• Vasculitis Guidelines

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises a group of rare, potentially life-threatening conditions that can be fatal if untreated. These diseases include granulomatosis with polyangiitis (GPA), (previously known as Wegener granulomatosis), Churg-Strauss syndrome (CSS), and microscopic polyangiitis (MPA). These complex conditions are characterized by systemic illness and multisystem disease, with vasculitis that can lead to aneurysm formation, hemorrhage, and infarction [Watts RA, Scott DG. Curr Opin Rheumatol 2003]. The kidneys, heart, lungs, upper and lower airways, and the nervous system can be affected by AAV.

In this presentation, Janice Mooney, MSc, RGN, University of East Anglia, Norwich, United Kingdom, discussed the management of AAV. AAV conditions are rare, with an estimated annual incidence of 20 per 1 million in Europe [Ntatsaki E. Rheum Dis Clin North Am 2010]. Historically, AAV had a poor prognosis, but available treatments have transformed it into a chronic relapsing condition.

EULAR published recommendations for the management of primary AAV in 2009 [Mukhtyar C. Ann Rheum Dis 2009]. Management of AAV requires a multidisciplinary approach. The aims of treatment are to induce remission, preserve organ function, reduce mortality, and reduce treatment-related toxicity. Treatment should begin as early as possible to avoid irreversible organ damage. Management is divided into 3 phases: induction of remission, maintenance, and long-term follow-up. The Birmingham Vasculitis Activity Score is used to assess organ involvement and the severity of disease, which guides the choice of an immunosuppressive regimen.

The EULAR guidelines have categorized AAV severity of disease into 5 categories: localized, early systemic, generalized, severe, and refractory (Table 1).

Cyclophosphamide and steroids are considered the first choice of treatment for induction of remission. Once remission is achieved, azathioprine is the safest maintenance therapy [Ntatsaki E. Rheumatology 2011]. Routine, careful follow-up is required for assessment of organ function and damage, early detection of relapse, and diagnosis of treatment toxicity.

The EULAR recommended treatments for AAV, according to disease severity, are shown in Table 2.

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