Introduction: Nomenclature and Classification

  • J. Charles JennetteEmail author
  • Ronald J. Falk
Part of the Rare Diseases of the Immune System book series (RDIS)


Nomenclature, classification, and diagnosis of vasculitis are difficult because of the broad spectrum of types and locations of vessels affected, multiple patterns of injury, diverse known etiologies and pathogenic mechanisms, absence of known etiologies and pathogenic mechanisms in some forms of vasculitis, and the myriad overlapping and nonspecific signs and symptoms caused by vasculitides. The goals of nomenclature, classification, and diagnostic systems are to enable effective communication among biomedical investigators and healthcare providers, guide clinical and basic research on well-defined cohorts (classes) of patients, and, most importantly, facilitate diagnosis and effective treatment of individual patients. This chapter reviews the historical basis for vasculitis nomenclature, classification, and diagnosis and the use of pathologic, clinical, and serologic data for classification and diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Physicians and scientists have made remarkable advances in the classification and diagnosis of vasculitides since the seminal observations in the 1800s; however, validated and widely applied classification criteria and diagnostic criteria that are sufficiently accurate and precise for clinical research and patient care, respectively, remain elusive. Even after well-validated criteria are established, they must be under constant scrutiny and adjusted as new knowledge emerges and new diagnostic modalities are introduced.


Antineutrophil cytoplasmic antibody (ANCA) Vasculitis Microscopic polyangiitis Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis 


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory Medicine, School of MedicineUniversity of North Carolina at Chapel HillChapel HillUSA
  2. 2.Department of Medicine, School of MedicineUniversity of North Carolina at Chapel HillChapel HillUSA

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