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I.P. Giles

and David A. Isenberg


July 30, 2015: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.


Revised (2012) International Chapel Hill Consensus Conference Nomenclature of Vasculitides.

Updated on 29 Aug 2013. The previous version of this content can be found here.
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date: 28 April 2017

About 1 in 20 people develop an autoimmune disease, many of which involve the musculoskeletal system. Young women are particularly at risk, but the development at any age of symptoms such as unexplained fever, rash, polyarthritis, Raynaud’s phenomenon or mouth ulcers should encourage serological screening for autoimmune rheumatic or vasculitic disorder.

Aetiology and pathogenesis—common to all of the autoimmune rheumatic diseases is the phenomenon of production of autoantibodies by activated B cells. In the primary vasculitides, a pathogenic role has been proposed for antiendothelial cell antibodies and sensitized T cells, but undoubtedly the most important role is that of antineutrophil cytoplasmic antibodies (ANCA).

Diagnosis—detection of antinuclear antibodies or rheumatoid factor in high titre favours the diagnosis of an autoimmune rheumatic disease and should lead to a search for more specific autoantibodies, e.g. anti-dsDNA linked to lupus, anticyclic citrullinated peptides linked to rheumatoid arthritis, antineutrophil cytoplasmic antibodies (ANCA) linked to granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Well-established and validated criteria have been set up for all the main autoimmune rheumatic diseases and vasculitides, but there is significant overlap between them. Physicians treating patients with these conditions need to be constantly aware of the possibility of organ involvement because prompt diagnosis and treatment may be necessary to prevent irreversible damage.

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