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Rheumatoid vasculitis
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Rheumatoid vasculitis

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Contributors: Edward Li PhD, Art Papier MD, Susan Burgin MD
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Synopsis

Rheumatoid vasculitis (RV) is a rare complication of rheumatoid arthritis (RA). RV usually occurs in the setting of prolonged severe RA with high rheumatoid factor (RF) titers, typically over a decade after the initial diagnosis of RA. Disease incidence has decreased from approximately 5% to 1%, most likely due to improvements in the clinical management of RA.

RV can affect blood vessels of any size and can cause clinical manifestations corresponding to the size of the blood vessels affected. It most commonly presents with petechiae and palpable purpura, which are manifestations of small-vessel disease. In severe presentations of RV characterized by medium-vessel involvement, nodules, ulcerations, infarctions, and necrosis can occur, reminiscent of polyarteritis nodosa. Manifestations of RV visceral organ involvement include aortitis, peripheral neuropathy, pericarditis, glomerulonephritis, scleritis, and gastrointestinal bleeding.

Genetic factors such as major histocompatibility (MHC) isoforms contribute to RV disease susceptibility. Thus far, cigarette smoking is the only environmental factor implicated in disease pathogenesis. Numerous viral infections and medications have been hypothesized to be triggers for RV without conclusive evidence.

Codes

ICD10CM:
M05.20 – Rheumatoid vasculitis with rheumatoid arthritis of unspecified site

SNOMEDCT:
400054000 – Rheumatoid vasculitis

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Last Reviewed: 02/18/2019
Last Updated: 02/25/2019
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Rheumatoid vasculitis
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Rheumatoid vasculitis : Erythema, Fingers, Malaise, Palpable purpura, Skin ulcer, Toes, Arthralgia, RF positive, Lower legs
Clinical image of Rheumatoid vasculitis
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