BSR amp; BHPR Guidelines for the Management of adults with ANCA BSR amp；BHPR指引ANCA成人的管理.docx

BSR Guidelines for the Management of adults with ANCA Associated VasculitisChair of the Guideline Working Group: Dr Richard A WattsApril 2013Section 1 Scope and Purpose1.1 Background to VasculitisThe primary systemic vasculitides are heterogeneous, multi-system disorders characterised by inflammation and necrosis of small and medium blood vessels. Their aetiology is unknown. Three distinct clinico-pathological syndromes, often associated with anti-neutrophil cytoplasmic antibodies (ANCA), called ANCA associated vasculitis (AAV), have been identified and collectively comprise the most common subgroup: granulomatosis with polyangiitis (Wegener’s granulomatosis) (GPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) and microscopic polyangiitis (MPA). Other forms of vasculitis (listed in Table 1) are usually ANCA negative and are defined by their clinico-pathological features. There are no validated diagnostic criteria for AAV. The American College of Rheumatology (ACR) devised classification criteria for different vasculitides including GPA and EGPA but not MPA, and the Chapel Hill consensus conference (CHCC) in 1994 recommended definitions for GPA, EGPA and MPA[1-4] .The CHCC definitions were not intended for classification or diagnosis but provide a useful description of disease and include some features that have been used for classification purposes. The CHCC definitions were updated in 2012 to accommodate developments in knowledge about aetiopathogenesis and ANCA [5]. Lanham reviewed EGPA in 1984 and provided a slightly different and mainly clinically orientated set of classification criteria when compared to the ACR for EGPA [6]].Table 1 Classification of the Vasculitides Adopted by the 2011-2012 International Chapel Hill Consensus Conference Nomenclature of the VasculitidesLarge Vessel Vasculitis Takayasu Arteritis Giant Cell Arteritis Medium Vessel Vasculitis Polyarteritis Nodosa Kawasaki Disease Small Vessel Vasculitis ANCA-Associated Vas