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Marantic endocarditis is an extremely rare extra-articular complication of rheumatoid arthritis. To date, documented cases typically occurred in the absence of other systemic features of disease activity. We report a rare and exceptional example of marantic endocarditis secondary to fulminant systemic rheumatoid vasculitis with multi-organ disease. Findings from this case and literature review suggest that marantic endocarditis associated with rheumatoid vasculitis displays a tendency to affect the mitral valve with high risk of embolization.

Small vessel multi-organ vasculitis and marantic endocarditis complicating rheumatoid arthritis