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Severe abdominal vasculitis with hepatitis B antigenemia
CASE REPORTS
Severe Abdominal Vasculitis With Hepatitis I3 Antigenemia Sinn Anuras, MD, FACP, Iowa City, Iowa Brian J. McMahon, MD, Anchorage, Alaska Kenneth C. Chow, MD, Iowa City, Iowa Robert W. Summers, MD, FACP, Iowa City, Iowa
An association between hepatitis B infection and vasculitis was first noted in 1970 [I]. Since then, several reports have appeared [2-241, and some investigators believe that hepatitis B surface antigen occurs in as many as 50 percent of patients with polyarteritis nodosa. The vasculitis may involve any artery, but symptomatic abdominal vasculitis is uncommon. Patients with abdominal vasculitis have had a higher mortality from intestinal perforation than the group as a whole [25]. Most patients have crampy abdominal pain not confined to the right upper quadrant for several months to several years before diagnosis. The results of medical therapy with corticosteroid and immunosuppressive agents in those patients have varied. However, early recognition and treatment may alter the course of the disease. We present two patients to call attention to this disease, which must be suspected in any patient who has persistently positive hepatitis B surface antigen and crampy abdominal pain not confined to the right upper quadrant or liver area. Case Reports Case I. A 47 year old man was followed at the University of Iowa Hospitals since 1971. He was treated initially for hairy cell leukemia with two doses of cytoxan, vincristine and prednisone. He remained on maintenance therapy with prednisone, 20 mg daily, until July 1973, when it was tapered and stopped. Since his first admission, he had had mild persistent elevations of alkaline phosphatase and serum glutamic oxalacetic transaminase. In June 1972, From the Departments of Medicine and Radiology, University of Iowa, Iowa City, Iowa. This study was supported by Biomedical Research Support Grant RR 05372 from the Biomedical Research Support Branch, Division of Research Facilities and Resources, National Institutes of Health, Bethesda, Maryland. Requests for reprints should be addressed to Sinn Anuras. MD, De-t of Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa 55242.
692
hepatitis B surface antigen was first noted to be positive. In 1974 the patient first began to complain of chronic postprandial cramping abdominal pain. At first the pain was diffuse, mild and intermittent, but it slowly progressed and finally emergency surgery for suspected appendicitis was required in August 1976. At laparotomy, multiple perforations were found in the terminal ileum and colon in association with extensive necrosis of the bowel. Pathologic examination disclosed acute inflammatory infiltrate throughout the bowel wall and thickening of the small arterial media with focal areas of inflammation diagnosed as extensive necrotizing vasculitis (Figure 1). Postoperatively angiography revealed narrowing and irregularity of both the superior and inferior mesenteric arteries with more severe involvement of the inferior mesenteric artery. After temporary relief of pain, the symptom complex of postprandial cramping recurred several months later and again progressed over the next 2 years. In April 1978, the patient returned with intractable upper abdominal pain requiring narcotics; the pain started about 30 minutes after a meal and lasted several hours with gradual relief. He especially avoided fatty foods and large meals and lost 30 pounds. In addition, severe hypertension developed in the range of 200/130 to 150 mm Hg. Attempts to control hypertension with multiple drugs produced severe aggravation of the abdominal pain, In 1978, the complete blood count was within normal limits and the erythrocyte sedimentation rate was 6 mm/hour. Serum electrolytes, blood urea nitrogen and creatinine were within normal limits, as were the results of all liver function studies. Chest x-rays showed only minimal right apical scarring. The findings on oral cholecystography and upper gastrointestinal and small bowel series were normal except for the absence of the terminal ileum and right colon. Serum amylase was 185 units/100 ml (normal 40 to 200), and 24 hour urinary analyses for vanillylmandelic acid, catecholamines and metanephrines were also within normal limits. The serum C3 and C4 were within normal limits. Hepatitis B surface antigen was positive.
The American Journal of Surgery
Abdominal Vasculitis With Hepatitis B
Angiography (Figures 2 and 3) revealed the normal caliber of the proximal segment of the superior mesenteric artery in sharp contrast to the abrupt, irregular tapering of its first division branches. Several first division branches and many distal branches were occluded at or near their origins and distal flow was through a complex network of
Ftgure 1. Terminal iieai biopsy. There was thickening and infiammation of the small arteries of terminal ileum. (Hematoxyiin-eostn stain; magnification X100, reduced 47 percent. )
Figure 2. Abdominal aortogram reveals marked attenuation of caliber and irregular contour of the intrarenai arteries beyond the segmental branches and several small aneurysms of the interiobar arteries. in addition, a very tiny irregular arterial channel is noted parallel to the inferior margin of the hepatic artery proper.
Figure 3. Selective arleriogram of the superior mesenteric artery demonstrates striking occlusion of several first- through third-order bran&es and multiple tortuous and narrow collateral channels. Two parallel collateral channels (arrowheads) appear to run parallel to the course of the proximaiiy occluded first-order jejunai branch. The arrow indicates the largest of several saccuiar aneurysms.
Volume 140, November 1990
693
Anuras et al
fine, tortuous collateral vessels. In several areas, these collateral vessels appeared to run parallel to the apparent course of the obstructed artery, suggesting recanalization within the wall of the occluded vessel. A similar, narrow channel was noted on the aortogram running parallel to an unobstructed hepatic artery. Several small aneurysms were also present. The marked irregularities in contour, the arterial occlusions, the aneurysms and the complex network of collaterals indicated very severe vasculitis. Although these features can be seen in vasculitis associated with polyarteritis nodosa or drug abuse, sparing of the proximal vessels and involvement of the distal vessels would be expected. The patient had been receiving 20 mglday of prednisone, which was increased to 80 mglday in the hospital with no change in the patient’s symptoms. Attempts to use antihypertensive drugs with a vasodilating effect were not successful. Initially the patient responded dramatically to the use of 15 mg of propantheline before each meal. However, the response was only temporary, and narcotics were again required for relief of pain. After this an elemental diet with a continuous infusion of Vivonexe, 1 calorie/cc was started. The patient was able to take in at least 2,000 calories/day with partial relief of symptoms. Long-term parenteral nutrition was planned, but an acute abdominal condition developed suddenly and the patient died from massive bowel infarction. Case II. A 50 year old white woman noted onset of a dry cough and fever in April 1976. In July she was admitted for evaluation by her physician. Serum glutamic oxalacetic transaminase was 140 III/liter, alkaline phosphatase was 135 mu/ml, and hepatitis B surface antigen was positive. Serum glutamic oxalacetic transaminase increased to 330 IU/liter and alkaline phosphatase to 625 mu/ml. A liver biopsy performed on August 11, 1976, showed chronic persistent hepatitis. A high fever developed and prednisone therapy, 5 mg four times a day, was started in late August. Five days later nausea and pain in the right lower quadrant of the abdomen developed. The symptoms progressed and in early September exploratory laparotomy was performed because of persistent lower abdominal pain and rebound tenderness in the right lower abdomen. At surgery, a large abcess from cecal perforation was found as well as a small bowel obstruction secondary to adhesions; a diverting ileostomy was performed. Since stones were palpable in the gallbladder, cholecystectomy was done as well as a wedge liver biopsy. Biopsy of the gallbladder showed necrotizing vasculitis. Liver biopsy showed thickening of the walls of the small arteries and increased fibrosis and mononuclear cell infiltration in the portal tracts. No biopsy was taken from the perforated
cecum. After an uncomplicated postoperative course, steroid therapy was tapered. However, the patient again became febrile. Prednisone therapy was started again and within 24 hours she was afebrile. She was then transferred to the University of Iowa Hospitals. Physical examination disclosed a thin, wasted woman with a healing midline surgical scar, a T tube and an ile-
694
ostomy. The liver was nontender and normal in size. Hemoglobin was 12 g/dl, white blood count 18,100/mm3 with a left shift, platelets 1,202,000/mm3, albumin 3.3 g/d1 and alkaline phosphatase 337 IU/liter. The remainder of the SMA 12/60,6/60 as well as amylase clearance were within normal limits. Prednisone therapy was stopped, and within 48 hours fever of up to 39°C developed. Multiple blood, urine, sputum and bile cultures were negative. Venereal disease reaction level, antinuclear antibody, C3, C4, antismooth muscle antibody and antimitochondrial antibody were all within normal limits. Serum and bile were both positive for hepatitis B surface antigen. Prednisone therapy was restarted, 40 mg daily in divided doses, and the patient became afebrile within 24 hours. Since discharge several attempts to decrease steroid dosage have resulted in episodes of abdominal paia, fever and leukocytosis.
Comments There are 102 reported cases of vasculitis associB antigenemia [l-24]. The association between viral hepatitis and necrotizing vasculitis has been known since World War II, when four cases were reported after yellow fever vaccination [26]. Vasculitis with hepatitis B was first described by Gocke et al, who presented four cases in 1970 [I]. Shortly after that, Trepo et al [17] tested the serum of 55 patients with proven polyarteritis and found hepatitis B surface antigen to be present in 30 of them [17]. For a short time it was thought that hepatitis B antigenemia was responsible for a significant percentage of cases of vasculitis. However, in more recent reviews of polyarteritis, hepatitis B surface antigen was present in only 3 of 30 [19] and 1 of 16 cases [18]. Hence the incidence of hepatitis B antigen in patients with polyarteritis is unknown. At least six patients had abdominal symptoms attributable to vasculitis. Three presented with an acute abdominal condition. The other three had chronic, intermittent, crampy abdominal pain. Diagnosis was established by laparotomy in three, angiography in two and rectal biopsy in one. It is difficult to evaluate the efficacy of corticosteroid and some immunosuppressive agents on vasculitis with hepatitis B because the number of patients is small and the regimens vary considerably from one medical center to another [2-101. As expected, the results have been conflicting. However, 75 percent of those treated showed some evidence of clinical improvement, and in 25 percent the disease was arrested [25]. There was no significant difference in response to therapy in those treated with prednisone alone and those treated with prednisone and immunosuppressive drugs. Patients who presented with abdominal vasculitis had a higher mortality and ated with hepatitis
The American Journal of Surgery
Abdominal
responded to therapy less often [%I. In four patients, abdominal pain was the only clinical symptom; vasculitis was not initially considered the cause of the pain in these patients. Delaying the diagnosis allowed the disease to progress unchecked. Five patients eventually had intestinal perforation and required a major surgical procedure, with all of its attenuated operative and postoperative risk. Any of the aforementioned reasons may account for the higher mortality in this group of patients. All eight patients who presented with abdominal vasculitis had abnormal liver enzymes sometime during the course of their illness. Hence, the combination of abnormal liver enzymes and either acute postprandial abdominal pain not localizing to the right upper quadrant or protracted diffuse crampy abdominal pain should alert the physician to the possibility of vasculitis with hepatitis B, and a blood specimen for hepatitis B surface antigen should be obtained. If this is positive, the next step is arteriographv - _ _ of the mesenteric vessels and rectal bionsv. which should establish the diagnosis. After diagnosis, antiinflammatory therapy, either prednisone alone or prednisone and immunosuppressive arents. should be-initiated to prevent irre&sible damage to the blood vessels. I
.zI
Summary Two cases of necrotizing abdominal vasculitis associated with hepatitis B are reported. The diagnosis of mesenteric vasculitis is often delayed, and intestinal perforation is common. Early recognition of the disease and early treatment with corticosteroid and immunosuppressive drugs may alter the course of the disease. Preoperative diagnosis is best made by mesenteric arteriography and rectal biopsy. References 1. Gocke DJ, Hsu K, Morgan C, Bombardieri S, Lockshin M, Christian CL. Association between polyarteritis and Australian antigen. Lancet 1970;2:1149. 2. Sergent J, Lockshin M, Christian C, Gocke D. Vasculitis with hepatitis B antigenemia. Medicine 1976;55:1. 3. Trepo C, Thivolet J, Lamberi R. Four cases of periarteritis nodosa associated with persistent Australian antigen. Digestion 1972;5: 100.
Volume 140, November 1990
Vasculitis
With Hepatitis
B
4. Duffy J, Lidsky M, Sharp J, et al. Polyarthritis, polyarteritis and hepatitis B. Medicine 1976;55:19. 5. Thompson DM, Heaf P, Robinson TW. Australian antigen polyarteritis treated with prednisone and Dapsone. Proc Roy Sot Med 1976;69:389. 6. Touillas G, Aimard G, Trepo C, Rambaud G, Tommasi M, Devic M. Neuropathy associated with the Australian antigen and periarteritis nodosa. Rev Neurol 1973;126:201. 7. Razzak I, Bauer F, ltzel W. Hepatitis-B-antigenemia with panarteritis, diffuse proliferative glomerulonephritis and malignant hypertension. Am J Gastroenterol 1975;63: 476. 8. Gerber M, Brodin A. Steinberg D, Vernace S, Yang D-P, Paronetto F. Periarteritis nodosa Australian antigen and lymphatic leukemia. N Engl J Med 1972;286:14. 9. Baker A, Kaplan M, Benz W, Side1 JS, Wolfe HJ. Polyarteritis associated with Australian antigen-positive hepatitis. Gastroenterology 1972;62:105. 10. Koff R, Widrich W, Robbins A. Necrotizing angiitis in a methamphetamine user with hepatitis B. N Engl J Med 1973;288:946. 11. Trepo C, Thivolet J. Hepatitis associated antigen and periarteritis nodosa. VOX Sang 1970;19:410. 12. Trepo C, Thivolet J, Prince A. Australian antigen and polyarteritis nodosa. Am J Dis Child 1972;123:390. 13. Kew MC, Marcus RS, Macnab C, Bersohn I. Hepatitis B (surface) antigen in mothers and their infants. S Afr Med J 1975;49: 1471. 14. Heathcote E, Dudley F, Sherlock S. Association of polyarteritis and Australian antigen. Gut 1972; 13:319. 15. Ziff M. Viruses and the connective tissue diseases. Ann Intern Med 1971;73:951. 16. Miller J, Robinson J, Panitch M, Soike K. Collagen diseases, hepatitis associated antigen and the Epstein-Barr virus. JAMA 1972;221:1517. 17. Trepo C, Zuckerman A, Bird R, Prince A. Role of circulating hepatitis B antigen/antibody immune complexes in the pathogenesis of vascular and hepatic manifestations in polyarteritis nodosa. J Clin Pathol 1974;27:863. 18. Sack M, Cassidy J, Bole G. Prognostic factors in polyarteritis. J Rheumatol 1975;2:411. 19. Conn D, McDuffie F, Holley K, Schroeter AL. Immunologic mechanisms in systemic vasculitis. Mayo Clin Proc 1976; 51:511. 20. Kohler P. Clinical immune complex disease. Medicine 1973; 52:419. 2 1. Boyer T, Tong J, Rakela J, Reynolds TB. Immunologic studies and clinical follow-up HB,Ag positive polyarteritis nodosa. Dia Dis 1977:22:497. 22. Fisher R, Graham D, Grammaych M, Travanino JG. Polyarteritis nodosa and hepatitis B surface antigen: role of angiography in diagnosis. AJR 1977; 129:77. 23. De Leo D. Periarteritis nodosa with a positive Australian antigen in a two year old. Alaska Med 1977:19:15. 24. Richlin D, Saltzman D, Wills J. Necrotizing angiitis and hepatitis in an amphetamine abuser. Del Med J 1977;49:469. 25. McMahon BJ, Meek ES, Anuras S. Vasculitis with hepatitis B: a review of the literature. J Iowa Med Sot (in press). 26. Paul1 R. Periarteritis nodosa with report of four proven cases. Calif Med 1947;67:309.
695
Severe Abdominal Vasculitis With Hepatitis I3 Antigenemia Sinn Anuras, MD, FACP, Iowa City, Iowa Brian J. McMahon, MD, Anchorage, Alaska Kenneth C. Chow, MD, Iowa City, Iowa Robert W. Summers, MD, FACP, Iowa City, Iowa
An association between hepatitis B infection and vasculitis was first noted in 1970 [I]. Since then, several reports have appeared [2-241, and some investigators believe that hepatitis B surface antigen occurs in as many as 50 percent of patients with polyarteritis nodosa. The vasculitis may involve any artery, but symptomatic abdominal vasculitis is uncommon. Patients with abdominal vasculitis have had a higher mortality from intestinal perforation than the group as a whole [25]. Most patients have crampy abdominal pain not confined to the right upper quadrant for several months to several years before diagnosis. The results of medical therapy with corticosteroid and immunosuppressive agents in those patients have varied. However, early recognition and treatment may alter the course of the disease. We present two patients to call attention to this disease, which must be suspected in any patient who has persistently positive hepatitis B surface antigen and crampy abdominal pain not confined to the right upper quadrant or liver area. Case Reports Case I. A 47 year old man was followed at the University of Iowa Hospitals since 1971. He was treated initially for hairy cell leukemia with two doses of cytoxan, vincristine and prednisone. He remained on maintenance therapy with prednisone, 20 mg daily, until July 1973, when it was tapered and stopped. Since his first admission, he had had mild persistent elevations of alkaline phosphatase and serum glutamic oxalacetic transaminase. In June 1972, From the Departments of Medicine and Radiology, University of Iowa, Iowa City, Iowa. This study was supported by Biomedical Research Support Grant RR 05372 from the Biomedical Research Support Branch, Division of Research Facilities and Resources, National Institutes of Health, Bethesda, Maryland. Requests for reprints should be addressed to Sinn Anuras. MD, De-t of Medicine, University of Iowa Hospitals and Clinics, Iowa City, Iowa 55242.
692
hepatitis B surface antigen was first noted to be positive. In 1974 the patient first began to complain of chronic postprandial cramping abdominal pain. At first the pain was diffuse, mild and intermittent, but it slowly progressed and finally emergency surgery for suspected appendicitis was required in August 1976. At laparotomy, multiple perforations were found in the terminal ileum and colon in association with extensive necrosis of the bowel. Pathologic examination disclosed acute inflammatory infiltrate throughout the bowel wall and thickening of the small arterial media with focal areas of inflammation diagnosed as extensive necrotizing vasculitis (Figure 1). Postoperatively angiography revealed narrowing and irregularity of both the superior and inferior mesenteric arteries with more severe involvement of the inferior mesenteric artery. After temporary relief of pain, the symptom complex of postprandial cramping recurred several months later and again progressed over the next 2 years. In April 1978, the patient returned with intractable upper abdominal pain requiring narcotics; the pain started about 30 minutes after a meal and lasted several hours with gradual relief. He especially avoided fatty foods and large meals and lost 30 pounds. In addition, severe hypertension developed in the range of 200/130 to 150 mm Hg. Attempts to control hypertension with multiple drugs produced severe aggravation of the abdominal pain, In 1978, the complete blood count was within normal limits and the erythrocyte sedimentation rate was 6 mm/hour. Serum electrolytes, blood urea nitrogen and creatinine were within normal limits, as were the results of all liver function studies. Chest x-rays showed only minimal right apical scarring. The findings on oral cholecystography and upper gastrointestinal and small bowel series were normal except for the absence of the terminal ileum and right colon. Serum amylase was 185 units/100 ml (normal 40 to 200), and 24 hour urinary analyses for vanillylmandelic acid, catecholamines and metanephrines were also within normal limits. The serum C3 and C4 were within normal limits. Hepatitis B surface antigen was positive.
The American Journal of Surgery
Abdominal Vasculitis With Hepatitis B
Angiography (Figures 2 and 3) revealed the normal caliber of the proximal segment of the superior mesenteric artery in sharp contrast to the abrupt, irregular tapering of its first division branches. Several first division branches and many distal branches were occluded at or near their origins and distal flow was through a complex network of
Ftgure 1. Terminal iieai biopsy. There was thickening and infiammation of the small arteries of terminal ileum. (Hematoxyiin-eostn stain; magnification X100, reduced 47 percent. )
Figure 2. Abdominal aortogram reveals marked attenuation of caliber and irregular contour of the intrarenai arteries beyond the segmental branches and several small aneurysms of the interiobar arteries. in addition, a very tiny irregular arterial channel is noted parallel to the inferior margin of the hepatic artery proper.
Figure 3. Selective arleriogram of the superior mesenteric artery demonstrates striking occlusion of several first- through third-order bran&es and multiple tortuous and narrow collateral channels. Two parallel collateral channels (arrowheads) appear to run parallel to the course of the proximaiiy occluded first-order jejunai branch. The arrow indicates the largest of several saccuiar aneurysms.
Volume 140, November 1990
693
Anuras et al
fine, tortuous collateral vessels. In several areas, these collateral vessels appeared to run parallel to the apparent course of the obstructed artery, suggesting recanalization within the wall of the occluded vessel. A similar, narrow channel was noted on the aortogram running parallel to an unobstructed hepatic artery. Several small aneurysms were also present. The marked irregularities in contour, the arterial occlusions, the aneurysms and the complex network of collaterals indicated very severe vasculitis. Although these features can be seen in vasculitis associated with polyarteritis nodosa or drug abuse, sparing of the proximal vessels and involvement of the distal vessels would be expected. The patient had been receiving 20 mglday of prednisone, which was increased to 80 mglday in the hospital with no change in the patient’s symptoms. Attempts to use antihypertensive drugs with a vasodilating effect were not successful. Initially the patient responded dramatically to the use of 15 mg of propantheline before each meal. However, the response was only temporary, and narcotics were again required for relief of pain. After this an elemental diet with a continuous infusion of Vivonexe, 1 calorie/cc was started. The patient was able to take in at least 2,000 calories/day with partial relief of symptoms. Long-term parenteral nutrition was planned, but an acute abdominal condition developed suddenly and the patient died from massive bowel infarction. Case II. A 50 year old white woman noted onset of a dry cough and fever in April 1976. In July she was admitted for evaluation by her physician. Serum glutamic oxalacetic transaminase was 140 III/liter, alkaline phosphatase was 135 mu/ml, and hepatitis B surface antigen was positive. Serum glutamic oxalacetic transaminase increased to 330 IU/liter and alkaline phosphatase to 625 mu/ml. A liver biopsy performed on August 11, 1976, showed chronic persistent hepatitis. A high fever developed and prednisone therapy, 5 mg four times a day, was started in late August. Five days later nausea and pain in the right lower quadrant of the abdomen developed. The symptoms progressed and in early September exploratory laparotomy was performed because of persistent lower abdominal pain and rebound tenderness in the right lower abdomen. At surgery, a large abcess from cecal perforation was found as well as a small bowel obstruction secondary to adhesions; a diverting ileostomy was performed. Since stones were palpable in the gallbladder, cholecystectomy was done as well as a wedge liver biopsy. Biopsy of the gallbladder showed necrotizing vasculitis. Liver biopsy showed thickening of the walls of the small arteries and increased fibrosis and mononuclear cell infiltration in the portal tracts. No biopsy was taken from the perforated
cecum. After an uncomplicated postoperative course, steroid therapy was tapered. However, the patient again became febrile. Prednisone therapy was started again and within 24 hours she was afebrile. She was then transferred to the University of Iowa Hospitals. Physical examination disclosed a thin, wasted woman with a healing midline surgical scar, a T tube and an ile-
694
ostomy. The liver was nontender and normal in size. Hemoglobin was 12 g/dl, white blood count 18,100/mm3 with a left shift, platelets 1,202,000/mm3, albumin 3.3 g/d1 and alkaline phosphatase 337 IU/liter. The remainder of the SMA 12/60,6/60 as well as amylase clearance were within normal limits. Prednisone therapy was stopped, and within 48 hours fever of up to 39°C developed. Multiple blood, urine, sputum and bile cultures were negative. Venereal disease reaction level, antinuclear antibody, C3, C4, antismooth muscle antibody and antimitochondrial antibody were all within normal limits. Serum and bile were both positive for hepatitis B surface antigen. Prednisone therapy was restarted, 40 mg daily in divided doses, and the patient became afebrile within 24 hours. Since discharge several attempts to decrease steroid dosage have resulted in episodes of abdominal paia, fever and leukocytosis.
Comments There are 102 reported cases of vasculitis associB antigenemia [l-24]. The association between viral hepatitis and necrotizing vasculitis has been known since World War II, when four cases were reported after yellow fever vaccination [26]. Vasculitis with hepatitis B was first described by Gocke et al, who presented four cases in 1970 [I]. Shortly after that, Trepo et al [17] tested the serum of 55 patients with proven polyarteritis and found hepatitis B surface antigen to be present in 30 of them [17]. For a short time it was thought that hepatitis B antigenemia was responsible for a significant percentage of cases of vasculitis. However, in more recent reviews of polyarteritis, hepatitis B surface antigen was present in only 3 of 30 [19] and 1 of 16 cases [18]. Hence the incidence of hepatitis B antigen in patients with polyarteritis is unknown. At least six patients had abdominal symptoms attributable to vasculitis. Three presented with an acute abdominal condition. The other three had chronic, intermittent, crampy abdominal pain. Diagnosis was established by laparotomy in three, angiography in two and rectal biopsy in one. It is difficult to evaluate the efficacy of corticosteroid and some immunosuppressive agents on vasculitis with hepatitis B because the number of patients is small and the regimens vary considerably from one medical center to another [2-101. As expected, the results have been conflicting. However, 75 percent of those treated showed some evidence of clinical improvement, and in 25 percent the disease was arrested [25]. There was no significant difference in response to therapy in those treated with prednisone alone and those treated with prednisone and immunosuppressive drugs. Patients who presented with abdominal vasculitis had a higher mortality and ated with hepatitis
The American Journal of Surgery
Abdominal
responded to therapy less often [%I. In four patients, abdominal pain was the only clinical symptom; vasculitis was not initially considered the cause of the pain in these patients. Delaying the diagnosis allowed the disease to progress unchecked. Five patients eventually had intestinal perforation and required a major surgical procedure, with all of its attenuated operative and postoperative risk. Any of the aforementioned reasons may account for the higher mortality in this group of patients. All eight patients who presented with abdominal vasculitis had abnormal liver enzymes sometime during the course of their illness. Hence, the combination of abnormal liver enzymes and either acute postprandial abdominal pain not localizing to the right upper quadrant or protracted diffuse crampy abdominal pain should alert the physician to the possibility of vasculitis with hepatitis B, and a blood specimen for hepatitis B surface antigen should be obtained. If this is positive, the next step is arteriographv - _ _ of the mesenteric vessels and rectal bionsv. which should establish the diagnosis. After diagnosis, antiinflammatory therapy, either prednisone alone or prednisone and immunosuppressive arents. should be-initiated to prevent irre&sible damage to the blood vessels. I
.zI
Summary Two cases of necrotizing abdominal vasculitis associated with hepatitis B are reported. The diagnosis of mesenteric vasculitis is often delayed, and intestinal perforation is common. Early recognition of the disease and early treatment with corticosteroid and immunosuppressive drugs may alter the course of the disease. Preoperative diagnosis is best made by mesenteric arteriography and rectal biopsy. References 1. Gocke DJ, Hsu K, Morgan C, Bombardieri S, Lockshin M, Christian CL. Association between polyarteritis and Australian antigen. Lancet 1970;2:1149. 2. Sergent J, Lockshin M, Christian C, Gocke D. Vasculitis with hepatitis B antigenemia. Medicine 1976;55:1. 3. Trepo C, Thivolet J, Lamberi R. Four cases of periarteritis nodosa associated with persistent Australian antigen. Digestion 1972;5: 100.
Volume 140, November 1990
Vasculitis
With Hepatitis
B
4. Duffy J, Lidsky M, Sharp J, et al. Polyarthritis, polyarteritis and hepatitis B. Medicine 1976;55:19. 5. Thompson DM, Heaf P, Robinson TW. Australian antigen polyarteritis treated with prednisone and Dapsone. Proc Roy Sot Med 1976;69:389. 6. Touillas G, Aimard G, Trepo C, Rambaud G, Tommasi M, Devic M. Neuropathy associated with the Australian antigen and periarteritis nodosa. Rev Neurol 1973;126:201. 7. Razzak I, Bauer F, ltzel W. Hepatitis-B-antigenemia with panarteritis, diffuse proliferative glomerulonephritis and malignant hypertension. Am J Gastroenterol 1975;63: 476. 8. Gerber M, Brodin A. Steinberg D, Vernace S, Yang D-P, Paronetto F. Periarteritis nodosa Australian antigen and lymphatic leukemia. N Engl J Med 1972;286:14. 9. Baker A, Kaplan M, Benz W, Side1 JS, Wolfe HJ. Polyarteritis associated with Australian antigen-positive hepatitis. Gastroenterology 1972;62:105. 10. Koff R, Widrich W, Robbins A. Necrotizing angiitis in a methamphetamine user with hepatitis B. N Engl J Med 1973;288:946. 11. Trepo C, Thivolet J. Hepatitis associated antigen and periarteritis nodosa. VOX Sang 1970;19:410. 12. Trepo C, Thivolet J, Prince A. Australian antigen and polyarteritis nodosa. Am J Dis Child 1972;123:390. 13. Kew MC, Marcus RS, Macnab C, Bersohn I. Hepatitis B (surface) antigen in mothers and their infants. S Afr Med J 1975;49: 1471. 14. Heathcote E, Dudley F, Sherlock S. Association of polyarteritis and Australian antigen. Gut 1972; 13:319. 15. Ziff M. Viruses and the connective tissue diseases. Ann Intern Med 1971;73:951. 16. Miller J, Robinson J, Panitch M, Soike K. Collagen diseases, hepatitis associated antigen and the Epstein-Barr virus. JAMA 1972;221:1517. 17. Trepo C, Zuckerman A, Bird R, Prince A. Role of circulating hepatitis B antigen/antibody immune complexes in the pathogenesis of vascular and hepatic manifestations in polyarteritis nodosa. J Clin Pathol 1974;27:863. 18. Sack M, Cassidy J, Bole G. Prognostic factors in polyarteritis. J Rheumatol 1975;2:411. 19. Conn D, McDuffie F, Holley K, Schroeter AL. Immunologic mechanisms in systemic vasculitis. Mayo Clin Proc 1976; 51:511. 20. Kohler P. Clinical immune complex disease. Medicine 1973; 52:419. 2 1. Boyer T, Tong J, Rakela J, Reynolds TB. Immunologic studies and clinical follow-up HB,Ag positive polyarteritis nodosa. Dia Dis 1977:22:497. 22. Fisher R, Graham D, Grammaych M, Travanino JG. Polyarteritis nodosa and hepatitis B surface antigen: role of angiography in diagnosis. AJR 1977; 129:77. 23. De Leo D. Periarteritis nodosa with a positive Australian antigen in a two year old. Alaska Med 1977:19:15. 24. Richlin D, Saltzman D, Wills J. Necrotizing angiitis and hepatitis in an amphetamine abuser. Del Med J 1977;49:469. 25. McMahon BJ, Meek ES, Anuras S. Vasculitis with hepatitis B: a review of the literature. J Iowa Med Sot (in press). 26. Paul1 R. Periarteritis nodosa with report of four proven cases. Calif Med 1947;67:309.
695