- 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)
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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Domino, Frank J., et al., editors. "Wegener Granulomatosis." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688843/all/Wegener_Granulomatosis.
Wegener Granulomatosis. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688843/all/Wegener_Granulomatosis. Accessed May 29, 2023.
Wegener Granulomatosis. (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688843/all/Wegener_Granulomatosis
Wegener Granulomatosis [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 May 29]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688843/all/Wegener_Granulomatosis.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Wegener Granulomatosis ID - 1688843 ED - Domino,Frank J, ED - Baldor,Robert A, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688843/all/Wegener_Granulomatosis PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -