Severe gangrene in a patient with anti-RNP positive limited cutaneous systemic sclerosis/rheumatoid arthritis overlap syndrome caused by vasculopathy and vasculitis

Abstract

In this paper, we describe a case of a male patient with anti-U1RNP positive limited cutaneous sys-temic sclerosis/rheumatoid arthritis overlap syndrome, who presented acutely with rapidly progres-sive digital ischemia, which lead to extensive gangrene. Management with conventional vasodilatortherapy was unsuccessful. There were constitutional symptoms and a marked inflammatory responsein the absence of evidence of infection, implying a component of vasculitis underlying the presenta-tion. Treatment with immunosuppression and intravenous immunoglobulin led to resolution of theinflammatory process with no further progression of tissue necrosis. Here we discuss pertinent issuesraised by the case, including the management of digital ischemia and gangrene in this context andthe relevance of the anti-U1RNP in systemic sclerosis overlap syndromes.