Wegener's granulomatosis is characterised by necrotising granulomatous inflammation and pauci-immune (i.e. a lack of deposits of immune complex) small vessel vasculitis. For those who eschew eponyms, granulomatosis with polyangiitis is the alternative term. An association with Staphylococcus aureus has been described and several possible mechanisms have been described.
- Upper airways involvement
- Nasal discharge
- Saddle nose deformity
- Lower airways involvement with inflammation/granuloma formation
- Lung e.g. abnormal CXR
- Renal (70%)
- Urinalysis/Microscopy - as screen for renal disease with common presentations
- c-ANCA - good specificity but poor sensitivity. A small number of cases are associated with positive p-ANCA.
Almost always involves a large number of infective and systemic diseases as often presents with non-specific upper or lower airways problems or unexpected renal impairment. c-ANCA does not distinguish between say microscopic polyangiitis or Churg-Strauss syndrome
- Steroids usually at high dose
- Cyclophosphamide for vasculitic complications
- Methotrexate for granulomatous complications
- ↑ Wegener F. On generalised septic vessel diseases. By Friedrich Wegener, 1937 (translation) Thorax. 1987 Dec; 42(12):918-9.
- ↑ GODMAN GC, CHURG J. Wegener's granulomatosis: pathology and review of the literature. A.M.A. archives of pathology. 1954 Dec; 58(6):533-53.
- ↑ Woywodt A, Haubitz M, Haller H, Matteson EL. Wegener's granulomatosis. Lancet. 2006 Apr 22; 367(9519):1362-6.(Link to article – subscription may be required.; Link hosted by author)
- ↑ Popa ER, Stegeman CA, Kallenberg CG, Tervaert JW. Staphylococcus aureus and Wegener's granulomatosis. Arthritis research. 2002; 4(2):77-9.