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Primary vasculitides 

Primary vasculitides
Primary vasculitides

Gavin Clunie

, Nick Wilkinson

, Elena Nikiphorou

, and Deepak R. Jadon

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date: 26 June 2022

This chapter describes large, medium, and small vessel primary vasculitides. The 2012 Chapel Hill Consensus Conference nomenclature of vasculitis is introduced, and acts as a framework for more detailed descriptions of polymyalgia rheumatic and giant cell arteritis, polyarteritis nodosa (systemic and cutaneous), granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, Takayasu arteritis, childhood-onset vasculitis, Kawasaki disease, Henoch–Schönlein purpura, and leucocytoclastic vasculitis. Other antineutrophil cytoplasmic antibody-associated vasculitides and primary angiitis of central nervous system in children are also covered. The epidemiology, aetiopathogenesis, classification criteria, presenting features, clinical manifestations, and management of these diseases are presented. In particular, diagnostic criteria for and mimics of polymyalgia rheumatic and giant cell arteritis are discussed in detail to permit rheumatologists and non-rheumatologists to confidently manage them.

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