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Wegener Granulomatosis

Wegener Granulomatosis is a topic covered in the 5-Minute Clinical Consult.

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  • A disease characterized by granulomatous vasculitis involving multiple organs
  • The characteristic triad of involvement includes the upper airway (e.g., otitis, sinusitis, nasal mucosa), lungs, and kidneys.
  • Other organ systems involved include the skin, joints, and nervous system (peripheral/central).
  • As the condition progresses untreated, upper airway erosions, necrotic pulmonary nodules, and renal failure are common.
  • Without treatment, mortality rate is high. With treatment, survival rate is ~75–90% at 5 years.
  • System(s) affected: upper airways (sinusitis, otitis); cardiovascular; gastrointestinal; nervous; pulmonary; renal/urologic; skin/exocrine
  • Synonym(s): antibodies to neutrophilic cytoplasmic antigens (ANCA)-associated vasculitis (to include patients with Wegener and microscopic polyangiitis); granulomatosis with polyangiitis (recommended to replace Wegener as diagnostic term)


  • ~0.4 to 1.2/100,000 persons per year and possibly increasing over the past decade
  • Mean age of onset is the mid-40s, but the disease has been described in all age groups.
  • Predominant sex: male > female (3:2)

3/100,000 persons

Etiology and Pathophysiology

  • Unknown etiology
  • Autoimmune phenomena and immune-complex deposition in arterial walls are implicated, and the activation of neutrophils by bacteria or other infectious agents may be important as well.
    • Role of antibodies directed against neutrophils is currently being investigated.
  • A specific triggering infectious agent has not been identified.

Increased presence in HLA-B8 and HLA-DR2, more recently increase in HLA-DP and genetic variants involved in α1-antitrypsin and protease-3 (1)

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Stephens, Mark B., et al., editors. "Wegener Granulomatosis." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816103/all/Wegener_Granulomatosis.
Wegener Granulomatosis. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816103/all/Wegener_Granulomatosis. Accessed March 22, 2019.
Wegener Granulomatosis. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816103/all/Wegener_Granulomatosis
Wegener Granulomatosis [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 March 22]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816103/all/Wegener_Granulomatosis.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Wegener Granulomatosis ID - 816103 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816103/all/Wegener_Granulomatosis PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -