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ILLUSTRATED HISTOPATHOLOGIC CLASSIFICATION CRITERIA FOR SELECTED VASCULITIS SYNDROMES J. T. LIE and MEMBERS AND CONSULTANTS OF THE AMERICAN COLLEGE OF RHEUMATOLOGY SUBCOMMITTEE ON CLASSIFICATION OF VASCULITIS We describe the histopathologic criteria for the diagnosis of 7 selected vasculitis syndromes: polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, hypersensitivity vasculitis, HenochSchonlein purpura, giant cell (temporal) arteritis, and Takayasu arteritis. The criteria apply to the stereotypical cases in each category; they were formulated after a review of submitted biopsy material from 278 of the 1,000 patients entered into the American College of Rheumatology Vasculitis Study Registry, and from prior experience with the pathologic diagnosis of vasculitis in 1,079 nonregistry patients. From the American College of Rheumatology Subcommittee on Classification of Vasculitis (Diagnostic and Therapeutic Criteria Committee of the Council on Research). J. T. Lie, MD: Mayo Clinic, Rochester, MN; Gene G. Hunder, M D Mayo Clinic, Rochester, MN, and Chair, Subcornmittee on Classification of Vasculitis; William P. Arend, MD: University of Colorado Health Science Center, Denver, CO; Daniel A. Bloch, PhD: Stanford University, Stanford, CA: Leonard H. Calabrese, D O Cleveland Clinic Foundation, Cleveland, OH; Steven M. Edworthy, MD: University of Calgary, Calgary, Alberta, Canada; Anthony S. Fauci, MD: NIAID, NIH, Bethesda, MD; Randi Y. Leavitt, MD, PhD NIAID, NIH, Bethesda, MD; Robert W. Lightfoot. Jr., MD: University of Kentucky, Lexington, KY; Alfonse T. Masi. MD, DrPH: University of Illinois College of Medicine, Peoria, IL; Dennis J. McShane, MD: Stanford University, Stanford, CA; Beat A. Michel, MD: Rheumaklinik Universitatsspital, Zurich, Switzerland; John A. Mills, M D Massachusetts General Hospital, Boston, MA; Mary Betty Stevens, MD: Johns Hopkins University, Baltimore, MD; Stanley L. Wallace, MD: SUNY Downstate Medical Center, Brooklyn, NY (Dr. Wallace is deceased); Nathan J. Zvaifler, M D University of California, San Diego, San Diego, CA. Address reprint requests to the American College of Rheumatology, 17 Executive Park Drive NE, Suite 480, Atlanta. GA 30329. Submitted for publication October 2, 1989; accepted April 3,1990. Arthritis and Rheumatism, Vol. 33, No. 8 (August 1990)

Few disease entities in medicine cause as much diagnostic consternation as the vasculitides because of the heterogenicity in their etiology and pathogenesis, their varied modes of manifestation, and their frequently overlapping clinical and pathologic features. A definitive diagnosis of vasculitis almost invariably requires histologic documentation of the disease, because few vasculitic syndromes have pathognomonic clinical findings and laboratory test results. The interpretation of histologic changes in a biopsy sample is subject to such variables as the pathologist's experience, tissue selection, sample size, chronologic age of the disease, and any prior treatment at the time of Table 1. Major vasculitis syndromes among I ,OOO Vasculitis Study Registry cases and 278 cases with biopsy review, according to the American College of Rheumatology Subcommittee on Classification of Vasculitis classification system

Number of cases Percent of cases Diagnostic category Polyarteritis nodosa Churg-Strauss syndrome Wegener's granulomatosis Hypersensitivity vasculitis Henoch-Schonlein purpura Giant cell (temporal) arteritis Takayasu arteritis Other vasculitis, type unspecified, and vasculitis associated with a connective tissue disease Kawasaki disease Nondiagnostic biopsies Total

Registry With Registry With entry biopsy entry biopsy 52 6 26 43 12 47 3 69

118

20 85

93 85 214 63

270

52 0

l,OOO

2

11.8 2.0 8.5

9.3 8.5

18.7 2.2

9.3 15.5 4.3

21.4

16.9

6.3 27.0

1.1 24.8

0

5.2

0

0

0

7.2

278

100.0

100.0

VASCULITIS: HISTOPATHOLOGIC CRITERIA Table 2. Distribution of 1,079 nonregistry cases of vasculitis with histologic documentation of diagnosis

Diagnostic category Polyarteritis nodosa Churg-Strauss syndrome Wegener’s granulomatosis Hypersensitivity vasculitis Henoch-Schonlein purpura Giant cell (temporal) arteritis Takayasu arteritis Other vasculitis, type unspecified, and vasculitis associated with a connective tissue disease Kawasaki disease Angiitis of the central nervous system Buerger’s disease Total

Number of cases 76 55 69 13 31

532

34 78 21

I5 89 1,079

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biopsy. A biopsy is considered adequate only when a verifiable diagnosis can be derived from it, but in practice, this ideal goal is not always attainable (1-4).

Database of histopathologic criteria for the diagnosis of vasculitis The American College of Rheumatology (ACR) Subcommittee on Classification of Vasculitis was given the charge of developing standard classification criteria for common forms of vasculitis. The purpose was to improve communication among physicians who diagnose and treat patients with these vasculitic syndromes and to permit direct comparison of studies on vasculitis performed by investigators in different research centers.

Figure 1. Characteristic histopathology of classic polyarteritis nodosa. A, Different and adjacent segments of the same interlobar artery of the kidney may be normal (short arrow) or drastically altered by a necrotizing arteritis with fibrinoid necrosis (long arrow)(hematoxylin and eosin stained, magnification x 64).B, In the same cross-section, part of the arterial wall may be normal (above) and part may be severely damaged by a necrotizing arteritis with microaneurysm formation (curved arrows) (hematoxylin and eosin stained, magnification x 160).

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Figure 2. Variegated histopathology of polyarteritis nodosa in small blood vessels. In serial sections of the same testicular biopsy, one arteriole (long arrows) may be severely (A) or minimally (B)affected by a necrotizing angiitis. while another (short arrows) may appear essentially normal (A) or show fibrinoid necrosis (B).C, A positive sural nerve biopsy in systemic necrotizing vasculitis. D,Higher magnification of boxed area in C. (Hematoxylin and eosin stained, magnification x 160 in A and B, X 64 in C,and x 400 in D.)

Decisions were made to formulate the classification criteria by an analytic, rather than empiric, approach and to limit the study to 7 selected forms of vasculitis that have been generally accepted as distinct clinical syndromes. These were polyarteritis nodosa, Churg-Strauss syndrome, Wegener’s granulomatosis, hypersensitivity vasculitis, Henoch-Schonlein purpura, giant cell (temporal) arteritis, and Takayasu arteritis. A miscellaneous category was created to accommodate “type unspecified vasculitis and vasculitis and a connective tissue disease,” but this category was not included in the final formulation of categoryspecific classification. Table 1 lists the distribution of the 1,000 cases that were entered into the ACR Vasculitis Study Registry, including 52 patients with Kawasaki disease

who were not further analyzed. Biopsy materials from 278 of the study patients were received for review. These had a slightly different distribution among the vasculitis categories but showed a similar prevalence trend when compared with the larger clinical group (Table 1). The formulation of descriptive histopathologic criteria for diagnosis of the 7 selected vasculitis syndromes also freely utilized data from prior experience with the pathologic diagnosis of vasculitis in 1,079 nonregistry patients (1-9) (Table 2).

Polyarteritis nodosa Polyarteritis nodosa (PAN) predominantly affects the medium-sized and small arteries and, less commonly, the arterioles and venules. The predilec-

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Figure 3. Vascular and extravascular lesions of spleen in Churg-Strauss syndrome. A, Typical extravascular eosinophilic granuloma with a central zone of necrosis, surrounded by a mixed cell infiltrate with prominent epithelioid cells. B and C, necrotizing vasculitis with fibrinoid

necrosis and eosinophil-rich inflammatory infiltrate. (Hematoxylin and eosin stained, magnification x 160.)

tion of lesions in vessel bifurcation, or branching, points is often stressed in the literature but has little diagnostic value in practice, when only isolated, and often single, tissue samples are examined. Involvement of the aorta and its primary branches and the elastic pulmonary arteries is virtually unknown in PAN. Segmental necrotizing glomerulonephritis, or a renal-limited variant of PAN, is considered to belong to the subset of microscopic polyarteritis (7). A number of different tissues have been biopsied for the diagnosis of PAN. Biopsies of the skeletal muscle, sural nerve, kidney, testis, liver, or rectum are preferable to biopsy of the skin, which, even when positive, is less diagnostic and is not necessarily indicative of systemic involvement. PAN has a wide spectrum of morphologic expression. Although the individual vascular lesions may not be specific for PAN, there are certain sets or combina-

tions of histologic features that, when present, are uniquely diagnostic for PAN. The vascular lesions of PAN are characteristically focal and segmental or sectoral (Figures 1 and 2); that is, a normal artery may lie side by side with one that is severely affected, only a segment of the artery may be affected along its length, or only a part of the circumference of the arterial wall may be affected. Necrotizing vasculitis with fibrinoid necrosis represents the acute or active lesion, and the sectoral involvement may result in the formation of a “blowout” type of microaneurysm (Figure 1). Thrombosis commonly accompanies necrotizing vasculitis (Figure 2). “Fibrinoid necrosis” alone is not specific for PAN; it occurs with regularity, for instance, in small-vessel lesions of malignant hypertension. The vascular inflammatory infiltrate in PAN is typically transmural and pleomorphic, with an admix-

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Figure 4. A, The lung in Wegener’s granulomatosis, showing large-vessel necrotizing angiitis and thrombosis, surrounded by a geographic pattern of granulomatous necrosis (GN) of the parenchyma (hematoxylin and eosin stained, magnification x 16). B, Typical pulmonary necrotizing granulomas seen at higher magnification (hematoxylin and eosin stained, magnification x 160).

ture of lymphomononuclear cells and variable numbers of neutrophils and eosinophils. Rarely, the vasculitis in PAN may be granulomatous. The individual vascular lesions are highly variable and may appear quite different, even in serial sections less than 20 pm apart (Figure 2). Another unique histologic feature of PAN is the coexistence of active necrotizing lesions and proliferative fibrotic healing or healed lesions in differenttissues or in different parts of the same tissue.

Churg-Strauss syndrome Originally described as an allergic granulomatosis and angiitis, Churg-Strauss syndrome is a relatively uncommon systemic disease, with clinical and pathologic features that overlap those of polyarteritis nodosa and Wegener’s granulomatosis. The syndrome is characterized by pulmonary and systemic vasculitis,

extravascular granulomas, and eosinophilia, occurring almost exclusively in patients with asthma or a history of allergy (2,8). Churg-Strauss syndrome may occur in limited form in isolated organs or tissues, and it has also been reported to occur in patients with a connective tissue disease or autoimmune disorder (7). Regardless of the organs involved, the 2 diagnostically essential lesions of Churg-Strauss syndrome are angiitis and extravascular necrotizing granulomas, usually with eosinophilic infiltrates (Figure 3). The angiitis may be granulomatous or nongranulomatous, and it characteristically involves both the arteries and the veins, as well as pulmonary and systemic blood vessels. Extrapulmonary lesions are found more commonly in the gastrointestinal tract, spleen, and heart than in the kidney, in distinction to those of Wegener’s granulomatosis. Cutaneous and subcutaneous lesions, so-called Churg-Strauss granulomas, lack diagnostic

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Figure 5. Small-vessel lesions of Wegener’s granulomatosis. A, Pulmonary nongranulomatous necrotizing vasculitis. B, Capillaritis in diffuse alveolar hemorrhage as a fatal pulmonary manifestation of Wegener’s granulomatosis. (Hematoxylin and eosin stained, magnification X 400.)

specificity because approximately 50% of such lesions occur in a variety of systemic diseases other than Churg-Strauss syndrome (2).

Wegener’s granulomatosis Wegener’s granulomatosis is basically a necrotizing granulomatous disease rather than a primary vasculitic disorder. The classic triad of Wegener’s granulomatosis consists of 1) necrotizing granulomas of the upper or lower respiratory tract, or both; 2) necrotizing or granulomatous vasculitis, usually of small arteries and veins, almost always in the lung, and more selectively in systemic organs; and 3) focal, segmental, necrotizing glomerulitis. The absence of detectable renal involvement in some patients has been described as a “limited form” of Wegener’s granulomatosis. It became known subsequently that all 3 classic criteria need not be present concurrently

in all patients with Wegener’s granulomatosis. The concept of the limited forms of Wegener’s granulomatosis should probably also be discarded, since these cases are probably early, occult, and protracted stages of the disease that may later expand or transform into the classic disseminated form (7,8). The typical pulmonary lesions of Wegener’s granulomatosis are necrotizing granulomas combined with necrotizing or granulomatous vasculitis. The vasculitis usually involves small arteries and veins but may also affect large blood vessels; the granulomas may be discrete or confluent, forming an irregular geographic pattern of lung parenchymal necrosis (Figure 4). Eosinophils may be present in the inflammatory infiltrate but are seldom in excessive number. The vasculitis is more often necrotizing than granulomatous, and diffuse lung hemorrhage from capillaritis (Figure 5 ) has been reported to occur in 5 4 5 % of biopsy- or autopsy-documented cases (10,ll). Focal,

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Figure 6. Renal vascular lesions (arrows) of Wegener’s granulomatosis. A, Nongranulomatousangiitis with fibrinoid necrosis, more typically seen in polyarteritis. B, Granulomatous vasculitis. (Hematoxylin and eosin stained, magnification x 160.)

segmental, necrotizing glomerulitis is the predominant type of renal lesion in Wegener’s granulomatosis. Renal vasculitis occurs in less than 50% of patients, and, when present, it may be the polyarteritis-type of necrotizing vasculitis or a granulomatous vasculitis (Figure 6).

Hypersensitivity vasculitis Vasculitides involving small blood vessels have many synonyms, and they are associated with a large and heterogeneous group of clinical syndromes. Originally, the term “hypersensitivity angiitis” was used to refer to patients with disseminated necrotizing vasculitis of arterioles and venules, both systemic and pulmonary, and considered distinguishable from polyarteritis nodosa. Clinically, the vascular lesions are seen most commonly in the skin, where they appear as purpura or urticaria. The term “hypersensitivity vas-

culitis” has also been used by some to refer to conditions in which skin involvement dominates and visceral involvement is absent, not detected, or occurs only rarely. Others prefer such synonyms as “allergic vasculitis,” “cutaneous-systemic angiitis,” “leukocytoclastic vasculitis,” or simply “small-vessel vasculitis” (4,8). Much of our understanding of small-vessel vasculitis is based on cutaneous vasculitis. Because they are easily accessible for biopsy, the cutaneous lesions of hypersensitivity vasculitis have been studied more extensively than lesions at other sites. The vascular damage is believed to be triggered by the deposition of immune complexes in vessel walls, with activation of the complement cascade. Polymorphonuclear leukocytes then migrate to the area and release lysosomal enzymes that damage blood vessels, leading to diapedesis of erythrocytes, fibrin deposition, and necrosis. Because leukocytoclastic (karyorrhexis of leukocytes)

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Figure 7. Typical cutaneous lesions of hypersensitivity vasculitis. A, Leukocytoclastic vasculitis with karyorrhexis of polymorphonuclear leukocytes and extravasation of red blood cells. B, Lymphocytic vasculitis. In both types, arterioles and venules are affected. (Hematoxylin and eosin stained, magnification x 160.)

Figure 8. Histopathologic variations of hypersensitivity vasculitis. A, Eosinophilic vasculitis. B, Granulomatous angiitis. (Hematoxylin and eosin stained, magnification x 160.)

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Figure 9. Characteristic histopathology of Henoch-Schonlein purpura, as focal segmental glomerulonephritis in the kidney (A) and as necrotizing vasculitis in the gastrointestinal tract (B). (Hematoxylin and eosin stained, magnification x 160.)

inflammation is such a prominent feature, these lesions are referred to as “leukocytoclastic vasculitis.” Hypersensitivity vasculitis can be divided into neutrophilic and lymphocytic subtypes (Figure 7). The former is frequently associated with hypocomplementemia, whereas the latter is not. However, they may also simply be different evolutional stages of the same disease process, and hypocomplementemia alone cannot serve as a constant distinguishing factor. Other histologic variants of hypersensitivity vasculitis are small-vessel eosinophilic vasculitis and granulomatous vasculitis (Figure 8).

Henoch-Schonlein purpura Henoch-Schonlein purpura (HSP), or anaphylactoid purpura, is a distinct type of hypersensitivity vasculitis of unknown cause. It occurs primarily in children and young adults. The clinical triad of pur-

pura, arthritis, and abdominal pain is seen in as many as 80% of patients with HSP. Renal involvement manifests as a proliferative and necrotizing glomerulonephritis (Figure 9A), which is generally mild and self-limiting, but, at times, it may be severe and persistent. Histologic evidence of a systemic necrotizing small-vessel vasculitis (Figure 9B) with polyarthritis and nephritis supports the contention that HSP may be an immune complex disease. By immunofluorescence microscopy, IgA is the most abundant, and sometimes the only, type of immunoglobulin detectable in the skin and renal glomeruli. The diagnostic specificity of IgA deposits in HSP has been confirmed by some investigators and denied by others (7).

Giant cell (temporal) arteritis Although a positive biopsy is still desirable for the proper documentation of giant cell (temporal)

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Figure 10. Variations of the histopathology of active temporal (giant cell) arteritis. Top, Two positive biopsies with near-occlusion of the arterial lumens. Bottom, Higher magnification of boxed areas of top views, showing the usual location of giant cells in granulomatous inflammation at the intima-media junction (A) and the less common location of intense granulomatous inflammation in the media and extending into the adventitia (B).(Hematoxylin and eosin stained, magnification x 40 top and x 160 bottom.)

arteritis, the lack of histologic proof of arteritis should not prevent or delay treatment of patients in whom temporal arteritis is strongly suspected on clinical grounds. The biopsy is important not only for confirmation of the diagnosis but also for assessment of disease activity and adjustment of corticosteroid therapy. Not infrequently, the diagnosis of giant cell arteritis is made on the basis of the biopsy results in patients who lack classic symptoms and physical signs of temporal arteritis, as well as in patients in whom extracranial giant cell arteritis of a relatively inaccessible (for biopsy) artery is suspected. Systemic arteritis other than giant cell arteritis may be, and has been, diagnosed serendipitously in a temporal artery biopsy sample (5). Temporal arteritis may be a bilateral disease, but involvement of a given artery, even when

clinically symptomatic, is characteristically focal and segmental. An adequate biopsy requires, at minimum, a 2-3 cm-long segment of the artery; if the selected artery is negative for arteritis, biopsy of the temporal artery on the opposite side is advisable, especially in patients strongly suspected of having giant cell arteritis. The classic picture of granulomatous giant cell arteritis (Figure 10) is seen in approximately 50% of positive temporal artery biopsy samples from symptomatic patients. The other 50% of positive biopsy samples show a panarteritis, with a mixed-cell inflammatory infiltrate that is predominantly lymphomononuclear and contains some neutrophils and eosinophils but no giant cells. Occasionally, a circumferential band of fibrinoid necrosis may also be present in the vessel wall of a positive specimen. The number of

LIE ET AL

Figure 11. The aorta in active Takayasu arteritis. Low-power views of granulomatous arteritis in A, a hematoxylin and eosin-stained section and B, an elastic-tissue-stained section of the aorta (magnification x 40). C, High-power view of the boxed area in A, showing a cluster of giant cells in the predominantly lymphoplasmacytic infiltrate (magnification x 160).

giant cells and the intensity and location (within the vessel wall) of inflammatory cell infiltrates are highly variable. There is no evidence that the length of history, the erythrocyte sedimentation rate, or any other clinical or laboratory test findings in a patient can be correlated with a particular histologic expression of temporal arteritis (5). Treatment with corticosteroids prior to biopsy does not completely efface or modify the diagnostic histopathology of arteritis in a temporal artery biopsy specimen. Patients biopsied before starting corticosteroid therapy have the highest incidence of a diagnostic result, about 80% ( 5 ) . The diagnostic yield would fall to approximately 60% if corticosteroids were taken for less than 1 week before the biopsy, and to approximately 20% for pre-biopsy treatment of longer periods (5). Fragmentation of the internal elastic lamella of the arterial wall occurs commonly in temporal arteritis, but this feature alone is not the sine qua non of

temporal arteritis. It occurs with regularity in all temporal arteries of the aged (>60 years), and it persists long after the active phase of the disease.

Takayasu arteritis Takayasu arteritis is a chronic, nonarteriosclerotic, inflammatory disease of the aorta and its main branches (including the proximal coronary and renal arteries) and the elastic pulmonary arteries. It has a worldwide distribution, with greatest prevalence in the Orient, and it typically affects young women between the ages of I5 and 45. Pulseless disease and aortic arch syndrome are the classic manifestations of Takayasu arteritis, but claudication and renovascular hypertension may be the more disabling complications of the disease. Although obliterative lesions are still most common, aneurysmal disease of the affected arteries,

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Figure 12. Exacerbation of Takayasu artentis of the aorta, 5 years after the initial diagnosis and treatment with corticosteroids. A, Low-power view of the aortic wall, showing marked intimal fibrosis and granulomatous inflammation in the media and adventitial fibrosis (hematoxylin and eosin stained, magnification x 40). B, High-power view of the boxed area in A, showing a group of giant cells amid the lyrnphoplasmacytic infiltrate (hematoxylin and eosin stained, magnification x 160).

especially the aorta, occurs in as many as 20% of patients (5). Takayasu arteritis has a distinct pattern of histopathologic changes at different stages of its chronologic development. In the early or active stage, a biopsy is diagnostic; it shows a granulomatous arteritis, with transmural inflammation and patchy destruction of the medial musculoelastic lamellae (Figure 11). The cell infiltrate is predominantly lymphoplasmacytic and confined to the media, with variable numbers of giant cells (both Langhans and foreign-body type). With exacerbation, the same active-disease picture will return, even after a long interval of previous treatment with corticosteroids (Figure 12). Healing and healed arteritis is indicated by progressive intimal and adventitial fibrosis, with scanty lymphocytic infil-

trate and extensive scarring of the media. Takayasu arteritis cannot be confidently diagnosed by biopsy alone at this chronic phase or end-stage of the disease.

Conclusion Each of the 7 major vasculitis syndromes selected for discussion has its own unique histopathologic features, but overlap is common and often confuses the unwary or casual observer (Table 3). A biopsy diagnosis of vasculitis does not stand alone in the proper medical management of a patient, and a diagnosis usually cannot be made conclusively without correlation with the patient’s clinical history, physical findings, and angiographic findings (when applicable), preferably, all three. The success of a biopsy diagnosisof vasculitis also

Demographic and environmental predisposition

Most patients have asthma or history of allergy

Extravascular necrotizing granulomas with prominent eosinophils; may manifest as “limited form”

Special features

Focal segmental involvement of vessels; coexisting acute and healed vascular lesions, or normal and affected vessels; microaneutysms Vascular lesion of infantile polyarteritis is indistinguishable from fatal cases of Kawasaki disease

Necrotising or granulomatous, with mixed cells and prominent eosinophils

Small arteries and Medium and small veins, often muscular arteries, arterioles and and sometimes venules arterioles Visceral, cutaneous, Upper and lower respiratory tract, and, infrequently, cerebral vessels viscera, heart, and lung and skin

Churg-Strauss syndrome

Necrotizing, with Type of vasculitis and inhnmatoly mixed cells and cell infiltrate few eosinophils, rarely granulomatous

Distribution and localization

Type of vessels involved

Polyarteritis nodosa ~

~~

Hypersensitivity vasculitis

Henoch-Schbnlein purpura ~

~~~

Giant cell (temporal) arteritis

with a slight male preponderance; associated with HLA-DR2; m y respond to antimicrobial agents

Occurs in all ages,

Usually small arteries and veins, sometimes larger vessels Upper and lower respiratory tract, often kidney, and infrequently skin, heart, viscera, and brain Necrotizing or granulomatous, with mixed cells and occasional eosinophils

Patients may have history of drug or chemical allergy, vaccination, or occult malignancy

Predominantly children and young adults

Virtually all patients with temporal arteritis are over age 50; may be clinically asymptomatic

Arterioles and Arterioles and Vessels of all sizes venules, often venules. often small arteries small arteries and and veins veins Predominantly skin Predominantly skin, Predominantly temporal arteries, gastrointestinal, and less kidney, and commonly and less often any other large, viscera, heart, synovium medium, and small and synovium vessels Granulomatous, with Leukocytoclastic Leukocytoclastic, variable number of mixed-cell, or or lymphocytic, with variable giant cells, lymphocytic, number of sometimes only with variable number of eosinophils, lymphoplasmacytic eosinophils occasionally granulomatous Affected extracranial Geographic pattern of May be associated IgA immune with myocarditis, tissue necrosis and deposits in large vessels positive affected tissue indistinguishable interstitial antineutrophil from Takayasu nephritis, or arteritis; may form cytoplasmic hepatitis antibodies; may aneurysm or cause dissection manifest as “limited form”

Wegener’s granulomatosis

Table 3. Comnarison of the oatholoeic characteristics of selected vasculitis svndromes

Most commonly in women of childbearing age; more prevalent in the Orient; an important cause of renovascular hypertension in adolescents

Aorta, arch vessels, and other major branches (coronary, renal, visceral), and pulmonary arteries Granulomatous, with few giant cells in active phase, and sclerosing fibrosis in chronic stage, with scanty infiltrate Aneurysmal in 20%; may be segmental, and cause rupture or dissection

Elastic arteries and selected muscular arteries

Takayasu arteritis

VASCULITIS: HISTOPATHOLOGIC CRITERIA depends on the pathologist’s experience, selection and preparation of tissues, sample size, chronologic age of the disease, and prior treatment of the disease.

REFERENCES 1. Lie JT:Nosology of pulmonary vasculitides. Mayo Clin Roc 52520-522, 1977 2. Lie JT: The classificationof vasculitis and a reappraisal of allergic granulomatosis and angiitis (Churg-Strauss syndrome). Mt Sinai J Med (NY)53:429439, 1986 3. Lie JT: Coronary vasculitis: a review in the current scheme of classification of vasculitis. Arch Pathol Lab Med 11 1:224-233, 1987 4. Lie JT:Classification and immunodiagnosis of vasculitis: a new solution or promises unfulfilled. J Rheumatol 15:72&732, 1988 5 . Lie JT: The classification and diagnosis of vasculitis in

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large and medium-sized blood vessels. Pathol Annu 22: 125-162, 1987 6. Lie JT: Thromboangiitis obliterans (Buerger’s disease) revisited. Pathol Annu 23:257-291, 1988 7. Lie JT: Systemic and isolated vasculitis: a rational approach to classification and pathologic diagnosis. Pathol Annu 24:25-114, 1989 8. Lie JT: Classification of pulmonary angiitis and granulomatosis: histopathologic perspectives. Semin Respir Med 10:I 1 1-121, 1989 9. Lie JT: The rise and fall and resurgence of thromboangiitis obliterans (Buerger’s disease). Acta Pathol Jpn 39: 153-158, 1989 10. Colby TV:Diffuse pulmonary hemorrhage in Wegener’s granulomatosis. Semin Respir Med 10:136-140, 1989 11. Travis WD, Carpenter HA, Lie JT: Capillaritis and pulmonary hemorrhage in Wegener’s granulomatosis. Am J Surg Pathol 13:78-79, 1989

Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis.

We describe the histopathologic criteria for the diagnosis of 7 selected vasculitis syndromes: polyarteritis nodosa, Churg-Strauss syndrome, Wegener's...
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