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Granuloma in ANCA-associated vasculitides: another reason to distinguish between syndromes?

Antje Mueller1, Konstanze Holl-Ulrich, Wolfgang L Gross

  • 1Department of Rheumatology, University of Luebeck, Ratzeburger Allee 160, 23538, Luebeck, Germany, antje.mueller@uksh.de.

Current Rheumatology Reports
|October 1, 2013
PubMed

Insights

Granuloma presence helps differentiate anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), particularly granulomatosis with polyangiitis (GPA), aiding in subclassification and improving patient treatment and clinical trial design.

Area of Science:

  • Rheumatology
  • Pathology
  • Otolaryngology

Background:

  • The Chapel Hill Consensus Conference (CHCC) named granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA) as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV).
  • Recent studies highlight the need for further differentiation among AAV variants, considering factors like outcome, ANCA reactivity, and airway involvement.
  • Extravascular granulomatosis is a key feature of GPA, distinguishing it from MPA.

Purpose of the Study:

  • To review new knowledge on granuloma in the head and neck region of AAV patients.
  • To explore histomorphological equivalents of granuloma in the respiratory tract.
  • To present evidence for a granulomatous phenotype in localized GPA variants and its potential for subclassification.

Main Methods:

  • Literature review focusing on granuloma in AAV, particularly GPA.
  • Analysis of histopathological findings in upper and lower respiratory tracts.
  • Examination of disease activity and damage scores for ENT lesions and relevant imaging techniques.

Main Results:

  • Granulomatous inflammation is characteristic of GPA, but not MPA.
  • Upper and lower airway disease, along with ANCA reactivity, are differentiating factors for AAV variants.
  • Extravascular manifestations in the head and neck region are linked to granulomatous inflammation.

Conclusions:

  • Necrotizing granulomatous inflammation and its clinical manifestations may serve as discriminators for AAV subclassification.
  • Identifying a granulomatous phenotype can aid in subtyping GPA and developing targeted clinical trials.
  • This approach can lead to more successful treatment strategies for AAV patients.

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