Prevalence, serological features, response to treatment and outcome of critical peripheral ischaemia in a cohort of lupus patients.

Objective: This study addresses the issue of risk factors and management of critical peripheral ischaemia (CPI) and gangrene in SLE and proposes rituximab as a novel therapy.

Method: We conducted a retrospective study of 485 patients with SLE attending a UK tertiary referral centre, followed up over 27 yrs. Demographics, clinical features, serological features, treatment and outcome data were assessed.

Results: Seven out of 485 patients (1.4%) had evidence of gangrene or CPI with onset at any stage of SLE disease from presenting feature to 27 yrs after SLE onset, aPL and LAC were over-represented in the CPI patients. All had active SLE at the time of CPI. All seven were treated with intravenous (IV) epoprostenol infusion and aPL-positive patients were anti-coagulated. One patient failed to respond to this treatment and to IV calcitonin gene-related peptide but responded to B-cell depletion therapy using rituximab. Five out of the seven patients suffered digit loss with auto-amputation.

Conclusion: CPI is a rare but potentially devastating complication of SLE associated with aPL, LAC and active SLE. B-cell depletion therapy with rituximab may be an option in severe ischaemia not improving with IV epoprostenol.