polyarteritis nodosa complicated by a ruptured intrahepatic aneurysm

3
106 BRIEF REPORT POLYARTERITIS NODOSA COMPLICATED BY A RUPTURED INTRAHEPATIC ANEURYSM A. A. M. BOOKMAN, E. GOODE, M. J. McLOUGHLIN, and ZANE COHEN Ruptured aneurysms complicate the course of polyarteritis nodosa only infrequently. Most often they occur in renal or mesenteric vessels (1-3). One case of hepatic aneurysm rupture has been described previously (4). This report describes the clinical fea- tures of a ruptured intrahepatic aneurysm, and the lifesaving role played by angiography. Case Report. The patient, a 42-year-old, Guy- ana-born actuarial clerk of East Indian heritage, was first seen at our clinic in 1978. He had a history of childhood asthma until age 13 with a transient recur- rence at age 28. At age 34 an asymptomatic enlarged liver was noted. In May 1978 he developed dull aching epigastric pain that lasted 1 month. He had two more short episodes with elevated amylase. Pancreatitis was diagnosed and managed on an outpatient basis with cimetidine. Oral cholecystogram results were normal. During August 1978 he lost 12 kg and had frequent bulky greasy stools. Endoscopic retrograde cholangio- pancreatography showed results characteristic of chronic pancreatitis. Treatment with dessicated pan- creatic supplement normalized his stools, and his weight increased. In October 1978 he developed severe myalgia and in November was admitted to Toronto General From the Division of Rheumatology, Toronto General Hospital, University of Toronto. A. A. M. Bookman, MD, FRCP(C): Assistant Professor of Medicine, Division of Rheumatology; E. Goode, MD: Resident in Internal Medicine; M. J. McLoughlin, MD, FRCP(C): Professor of Radiology; Zane Cohen, MD, FRCS(C): Assistant Profes or of Surgery, Division of General Surgery, Toronto General Ho&tal, University of Toronto. Address reprint requests to A.A.M. Bookman, MD, 99 Avenue Road, Toronto, Ontario, Canada, MSR 2GS. Submitted for publication February 5, 1982: accepted in revised form June 18, 1982. Hospital with a 10-kg weight loss over 6 weeks. A muscle biopsy showed polyarteritis nodosa of medium sized muscular arteries with lesions in various stages of evolution. Selective celiac angiogram demonstrated slight stenosis of the right hepatic artery and extensive microaneurysm formation in both lobes of the liver. Splenic, renal, and mesenteric arteries were normal (Figure 1). The following studies showed negative or normal results: cryoglobulins, complement, rheuma- toid factor, hepatitis B surface antigen, Trichinella complement fixation test, VDRL, antinuclear factor, urinalysis, creatinine clearance, and creatine phospho- kinase. Hemoglobin was 13.0 gm%, white blood count (WBC) 8,900/mm3,platelets 750,000/mm3, erythrocyte sedimentation rate (ESR) 57 mm/hour (Wintrobe), eosinophils 5%. Chest roentgenogram showed chronic obstructive lung disease; sinuses were normal. Stool collection and secretin test results confirmed pancreat- ic insufficiency. The patient was discharged on a regimen of prednisone 60 mg a day, which was tapered to 10 mg a day over the next 12 months (5). At age 44, he developed asthma and pulmonary cavitations. He was readmitted to Toronto General Hospital on November 29, 1980. All cultures were sterile and biopsy was deemed clinically inadvisable. Prednisone 60 mg daily induced resolution of the lung lesions, seen radiologically. His ESR returned to nor- mal; complement, antinuclear factor, rheumatoid fac- tor, hepatitis B surface antigen, eosinophils, and cryo- globulins remained normal. Renal function studies and sinus roentgenograms were again unremarkable. After 2 months of receiving this dose of predni- sone, the patient abruptly developed diffuse abdominal pain. This became localized to the right upper quad- rant after 48 hours. On February 5, 1981 he was Arthritis and Rheumatism, Vol. 26, No. 1 (January 1983)

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Page 1: Polyarteritis nodosa complicated by a ruptured intrahepatic aneurysm

106

BRIEF REPORT

POLYARTERITIS NODOSA COMPLICATED BY A RUPTURED INTRAHEPATIC ANEURYSM

A. A. M. BOOKMAN, E. GOODE, M. J. McLOUGHLIN, and ZANE COHEN

Ruptured aneurysms complicate the course of polyarteritis nodosa only infrequently. Most often they occur in renal or mesenteric vessels (1-3). One case of hepatic aneurysm rupture has been described previously (4). This report describes the clinical fea- tures of a ruptured intrahepatic aneurysm, and the lifesaving role played by angiography.

Case Report. The patient, a 42-year-old, Guy- ana-born actuarial clerk of East Indian heritage, was first seen at our clinic in 1978. He had a history of childhood asthma until age 13 with a transient recur- rence at age 28. At age 34 an asymptomatic enlarged liver was noted. In May 1978 he developed dull aching epigastric pain that lasted 1 month. He had two more short episodes with elevated amylase. Pancreatitis was diagnosed and managed on an outpatient basis with cimetidine. Oral cholecystogram results were normal. During August 1978 he lost 12 kg and had frequent bulky greasy stools. Endoscopic retrograde cholangio- pancreatography showed results characteristic of chronic pancreatitis. Treatment with dessicated pan- creatic supplement normalized his stools, and his weight increased.

In October 1978 he developed severe myalgia and in November was admitted to Toronto General

From the Division of Rheumatology, Toronto General Hospital, University of Toronto.

A. A. M. Bookman, MD, FRCP(C): Assistant Professor of Medicine, Division of Rheumatology; E. Goode, MD: Resident in Internal Medicine; M. J. McLoughlin, MD, FRCP(C): Professor of Radiology; Zane Cohen, MD, FRCS(C): Assistant Profes or of Surgery, Division of General Surgery, Toronto General Ho&tal, University of Toronto.

Address reprint requests to A.A.M. Bookman, MD, 99 Avenue Road, Toronto, Ontario, Canada, MSR 2GS.

Submitted for publication February 5 , 1982: accepted in revised form June 18, 1982.

Hospital with a 10-kg weight loss over 6 weeks. A muscle biopsy showed polyarteritis nodosa of medium sized muscular arteries with lesions in various stages of evolution. Selective celiac angiogram demonstrated slight stenosis of the right hepatic artery and extensive microaneurysm formation in both lobes of the liver. Splenic, renal, and mesenteric arteries were normal (Figure 1). The following studies showed negative or normal results: cryoglobulins, complement, rheuma- toid factor, hepatitis B surface antigen, Trichinella complement fixation test, VDRL, antinuclear factor, urinalysis, creatinine clearance, and creatine phospho- kinase. Hemoglobin was 13.0 gm%, white blood count (WBC) 8,900/mm3, platelets 750,000/mm3, erythrocyte sedimentation rate (ESR) 57 mm/hour (Wintrobe), eosinophils 5%. Chest roentgenogram showed chronic obstructive lung disease; sinuses were normal. Stool collection and secretin test results confirmed pancreat- ic insufficiency. The patient was discharged on a regimen of prednisone 60 mg a day, which was tapered to 10 mg a day over the next 12 months (5 ) .

At age 44, he developed asthma and pulmonary cavitations. He was readmitted to Toronto General Hospital on November 29, 1980. All cultures were sterile and biopsy was deemed clinically inadvisable. Prednisone 60 mg daily induced resolution of the lung lesions, seen radiologically. His ESR returned to nor- mal; complement, antinuclear factor, rheumatoid fac- tor, hepatitis B surface antigen, eosinophils, and cryo- globulins remained normal. Renal function studies and sinus roentgenograms were again unremarkable.

After 2 months of receiving this dose of predni- sone, the patient abruptly developed diffuse abdominal pain. This became localized to the right upper quad- rant after 48 hours. On February 5 , 1981 he was

Arthritis and Rheumatism, Vol. 26, No. 1 (January 1983)

Page 2: Polyarteritis nodosa complicated by a ruptured intrahepatic aneurysm

BRIEF REPORTS 107

On February 8, 1981 the patient was take? to the operating room. Laparotomy showed a leaking subcapsular hematoma of the liver with bleeding from a right lobe fissure. Cholecystectomy was performed to reach the site of hemorrhage, and the fissure was debrided and the bleeding controlled.

Twenty-four hours after surgery the hemor- rhage recurred, and the patient went into shock. On February 9, repeat laparotomy revealed a new fissure, which was then packed. Within 12 hours, the patient developed profound hypotension. While his vascular status was maintained with rapid transfusion, he un- derwent emergency angiogram of the celiac plexus. Active hemorrhage from one of many large intrahe- patic aneurysms was seen (Figure 2). The right hepatic artery was embolized with the use of a Gianturco coil (6) , and all hemorrhage ceased (Figure 3).

Careful sectioning of the gallbladder showed active polyarteritis despite administration of 60 mg prednisone. Cyclophosphamide 150 mg a day was added. Ten months later the patient is clinically stable, receiving 15 mg prednisone and 100 mg cyclophospha- mide daily.

Discussion. This is the second reported case of ruptured hepatic aneurysm in a patient with polyarteri- tis. Ayers and Fitchett (4) reported a patient who presented with shock, isotope scan evidence of sub- capsular hematoma, and angiographic evidence of i large intrahepatic aneurysms. Fortunately this hemor-

I I i l

Figure 1. Celiac angiogram, November 21, 1978. Microaneurysms of medium sized hepatic arteries (arrows) can be seen within liver parenchyma at time of diagnosis of polyartentis.

rhage was contained by the liver capsu1e and SpOntaneOUSly.

readmitted to Toronto General Hospital. Physical ex- amination showed a thin male with muscle wasting, obvious steroid myopathy, and cushingoid features. Vital signs were normal. He had right upper quadrant guarding without rebound tenderness. The liver was 14 cm by percussion. The spleen was not palpable. Bowel sounds were normal. On admission, hemoglobin was 11.8 gm%, WBC 19,700/mm3 with 90% neutrophils, serum glutamic oxaloacetic transaminase 147 units/ liter (normal 0-60), alkaline phosphatase 83 unitdliter (norinal 30-90). Ultrasound showed the gallbladder to be normal. The pain eased over the next 12 hours.

Forty-eight hours after admission, the patient developed a pulse of 120/minute and diaphoresis with abdominal distention. Blood pressure was 100/60 mm Hg. Peritoneal aspirate showed bloody fluid. Hemo-

Figure 2. Celiac angiogram, February 10. 1981. lntrahepatic aneu- rysms have enlarged considerably. Contrast material is rigorously extruded from one lesion (dark arrow) and can be seen tracking

globin was now 6.6 gm%. through the hepatic parenchyma (light arrow).

Page 3: Polyarteritis nodosa complicated by a ruptured intrahepatic aneurysm

108 BRIEF REPORTS

Figure 3. Celiac angiogram. February I ? , 19dl. After insertion of Gianturco coils (6). blood Row has ceased in right hepatic arwry (arrow). Insert shows close-up of spring embolus alone and extrud- ing from its cartridge.

In the case presented here, surgical interven- tion was necessitated by iptraperitoneal hemorrhage and shock, with near tragic consequences. Angiogra- phy not only provided dramatic visualization of the hemorrhaging aneurysm, but it also permitted lifesav- ing embolization of the right hepatic artery. The liver remains viable by drawing on collateral blood supply for nutrition (7).

In pol yarteritis nodosa, hemorrhage from he- patic aneurysm is rare, but perinephric and mesenteric artery aneurysm ruptures have been reported. Most of these patients are hypertensive with active vasculitis (1-3). Size is not always important, since most aneu- rysms are less than 5 mm in diameter. In contrast, the patients with intrahepatic hemorrhage suffered large sacculations (6-12 mm).

Cyclophosphamide has produced angiographic resolution of aneurysms in polyarteritis (8). Our pa-

tient certainly had active progressive disease while receiving 60 mg of prednisone. Selective embolization of the hepatic artery has stabilized him, allowing further clinical resolution with the immunosuppres- sant.

Selective embolization for control of massive hepatic hemorrhage has been utilized with great suc- cess since 1977 (7) and was recently used to treat intrahepatic mycotic aneurysms (9). Immediate collat- eral circulation to the embolized area can be shown angiographically ( 10). so that liver infarction rarely occurs.

An acute intraabdominal hemorrhage in a pa- tient with polyarteritis should thus be indication for urgent angiography. The site of bleeding can be accu- rately localized and occasionally controlled with this procedure.

Acknowledgment. The authors thank Dr. R . F. Cola- pinto for his assistance with angiographic illustration.

REFERENCES

1. Ostrum BJ. Soder PD: Periarteritis nodosa complicated by spontaneous haematoma: roentgenographic findings in 3 cases and review of the literature. Am J Roent

2. Peterson C, Willerson JT. Doppman JL, Dicker JL: Polyarteritis nodosa with bilateral renal artery aneu- rysms and perirenal haematomas: angiographic and nephrotomographic features. Br J Radiol 4?:62-71, 1970

3. Cabal E. Holtz S: Polyarteritis as a cause of intestinal hemorrhage. Gastroenterology 61:91-105, 1971

4. Ayers AB, Fitchett DH: Hepatic haernatoma in polyar- tentis nodosa. Br J Radiol 49:184-185. 1976

5 . Thorne MD, Bookman AAM, Stevens H: A ease of polyarteritis presenting as abrupt onset of pancreatic insufficiency. J Rheumatol 7:583-586. 1980

6. Chuang VP, Wallace S, Gianturco C: A new improved coil for tapered tip catheter for arterial occlusion. Radi-

7. Ruben B, Katzen BT: Selective hepatic artery emboliLa- tion to control massive hepatic hemorrhage after trau- ma. J Roentgen01 1?9:253-256, 1977

8. Robins JM. Bookstein JJ: Regressing aneurysms in penartentis nodosa. Radiology 104:39-42, 1972

9. Porter LL. Houston MC, Kadir S: Mycotic aneurysms of the hepatic artery: treatment with arterial emboliza- tion. Am J Med 67:697-701, 1979

10. Koehler RE, Korobkin M , Lewis F: Arteriographic demonstration of collateral arterial supply to the liver after hepatic artery ligation. Radiology 117:49-54. 1975

84~849-860, 1960

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