Polyarteritis Nodosa: Spectrum of Angiographic

EDUCATION EXHIBIT 151 Polyarteritis Nodosa: Spectrum of Angiographic Findings1 Anthony W. Stanson, MD • Jeremy L. Friese, BS • C. Michael Johnson, M...
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EDUCATION EXHIBIT

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Polyarteritis Nodosa: Spectrum of Angiographic Findings1 Anthony W. Stanson, MD • Jeremy L. Friese, BS • C. Michael Johnson, MD • Michael A. McKusick, MD • Jerome F. Breen, MD • Enrique A. Sabater, MD • James C. Andrews, MD The purpose of this study was to review the positive angiographic findings in patients with polyarteritis nodosa (PAN). The authors reviewed the angiograms of 56 consecutive patients (25 women and 31 men; age range, 18–81 years; mean age, 55 years) with PAN and arterial abnormalities consistent with necrotizing vasculitis. Aneurysms were present in 27 patients and segments of ectasia were present in seven patients, for a total of 34 (61%) of 56 patients with aneurysmal lesions. The remaining 22 (39%) patients had arterial lesions that were occlusive: luminal irregularity, stenosis, or occlusion. All but one of the patients with an aneurysm also had occlusive lesions. Therefore, 55 (98%) of the 56 patients were found to have occlusive lesions. Skeletal muscle arteries were affected in 18 patients, nine in the extremities. The most frequent finding in patients with PAN was occlusive arterial lesions. Although the presence of aneurysms increases specificity for the diagnosis of PAN, many patients have only occlusive lesions. Involvement of skeletal muscle arteries was common.

Abbreviation: PAN = polyarteritis nodosa Index terms: Arteries, splenic, 954.621 • Arteries, superior mesenteric, 955.621 • Arteritis, 9*.6212 • Hepatic arteries, 952.621 • Renal arteries, 961.621 RadioGraphics 2001; 21:151–159 1From the Department of Vascular and Interventional Radiology, Mayo Clinic, 200 First St SW, Rochester, MN 55905. Recipient of a Certificate of Merit award for a scientific exhibit at the 1999 RSNA scientific assembly. Received April 3, 2000; revision requested May 9 and received June 27; accepted June 29. Address correspondence to A.W.S. (e-mail: [email protected]). 29*.Vascular ©RSNA,

system, location unspecified

2001

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Introduction Polyarteritis nodosa (PAN) is one of a spectrum of diseases that belongs to the pathologic category of necrotizing vasculitis. PAN is characterized by the presence of inflammatory reactions of blood vessels of medium or small caliber that lead to necrosis and destruction of the walls of vessels. Diseases included in this group are Churg-Strauss syndrome, microscopic PAN, Kawasaki disease, rheumatoid vasculitis, Wegener granulomatosis, and hypersensitivity vasculitis (1,2). The diagnosis is ideally made by means of biopsy of involved tissue in a patient with the appropriate clinical symptoms and laboratory data, but an angiogram provides the proof in some cases. Most patients with PAN have positive angiographic evidence of their disease, predominantly in the visceral arteries but also in arteries of the extremities and in small branches of the aorta. The most well-known angiographic feature is the presence of so-called microaneurysms in medium or small arteries. Arterial occlusive lesions are also a feature, but their frequency is not reported. In this article, the epidemiology, pathology, clinical features, and diagnosis of PAN are presented. In addition, the design and results of our study of patients with PAN are discussed.

Epidemiology PAN occurs twice as frequently in men as in women, and it is found in all age groups but most commonly in the 5th–7th decades. The estimated annual frequency ranges from four to nine per million people in the general population to more than 70 per million people in the population of patients with hepatitis B virus (3). In studies reported from France (4,5), the percentage of cases of PAN attributed to hepatitis B viral infection decreased from 36% to 7% during the past decade after the development of vaccines against viral hepatitis.

Pathology The cause of PAN is unknown for most patients. Viruses play a role in the pathogenesis in some cases, most notably hepatitis B virus (6,7) but also human immunodeficiency virus (8,9). PAN

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is a focal panmural necrotizing vasculitis in small and medium sized arteries (and sometimes small veins) that can involve any organ and in varying degrees. The kidneys may be involved in 70%– 80% of cases; the gastrointestinal tract, peripheral nerves, and skin in 50%; skeletal muscles and mesentery in 30%; and the central nervous system in 10% (10). The heart, testicles, lung, and spleen are rarely involved. Arterial inflammation is thought to be a result of immune-complex deposition, which is frequently found in the circulation but rarely found in the involved tissues.

Clinical Features Recognition of PAN can be a clinical challenge given its varied spectrum of organ involvement, wide range of clinical symptoms, and variations in severity. The clinical course lasts several months to more than a year. Relapse occurs in 40% of treated patients, with a median interval of 33 months (11). The disease may be fulminant; if untreated, the 5-year survival is less than 15%. Survival increases to 80% with steroid treatment, with or without cytotoxic drugs (12,13). Most tests are nonspecific, although the erythrocyte sedimentation rate is usually elevated. Fever, malaise, and weight loss are common. Many of the clinical symptoms are related to organ ischemia secondary to arterial branch occlusions. Aneurysm rupture is a less common cause of pain. Arthralgias are noted in 50% of patients as are peripheral neuropathies (mononeuritis multiplex), which are often symptomatic early. Renal involvement including proteinuria and hypertension are found in 75% (14). Branch vessel occlusions can lead to multiple renal infarcts. Abdominal pain from ischemia or infarction secondary to occlusive lesions is the most common gastrointestinal complaint. Abdominal pain may also be caused by rupture of an aneurysm in one of the viscera: liver, kidney, or mesentery (15). Cutaneous lesions include palpable purpura, infections, and ischemic ulcers. Muscle pain and limb claudication may occur.

Diagnosis Early diagnosis and treatment of PAN are necessary to prevent serious organ damage. Diagnosis is often delayed because of the diverse symptoms of

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Angiographic Findings in Patients with PAN Arterial Bed Distribution Gastric Gastroduodenal Hepatic Splenic Superior mesenteric artery Inferior mesenteric artery Left renal Right renal Intercostal Lumbar Upper extremity Lower extremity

No. of Patients with Disease Occlusive

Aneurysm

Ectasia

Percentage of Patients with Positive Findings

8 1 29 15

4 3 11 8

4 0 4 1

133 (12 of 36) 18 (3 of 36) 189 (32 of 36) 147 (17 of 36)

37

13

7

197 (38 of 39)

13 34 32 3 7 7 3

4 10 13 3 2 2 1

3 4 3 2 3 1 1

100 (14 of 14) 192 (36 of 39) 187 (34 of 39) 24 (4 of 17) 47 (8 of 17) 100 (9 of 9) 175 (3 of 4)

the disease. PAN should be suspected in patients with a febrile illness, weight loss, and evidence of multiple organ involvement. Establishment of the differential diagnosis may be difficult, however, and includes infectious diseases and occult malignancy. The diagnostic criteria of PAN have been classified by the American College of Rheumatology (16). Three of the 10 criteria must be present for the diagnosis of PAN. A positive angiogram with typical findings is one of the 10 criteria. A definitive diagnosis may be made in certain clinical settings by performing tissue biopsy from a symptomatic organ site, but sampling errors and lack of disease specificity may be problematic. The role of angiography is to help confirm or support the clinical impression when a suitable biopsy site is lacking or when the biopsy results are inconclusive. In patients with generalized systemic symptoms—especially abdominal complaints, nephropathy, hypertension, or generalized malaise—angiography is a valuable diagnostic tool that can lead to the diagnosis in occult cases. Angiographic findings, including aneurysms, ectasia, or occlusive disease, are present in about 40%–90% of patients at the time clinical symptoms appear (17). Unfortunately, a few other types of vasculitis, such as rheumatoid vasculitis,

systemic lupus erythematosus, and Churg-Strauss syndrome, may have similar angiographic findings. Thus, correlation with the clinical evaluation is important. PAN may also be a manifestation of rheumatoid arthritis (18,19), and similar angiographic lesions are found in some cases of drug abuse (20).

Clinical Study All patients seen at our institution between 1975 and 1998 with a clinical diagnosis of PAN at discharge and with peripheral angiograms (conventional and intraarterial digital subtraction angiograms) with positive findings of vasculitis available for review were included in this study. There were 56 consecutive patients (31 men and 25 women; age range, 18–81 years; mean age, 55 years). Patients with neuroangiographic involvement were not included. Each angiographic study was examined for arterial lesions of aneurysms, ectasia, and occlusive disease (Table). A wide range of anatomic areas was included on the angiograms. The patient’s clinical symptoms usually directed the focus of the examination,

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a. b. Figure 1. Microaneurysms in a 33-year-old man with PAN. Angiograms demonstrate multiple microaneurysms (arrows) of the liver (a) and right kidney (b).

a.

c.

Figure 2. Diffuse involvement of the abdominal visceral arteries in a 63-year-old woman with PAN. (a) Angiogram depicts the superior mesenteric artery with extensive fusiform aneurysms and one large saccular aneurysm of the middle colic branch (arrow). Occlusive lesions are also evident (arrowheads). (b) Angiogram shows aneurysms of the hepatic and splenic branches (arrows). (c) Right renal arteriogram shows one small aneurysm of an upper lobar branch (arrow) and irregular ectasia of the main renal artery (arrowhead). b.

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a. b. Figure 3. Large false aneurysm in a 68-year-old woman with PAN. (a) Angiogram depicts the false aneurysm arising from the distal gastroduodenal artery (arrow). In addition, other aneurysms of the distal splenic artery and at multiple sites in branches of the hepatic artery are seen. (b) CT scan shows the false aneurysm (arrow) of the gastroduodenal artery.

b. Figure 4. Ectasia in a 61-year-old man with PAN. Angiograms reveal arterial ectasia (arrow) of the right colic artery (a) and branches (arrows) of the inferior mesenteric artery (b).

a.

which influenced the percentages of positive findings somewhat in the various arterial beds (Table). Indeed, one or more of the examined arterial beds was free of disease in 61% of the patients.

Multiple aneurysms or ectatic lesions were found in most patients with angiographic evidence of PAN (Figs 1–3). Aneurysms were present in 27 patients (48%). Arterial lesions of expansion that accounted for less than a 50% increase in diameter were classified as ectasia and were present in seven additional patients (12%) (Fig 4). In most of the 27 patients with aneurysms, segments or foci of ectasia and occlusive

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Figure 5. Occlusive lesion in a 74-year-old man with PAN. Angiogram shows stenosis of a branch of the right hepatic artery (left arrow) and an aneurysm of the middle branch of the hepatic artery (right arrow). Note the luminal irregularity in the intercostal artery (arrowhead).

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Figure 6. Occlusive lesion in a 24-year-old man with PAN. Angiogram demonstrates stenoses of the branches of the left renal artery (arrows).

a. b. Figure 7. Occlusive lesions in a 58-year-old man with PAN. (a) Angiogram obtained in the arterial phase shows partial obliteration, or pruning, of the renal arteries distally (b). Angiogram obtained in the parenchymal phase shows reduced opacity and lack of demarcation of the cortical medullary junction.

lesions were also seen (Fig 5). Therefore, aneurysmal disease was seen in 61% of the patients. The absence of aneurysms did not rule out the diagnosis of PAN, however, because the presence of other arterial abnormalities may suggest the disease. Angiograms in 22 patients (39%) in this study were positive, but they did not depict aneurysms

or any evidence of arterial ectasia. The only arterial abnormalities in these patients were occlusive lesions (luminal irregularities resulting in reduction of caliber, stenosis, or occlusion) (Figs 6–9). Including patients with aneurysms and ectasia, occlusive lesions were present in 55 of the 56 patients (98%) in this study. Only one patient with aneurysms (one large ruptured aneurysm and three small aneurysms) had no occlusive arterial lesions. Arterial complications of PAN include

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a. b. Figure 8. Occlusive disease in an 18-year-old woman with PAN. (a) Angiogram obtained in the arterial phase shows severe arterial occlusive disease of the right kidney. (b) Angiogram obtained in the parenchymal phase shows extensive infarcts.

a. b. Figure 9. Occlusive disease in a 19-year-old woman with PAN. Angiograms demonstrate occlusive disease of the proximal ileal branches of the mesentery artery (a) and branches of the hepatic artery (b).

aneurysm rupture and arterial occlusion that lead to organ infarction. In our study group, five patients (9%) experienced rupture of an aneurysm, but none of them died. Three of the ruptured aneurysms were successfully embolized by means of coils placed via a catheter. Computed tomography (CT) helped identify the site of hemorrhage and determine the possible diagnosis of PAN (Figs 3b, 10). Skeletal muscle involvement was found in 18 patients. Involvement of lumbar and intercostal arteries was found incidentally in nine of the 47 patients (19%) who underwent visceral arteriography or Figure 10. Aneurysm in an 81-year-old woman with PAN. CT scan reveals a ruptured aneurysm (arrow) in the liver.

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Figures 11, 12. Occlusive disease with skeletal muscle involvement. (11) Lateral thoracic aortogram of a 74-year-old man with PAN shows aneurysms of the upper intercostal arteries (arrows). (12) Angiogram of a 61-year-old man with PAN demonstrates stenosis of the left lumbar artery (arrow). 11.

aortography (Figs 11, 12). Nine of the 56 (16%) patients underwent arteriography to evaluate symptoms limited to the extremities, and eight of them experienced ischemia (Fig 13). Occlusive disease was predominant, but five patients also had aneurysms in the arteries of skeletal muscle.

Discussion The true frequency of specific angiographic findings and of organ system involvement by PAN was not determined in this study or in many other published studies (17,18,21). The true frequency is difficult to determine because of the nonspecificity of angiographic findings and sampling bias. Other diseases, including rheumatoid vasculitis, systemic lupus erythematosus, and ChurgStrauss syndrome, may have similar angiographic findings of aneurysms and occlusive lesions (18). Sampling bias can occur because of the natural tendency to direct the angiographic examination toward the symptomatic sites. Total abdominal angiography was seldom performed in the past, but it is commonly performed now. In a patient without advanced disease, such a practice would lead to a higher frequency of positive cases and a more accurate appraisal of organ involvement (17). High-spatial-resolution angiographic technique is important for maximizing the diagnostic potential of the procedure. The diagnostic features of PAN, such as minimal luminal irregularity or narrowing, are difficult to detect on suboptimal studies. Aneurysms may be small, difficult to detect, few in number, or isolated to one organ. A complete angiographic study of the abdomen should be considered to help detect occult aneurysms. Prophylactic treatment of large aneurysms by means of catheter embolization should be consid-

12.

ered in anticipation of the risk of rupture. Aortography may not substitute adequately for selective injections of the viscera, but the retroperitoneal branches are depicted, and they can also be involved with aneurysms that may rupture in rare cases. In a small percentage of patients with PAN, aneurysm rupture in an organ or retroperitoneal branch may be the first clinical evidence of the disease. Embolization therapy is often the treatment of choice (22,23). The presence of aneurysms may relate to the phase of the disease or its severity. Arterial segments of ectasia may be either a precursor to a fully expanded aneurysm or a phase in the healing process. Aneurysms of PAN may resolve over time as remission occurs (24).

Conclusions As seen in this review of the spectrum of angiographic findings in PAN, a variety of arterial beds may be affected and a variety of lesions may be seen. Skeletal muscle arterial disease was seen in nearly one-third of our patients. The lesions seen most frequently were occlusive. The presence of aneurysms increases the specificity of the diagnosis of PAN, but in their absence other arterial lesions such as luminal irregularities, stenoses, and occlusions can suggest the diagnosis. In a patient without fulminant disease but in whom clinical suspicion is firm, comprehensive angiography may lead to the diagnosis of PAN on the basis of the constellation of angiographic findings.

References 1. Hunder GG, Lie JT. The vasculitides. Clin Cardiovasc Dis 1983; 12:261–291. 2. Lie JT. Diagnostic histopathology of major systemic and pulmonary vasculitic syndromes. Rheum Dis Clin North Am 1990; 16:269–292.

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Figure 13. Severe obliterative disease in a 19-year-old woman with PAN. Angiograms of the arteries in the right upper thigh (arrows = deep femoral artery) (a) and in the right hand, wrist, and forearm (arrow = radial artery at the wrist) (b) show severe obliterative disease of the arteries. a.

b.

3. Conn DL. Polyarteritis. Rheum Dis Clin North Am 1990; 16:341–362. 4. Guillevin L, Le THD, Godeau P, Jais P, Wechsler B. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br J Rheumatol 1988; 27:258–264. 5. Guillevin L, Lhote F, Cohen P, et al. Polyarteritis nodosa related to hepatitis B virus: a prospective study with long-term observation of 41 patients. Medicine (Baltimore) 1995; 74:238–253. 6. Gocke DJ, Hsu K, Morgan C, Bombardieri S, Lockshin M, Christian CL. Association between polyarteritis and Australia antigen. Lancet 1970; 2:1149–1153. 7. Fisher RG, Graham DY, Granmayeh M, Trabanino JG. Polyarteritis nodosa and hepatitis-B surface antigen: role of angiography in diagnosis. AJR Am J Roentgenol 1977; 129:77–81. 8. Bardin T, Gaudouen C, Kuntz D, et al. Necrotizing vasculitis in human immunodeficiency virus infection (abstr). Arthritis Rheum 1987; 30 (suppl):S105. 9. Libman BS, Quismorio FP Jr, Stimmler MM. Polyarteritis nodosa-like vasculitis in human immunodeficiency virus infection. J Rheumatol 1995; 22: 351–355. 10. Wold LE, Baggenstoss AH. Gastrointestinal lesions of periarteritis nodosa. Proc Staff Meeting Mayo Clin 1949; 24:28–35. 11. Gordon M, Luqmani RA, Adu D, et al. Relapses in patients with a systemic vasculitis. Q J Med 1993; 86:779–789. 12. Guillevin L, Jarrousse B, Lok C, et al. Long-term follow-up after treatment of PAN and ChurgStrauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange: a prospective randomized trial of 71 patients. J Rheumatol 1991; 18:567–574. 13. Guillevin L, Fain O, Lhote F, et al. Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. Arthritis Rheum 1992; 35:208–215.

14. Cupps TR, Fauci AS. The vasculitides. Philadelphia, Pa: Saunders, 1981. 15. Jaques PF, Parker LA, Mauro MA. Fulminant systemic necrotizing arteritis: CT findings. J Comput Assist Tomogr 1988; 12:104–108. 16. Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990: criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990; 33:1088–1093. 17. Ewald EA, Griffin D, McCune WJ. Correlation of angiographic abnormalities with disease manifestations and disease severity in polyarteritis nodosa. J Rheumatol 1987; 14:952–956. 18. Hekali P, Kajander H, Pajari R, Stenman S, Somer T. Diagnostic significance of angiographically observed visceral aneurysms with regard to polyarteritis nodosa. Acta Radiol 1991; 32:143–148. 19. Valente RM, Hall S, O’Duffy JD, Conn DL. Vasculitis and related disorders. In: Kelley WN, ed. Textbook of rheumatology. Philadelphia, Pa: Saunders, 1997; 1079–1122. 20. Citron BP, Halpern M, McCarron M, et al. Necrotizing angiitis associated with drug abuse. N Engl J Med 1970; 283:1003–1011. 21. Sellar RJ, Mackay IG, Buist TAS. The incidence of microaneurysms in polyarteritis nodosa. Cardiovasc Intervent Radiol 1986; 9:123–126. 22. Bookman AAM, Goode E, McLoughlin MJ, Cohen Z. Polyarteritis nodosa complicated by a ruptured intrahepatic aneurysm. Arthritis Rheum 1983; 26:106–108. 23. Hachulla E, Bourdon F, Taieb S, et al. Embolization of two bleeding aneurysms with platinum coils in a patient with polyarteritis nodosa. J Rheumatol 1993; 20:158–161. 24. Guillevin L, Ruel M, Merrouche Y, Gayraud M, Royer I. Regressing aneurysms in polyarteritis nodosa related to hepatitis B virus. Eur J Intern Med 1990; 1:267–272.

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