Rheumatoid vasculitis (RV) is an uncommon but potentially fatal extra-articular manifestation of rheumatoid arthritis (RA), characterized by severe systemic inflammation and high mortality despite advances in disease-modifying therapies. We describe a 52-year-old woman with long-standing seropositive RA and irregular use of methotrexate who presented with progressive digital ischemia and extensive necrotic cutaneous ulcers, in the absence of active synovitis. Histopathology revealed small-vessel vasculitis, accompanied by markedly elevated rheumatoid factor and anti-cyclic citrullinated peptide titers, hypocomplementemia, and negative antineutrophil cytoplasmic antibodies. Despite high-dose corticosteroids, vasodilator therapy, and broad-spectrum antibiotics, the disease rapidly evolved to multiorgan involvement, including respiratory failure requiring mechanical ventilation, rapidly progressive renal failure requiring hemodialysis, and ischemic central nervous system lesions, culminating in refractory multiorgan failure and death. This case illustrates a fulminant presentation of RV with aggressive cutaneous and visceral involvement, emphasizing that severe vasculitis may occur independently of articular disease activity and is associated with poor prognosis. RV should be suspected in patients with long-standing RA who develop unexplained ischemic or ulcerative lesions, even in the absence of active arthritis. Early recognition and timely, sustained immunosuppression are critical to prevent irreversible organ damage and fatal outcomes.
Montenegro et al. (Fri,) studied this question.