title: Prevalence, serological features, response to treatment and outcome of critical peripheral ischaemia in a cohort of lupus patients.
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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: R C
authors.initials: RC
authors.lastname: Jeffery
authors.forename: C B
authors.initials: CB
authors.lastname: Narshi
authors.forename: D A
authors.initials: DA
authors.lastname: Isenberg
mesh_terms: D000293:Adolescent; D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents / Q000627:therapeutic use; D005260:Female; D005385:Fingers / Q000098:blood supply*; D005734:Gangrene / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D007511:Ischemia / Q000188:drug therapy / Q000209:etiology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab; D014034:Toes / Q000098:blood supply*; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents; D000069283:Rituximab