Nasal cavity, paranasal sinuses, nasopharynx
Inflammatory / infectious lesions
Granulomatosis with polyangiitis (Wegener's)

Author: Nat Pernick, M.D. (see Authors page)

Revised: 24 September 2018, last major update November 2004

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Granulomatosis with polyangiitis[TI] nasal

Cite this page: Pernick, N. Granulomatosis with polyangiitis (Wegener's). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/nasalwegeners.html. Accessed November 21st, 2018.
Definition / general
  • Rapidly progressive condition involving nasal cavity, lungs and kidney
  • Biopsies of 5 mm or more improve diagnostic yield
  • Note: lymphocyte rich biopsies more likely represent lymphoma
  • Diagnostic: granulomatous inflammation, necrosis and vasculitis, in addition to clinical involvement of lung or kidney; OR two of three microscopic features and lung and kidney involvement
  • Probable: two of three microscopic features and (lung or kidney involvement) but not both
  • Suggestive: one of three microscopic features and lung and kidney involvement
  • Suspicious: one of three microscopic features and (lung or kidney involvement) but not both
  • Nonspecific: no microscopic features, either lung or kidney involvement
Terminology
Gross description
  • Early disease usually lacks destruction of cartilage or bone
Microscopic (histologic) description
  • Leukocytoclastic vasculitis of arterioles, small arteries and veins with geographic necrosis surrounded by palisading histiocytes, variable poorly formed granulomas with minimal lymphocytes
  • Variable giant cells
  • Granulomas and giant cells are distant from vessels or adjacent / within vessel wall
  • Epithelial ulcers
  • Vessel may be obliterated by inflammatory cells and thrombus and be difficult to identify without elastic stain
  • All stages of vasculitis are present (acute: neutrophils with fibrinoid necrosis; chronic / healed: narrowed or obliterated lumina with concentric rims of perivascular collagen)
  • Usually no significant lymphoid infiltrate
Positive stains
Negative stains