Prevalence, serological features, response to treatment and outcome of critical peripheral ischaemia in a cohort of lupus patients
Prevalence, serological features, response to treatment and outcome of critical peripheral ischaemia in a cohort of lupus patients
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DOI：
10.1093/rheumatology/ken210
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发表时间：
2008-09-01
期刊：
影响因子：
5.5
通讯作者：
Isenberg, D. A.
中科院分区：
文献类型：
--
作者：
Jeffery, R. C.;Narshi, C. B.;Isenberg, D. A.
Objective.本研究旨在探讨SLE患者发生严重外周缺血（CPI）和坏疽的危险因素和治疗方法，并提出利妥昔单抗作为一种新的治疗方法。我们对485例在英国三级转诊中心就诊的SLE患者进行了回顾性研究，随访超过27年，评估了人口统计学、临床特征、血清学特征、治疗和结局数据。485例患者中有7例（1.4%）在SLE疾病的任何阶段（从出现症状到发病后27年）均有坏疽或CPI的证据，CPI患者中aPL和LAC的比例过高。所有患者在CPI时均患有活动性SLE。所有7例患者均接受静脉（IV）依前列醇输注治疗，aPL阳性患者接受抗凝治疗。1例患者对该治疗和IV降钙素基因相关肽治疗无效，但对利妥昔单抗B细胞耗竭治疗有效。7例患者中5例出现手指缺损伴自体截肢。CPI是一种罕见的但潜在破坏性的SLE并发症，与aPL、LAC和活动性SLE相关。利妥昔单抗的B细胞耗竭治疗可能是IV依前列醇未改善的重度缺血的一种选择。
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.