Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment

J Oral Pathol Med. 2013 Aug;42(7):507-16. doi: 10.1111/jop.12030. Epub 2013 Jan 10.


Wegener's granulomatosis (WG) is an idiopathic, systemic inflammatory disease characterized by necrotizing granulomatous inflammation and pauci-immune small-vessel vasculitis of upper and lower respiratory tract and kidneys. The condition affects both genders equally, although some inconsistent gender differences have been observed. The aetiology of WG remains unknown although a number of exogenous factors have been suggested to be of aetiological relevance. Most clinical characteristics of this disease are non-specific, making clinical diagnosis challenging. Histopathological examination of lesional and peritoneal tissue is not pathognomonic, but is an essential investigation to confirm the presence of disease and exclude other disorders. At present, despite the increasingly wide range of potential therapies, cyclophosphamide plus corticosteroids remain the most recognized and effective means of inducing and sustaining remission of WG.

Keywords: Wegener's granulomatosis; granulomatous disease; oral disease.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / pathology*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Macrophages / pathology
  • Mouth Diseases / diagnosis
  • Necrosis
  • Vasculitis / pathology


  • Immunosuppressive Agents