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Rheumatology Advance Access originally published online on June 27, 2008
Rheumatology 2008 47(9):1379-1383; doi:10.1093/rheumatology/ken210
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

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

1Department of Rheumatology, Northampton General Hospital, Northampton and 2University College Hospital, Centre for Rheumatology Research, London, UK.

Correspondence to: D. A. Isenberg, Royal Free and University College Medical School, University College London Division of Medicine, Centre for Rheumatology Research, Room 331, 3rd floor, Windeyer Building, 46 Cleveland Street, London W1T 4JF, UK. E-mail: d.isenberg{at}ucl.ac.uk


  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.

KEY WORDS: Systemic lupus erythematosus, Anti-phospholipid syndrome, Digital ischaemia, Gangrene, Epoprostenol, Rituximab, B-cell depletion therapy

Submitted 30 January 2008; revised version accepted 30 April 2008.
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