Criteria - both of the following:
(1) presence of rheumatoid arthritis (meets ACR criteria)
(2) one or more of the following:
(2a) mononeuritis multiplex
(2b) acute peripheral neuropathy
(2c) peripheral gangrene
(2d) biopsy evidence of acute necrotizing vasculitis AND systemic symptoms (fever, anorexia with weight loss, sweats, etc.)
(2e) biopsy evidence of acute necrotizing vasculitis AND deep cutaneous ulcers
(2f) biopsy evidence of acute necrotizing vasculitis AND active extra-articular disease (pleuritis, pericarditis, scleritis)
(2g) typical digital infarcts AND deep cutaneous ulcers
(2g) typical digital infarcts AND active extra-articular disease (pleuritis, pericarditis, scleritis)
Exclusions:
(1) diabetic arteriopathy
(2) digital infarcts alone
(3) digital nailfold infarcts alone
(4) atherosclerosis
Differential diagnosis:
(1) overlap syndrome
Limitation:
• The criteria have not (as of 1998) been validated formally.
