Abstract

Abstract Background/Aims Gangrene is a well-recognised complication of diabetes, peripheral arterial disease, frostbite and meningococcal disease amongst other conditions but is rare in patients with systemic lupus erythematosus [SLE]. Most reports describe individual patients and the prevalence in cohorts is very scarce. In 2008, our group reported five patients who had developed digital gangrene and two patients with “near gangrene”. We reviewed all the SLE patients seen in our lupus clinic between January 1978 and June 2022.The main goal was to address the prevalence and identify the possible trigger factors, aiming to find common clinical and serological features. We have also reviewed the acute and long-term management of gangrene in these patients as well as their long-term outcome. Methods We reviewed 820 patients with SLE attending the SLE Clinic in our UK tertiary referral center, followed up over 44 years, assessing demographics, clinical and serological features, treatment in the acute phase, outcome and long-term management. Results Eight out of 820 patients (0.97%) have developed gangrene. The age of onset of disease ranged from 12 to 26 years (mean 17.5 years, SD 4.609). The first episode of gangrene ranged from between presentation to 27 years after SLE onset, with a mean duration of SLE at the onset of the gangrene of 15.5 years (SD 11.711). All had active SLE at the time of critical peripheral ischaemia and anti-phospholipid (PL) antibodies were present in five out of the eight reported patients. The acute treatment was intravenous (IV) iloprost infusions with antiphospholipid-positive patients being anti-coagulated, most staying on long term anticoagulation therapy. The possible identified triggers (such as infection and cancer) were treated accordingly. Two patients did not respond to the initial treatment and needed further immunosuppression. All of these patients suffered digit loss. Conclusion Gangrene is a rare complication of SLE in patients with active disease but potentially able to cause severe damage. Anti-phospholipid antibodies are overrepresented in these patients but infection and cancer are other possible contributors. Anticoagulation therapy, steroids and iloprost are the first line therapy but in non-responders further immunosuppression may be needed to stop the evolution of gangrene. Disclosure J. Rua: None. D. Isenberg: Honoraria; Servier, Anges, Astrazeneca, UCB, Eli Lilly.

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