Criteria for the classification of Takayasu arteritis were developed by comparing 63 patients who had this disease with 744 control patients with other forms of vasculitis. Six criteria were selected for the traditional format elass$cation: onset at age 1 4 0 years, claudication of an extremity, decreased brachial artery pulse, >10 mm Hg difference in systolic blood pressure between arms, a bruit over the subclavian arteries or the aorta, and arteriographic evidence of narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities. The presence of 3 or more of these 6 criteria demonstrated a sensitivity of 90.5% and a specificity of 97.8%. A classification tree also was constructed with 5 of these 6 criteria, omitting claudication of an extremity. The classification tree demonstrated a sensitivity of 92.1% and a specificity of 97.0%.

Criteria for the classification of giant cell (temporal) arteritis were developed by comparing 214 patients who had this disease with 593 patients with other forms of vasculitis. For the traditional format classification, 5 criteria were selected: age 1 5 0 years at disease onset, new onset of localized headache, temporal artery tenderness or decreased temporal artery pulse, elevated erythrocyte sedimentation rate (Westergren) 2 5 0 mml hour, and biopsy sample including an artery, showing necrotizing arteritis, characterized by a predominance of mononuclear cell infiltrates or a granulomatous process with multinucleated giant cells. The presence of 3 or more of these 5 criteria was associated with a sensitivity of 93.5% and a specificity of 91.2%. A classijication tree was also constructed using 6 criteria. These criteria were the same as for the traditional format, except that elevated erythrocyte sedimentation rate was excluded, and 2 other variables were included: scalp tenderness and claudication of the jaw or tongue or on deglutition. The classification tree was associated with a sensitivity of 95.3% and specificity of 90.7%.

Criteria for the classification of Churg-Strauss syndrome (CSS) were developed by comparing 20 patients who had this diagnosis with 787 control patients with other forms of vasculitis. For the traditional format classification, 6 criteria were selected: asthma, eosinophilia >lo% on differential white blood cell count, mononeuropathy (including multiplex) or polyneuropathy, non-fixed pulmonary infiltrates on roentgenography, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils. The presence of 4 or more of these 6 criteria yielded a sensitivity of 85% and a specificity of 99.7%. A classijication tree was also constructed with 3 selected criteria: asthma, eosinophilia > 10% on differential white blood cell count, and history of documented

Criteria for the classification of Wegener's gram ulomatosis (WG) were developed by comparing 85 patients who had this disease with 722 control patients with other forms of vasculitis. For the traditional format cZass@cation, 4 criteria were selected: abnormal urinary sediment (red cell casts or >5 red blood cells per high power field), abnormal findings on chest radiograph (nodules, cavities, or fixed infiltrates), oral ulcers or nasal discharge, and granulomatous inflammation on biopsy. The presence of 2 or more of these 4 criteria was associated with a sensitivity of 88.2% and a specificity of 92.0%. A classiJication tree was also constructed with 5 criteria being selected. These criteria were the same as for the traditional format, but included hemoptysis. The

Criteria for the classification of polyarteritis nodosa were developed by comparing 118 patients who had this disease with 689 control patients who had other forms of vasculitis. For the traditional format class$cation, 10 criteria were selected: weight loss 2 4 kg, livedo reticularis, testicular pain or tenderness, myalgias, mononeuropathy or polyneuropathy, diastolic blood pressure >90 mm Hg, elevated blood urea nitrogen or serum creatinine levels, presence of hepatitis B reactants in serum, arteriographic abnormality, and presence of granulocyte or mixed leukocyte infiltrate in an arterial wall on biopsy. The presence of 3 or more of these 10 criteria was associated with a sensitivity of 82.2% and specificity of 86.6%. A classification tree was also constructed, with 6 criteria being selected. Three of these, angiographic abnormality, biopsy-proven granulocyte or mixed leukocyte infiltrate in arterial wall, and neuropathy, were criteria used in the traditional format. The other 3 criteria used in the tree format included the patient's sex, weight loss s6.5 kg, and elevated serum aspartate aminotransferase or alanine aminotransferase levels above the range of normal. The classification tree yielded a sensitivity of 87.3% and a specificity of 89.3%.Kussmaul and Maier described the syndrome of polyarteritis nodosa (PAN) in 1866 (1). In that and subsequent descriptions, PAN has been depicted as a necrotizing arteritis of small and medium-sized muscular arteries, affecting multiple organ systems throughout the body. The incidence and prevalence of PAN in the population are unknown, possibly because of difficulties in diagnosing and classifying the vasculitis syndromes, but it is most commonly reported in middle-aged adults, with a male predominance. A study of mortality in the city of New York from 1951 through 1959, disclosed that polyarteritis nodosa caused one-third the number of deaths as systemic lupus erythematosus during that same decade (2). Leavitt and Fauci described vasculitis overlap syndromes in several patients who fulfilled the thenextant, and largely anecdotal, diagnostic criteria for both PAN and another vasculitis syndrome (3).The etiology of PAN remains unknown, but the histopathologic resemblance to chronic serum sickness has suggested an immune complex pathogenesis. There is substantial evidence that at least one subset of PAN patients experiences systemic vasculitis as a result of chronic hepatitis B antigen-associated immune complex disease (4). Other reports suggesting an immune complex etiology have included the description of classic PAN following shortly after the occurrence of serous otitis media in adults (S), and the

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