped that further experience with this technique will improve the incidence of premortem diagnosis of polyarteritis nodosa. T h e etiology of this patient's disease is unknown but the evidence that drug abuse might be responsible is minimal. 4 REFERENCES

1.

Fleming, R. J., and Stern, L. Z.: Multiple intraparenchymal renal aneurysms in polyarteritis nodosa, Radiology 84: 100, 1965.

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2. Herschmann, A., Blum, R., and Lee, Y. C.: Angiographie findings in polyarteritis nodosa, Radiology 94: 147, 1970. 3. Dornfeld, L., Lecky, J. W., and Peter, J. B.: Polyarteritis and intrarenal artery aneurysms, J. A. M. A. 215: 1950, 1971. 4. Citron, B. P., Halpern, M., McCarron, M., Lundberg, G. D., McCormick, R., Pincus, I. J., Tatter, D., and Haverback, B. J.: Necrotizing angiitis associated with drug abuse, N. Engh J. Med. 283: 1003, 1970.

5. Hamburger, J.: Nephrology, vol. 2, Philadelphia, 1968, W. B. Saunders Company, p. 843. 6. Davson, J., Ball, J., and Platt, R.: The kidney in periarteritis nodosa, Quart. J. Med. 17: 175, 1948. 7. Heptinstall, R. H.: Pathology of the kidney, Boston, 1966, Little, Brown & Company, p. 509.

Disseminated i n t ra ra scu la r

at a rural lake. He was reportedly bitten by an unidentified insect. Several hours later he developed headache and vomiting. He was admitted to another hospital where a vesicle surrounded by ecchymosis was noted on his back. This area became necrotic and he developed progressive iaundice and gross hematuria over the next 24 hours. He was then referred to Children's Memorial Hospital. The physical examination revealed a pale, lethargic child who was critically ill. His blood pressure was 100/50, temperature 37.5~ C., and pulse 100. Perioral cyanosis and acrocyanosis were present, and the skin and sclerae were icteric. Located in the interscapular area was a small necrotic lesion with a 5 x 9 cm. circumferential ecchymotic area which followed a dependent pattern. There was swelling and tenderness of the sm'rounding soft tissues. Petechiae were noted over the shoulders, thighs, and buttocks. The liver was palpable 2 cm. below the right costal margin, Laboratory data included: hemoglobin, 6.8 rag. per 100 ml.; hematocrit, 11 per cent; white blood count, 22,700 per cubic millimeter with 89 per cent neutrophils, 8 per cent bands, and 3 per cent lymphocytes. The peripheral blood smear showed spherocytosis, many schistocytes, and decreased platelets. The platelet count was 25,000 per cubic millimeter by phase microscopy. The partial thromboplastin time was 77.4 seconds (normal <50 seconds), and the prothrombin time was 21.9 seconds (control 12.9 seconds). The fibrinogen was 88 mg. per cent (norreal >160 rag. per cent). The fibrin split products were 80 tag per cent (normal < 5 /xg per cent). The urine and serum had a dark brown color. The urine was positive for hemoglobin. Other laboratory studies were as follows: biHrubin, total 8.2, direct 5.3; SGOT, 583u; blood urea nitrogen, 44 rag. per cent; CO~, 15 ram. per liter; potassium, 5.4 mEq. per liter; chloride, 104 mEq. per liter~ and sodium, 135 mEq. per liter.

coagulopatby f olloMng fatal brown spider bite (necrotic aracbnidism) Hal Vorse, M.D., Paul Seccareccio, M.D., Kay Woodruff, M.D., and G.

Bennett Humphrey,

M.D., Ph.D.,

O k l a h o m a City, Okla. ALTJ~Ob'O~t the brown spider, Loxosceles reclusa, has been k n o w n to exist in the Southwestern U n i t e d States for m a n y years, 1 it was n o t associated with necrotic arachnidism until the work of Atkins a n d associates 2 in 1957. Since that time, the bite of the b r o w n recluse spider has been recognized as a frequent cause of illness in a large area of the U n i t e d States. a-6 Serious systemic symptoms may occur, particularly intravascular hemolysis a n d renal failure?. 4-s These complications are most f r e q u e n t in children a n d m a y lead to death. We have observed a child in whom disseminated intravascular coagulation was d o c u m e n t e d following systemic necrotic arachnidism.

CASE REPORT Patient J. D. was a healthy 6-year-old Cauca~.ian boy who had been pIaying under a wharf

From the Departments o[ Pediatrics and Pathology, and the Children's Memorial Hospital, University o[ Oklahoma Medical Center.