title: [Classification of systemic vasculatides].
abstract: Vasculitides are defined by inflammation of blood vessel walls leading to vascular stenosis or occlusion, with various degrees of fibrinoid necrosis of the media and inflammatory infiltration, mainly neutrophilic and sometimes granulomatous. Various classifications of the vasculitides have been proposed. The classifications used most today are the 1990 American College of Rheumatology classification and the Chapel Hill nomenclature, published in 1994 . Only the latter distinguished between polyarteritis nodosa and microscopic polyangiitis and stressed the importance of antineutrophil cytoplasm autoantibodies (ANCA). In practice, primary systemic vasculitides are classified according to their clinical presentations, their precise histological features, and the size of the predominantly affected vessels. Some small-vessel vasculitides are associated with the presence of ANCA: 90% of patients with systemic Wegener's granulomatosis (mainly ANCA with cytoplasm labeling on indirect immunofluorescence and proteinase 3 specificity), 80% of the subjects with microscopic polyangiitis (mostly pANCA with myeloperoxidase specificity), and more than one third of those with Churg-Strauss syndrome (mostly pANCA).
authors.affiliation: Centre de référence national, Plan maladies rares, vascularites et sclérodermie systémique, Hôpital Cochin, AP-HP, Université Paris 5 - René Descartes, Paris, France. loic.guillevin@cch.aphp.fr
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Philippe
authors.initials: P
authors.lastname: Guilpain
mesh_terms: D000328:Adult; D000367:Age Factors; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D015415:Biomarkers; D002648:Child; D002675:Child, Preschool; D015267:Churg-Strauss Syndrome / Q000175:diagnosis / Q000276:immunology; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D019084:Fluorescent Antibody Technique, Indirect; D013700:Giant Cell Arteritis / Q000175:diagnosis; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis; D007223:Infant; D008875:Middle Aged; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis; D053146:Myeloblastin; D010488:Polyarteritis Nodosa / Q000175:diagnosis; D011379:Prognosis; D012680:Sensitivity and Specificity; D013625:Takayasu Arteritis / Q000175:diagnosis / Q000473:pathology; D009626:Terminology as Topic; D013919:Thromboangiitis Obliterans / Q000175:diagnosis; D014657:Vasculitis / Q000145:classification* / Q000175:diagnosis / Q000276:immunology / Q000473:pathology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D053146:Myeloblastin