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

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Rheumatology Advance Access published online on June 27, 2008

Rheumatology, doi:10.1093/rheumatology/ken210

Prevalence, serological features, response to treatment and outcome of

critical peripheral ischaemia in a cohort of lupus patients

R. C. Jeffery1, C. B. Narshi2 and D. A. Isenberg2

Abstract

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.

http://rheumatology.oxfordjournals.org/cgi/content/abstract/ken210v1

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