Critical peripheral ischemia precipitated by severe episode of Raynaud’s phenomenon in a patient with aPL-positive systemic lupus erythematosus, upon high titer anti-RNP seroconversion

Abstract

A 35-year-old female with long standing aPL-positive lupus without history of thromboembolic events, who has developed critical peripheral ischemia (CPI) is described. An episode of severe Raynaud's phenomenon rapidly progressed to an extensive digit-threatening ischemia, involving bilateral hands and feet. She was successfully treated with corticosteroids, anticoagulation, iloprost, sildenafil, and nifedipine. Her serological studies were remarkable for the emergence of high titer anti-RNP seroconversion and an increase in aPL titer, suggesting that these autoantibodies played a role in the pathogenesis of CPI. It is important to note that such observation should herald this potentially devastating complication of systemic lupus erythematosus.

Systemic lupus erythematosus and Raynaud's phenomenon

“…Some authors have regarded it merely as a disease marker. 13 Interestingly, Levy et al 18 described the case of a lupus patient in which the appearance of severe RP was associated with the emergence of high titer anti-RNP seroconversion, suggesting that these autoantibodies may play a role in the pathogenesis of peripheral ischemic attacks.…”