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Primary systemic vasculitis

This section deals with the clinical condition primary systemic vasculitis, listing the relevant immunological tests, together with a short explanation of their use.

Relevant immunological tests:

Some forms of systemic vasculitis are strongly associated with circulating anti-neutrophil cytoplasmic antigens (ANCA).

In Wegener's Granulomatosis, (WG) (lung, renal) there is a diffuse cytoplasmic pattern (c-ANCA), as well as polyclonal elevations of IgG, IgA, IgE and raised CRP.

ANCAs with a perinuclear pattern (p-ANCA) are seen in some patients with polyarteritis nodosa, (PAN) (weight loss, musculoskeletal, renal) and Churg-Strauss ("asthma", eosinophilia, hypocomplemenaemia, raised IgE).

Both types of ANCA may be seen in microscopic polyarteritis (MPA) (clinical overlap between classic PAN and WG).

Atypical forms of ANCA reactivity may be seen in association with Henoch-Schönlein Purpura (sometimes IgA raised), Kawasaki's syndrome, and in other autoimmune disorders (eg. SLE, ulcerative colitis).


(Last updated October 9th 2014)