The ANCA –associated vasculitides (AAV’s), Granulomatosis with polyangiitis (Wegener’s) (GPA), Churg -Strauss syndrome (CSS) and microscopic polyangiitis (MPA) are a group of rare, potentially life-threatening conditions which if untreated can be fatal. They are characterized by systemic illness, multi-system disease, with inflammation of blood vessel walls (vasculitis), which can lead to aneurysm formation, haemorrhage and infarction (Watts & Scott 2003). Many organs can be affected such as the kidney, heart, lung, upper and lower airways and the nervous system. They share a number of clinical features and are therefore treated using similar treatment protocols.
Management of AAV The aim of treatment is to induce remission, preserve organ function, and reduce mortality and toxicity of medication. Treatment should commence as early as possible to avoid irreversible organ damage. Treatment is mainly split into three phases 1) induction of remission 2) maintenance and 3) long term follow up. Guidelines have been published for the management of AAV (Lapraik et al 2007, Mukhtyar et al 2009) and there is general consensus that cyclophosphamide and steroids are the first choice for induction of remission. Once remission is achieved azathioprine continues to have the safest profile (Ntatsaki et al 2011).These patients require regular and careful follow up to assess organ function and damage, early detection of disease relapse and detection of drug toxicity and side effects of medication. The role of the nurse will be discussed in the management of these patients.
Lapraik C, Watts R, Bacon P, et al. (2007) B SR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology; 4: 1615 -16
Mukhtyar C, Guillevin L, Cid MC, et al. (2009) EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis; 68: 310 -17
Ntatsaki E, Mooney J, Watts R.A (2011) ANCA vasculitis—time for a change in treatment, paradigm? Not yetRheumatology, 50(6):1019-1024
Watts RA. Scott DGI (2003)Overview of the inflammatory vasculitides. Rheumatology 3Ed. Eds Hochberg MC, Silman A, Smolen JS, Weinblatt ME, Weissman MH; 1583-91
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