背景:关于系统性红斑狼疮(SLE)中数字缺血的文献有限。我们报告了患病率,来自印度SLE起始队列(INSPIRE)的数字梗塞和坏疽的关联和结局。
方法:从基于Web的INSPIRE数据库,我们在纳入队列时提取了“数字梗死”和“数字坏疽”患者的信息,一起被认为是严重的外周缺血(CPI);所有其他均为对照。我们描述了CPI与SLE临床表型的关联,自身抗体,和入学时的疾病活动。我们还报告了短期结果,即。迄今为止,数字组织丢失和长达6个月的早期死亡率以及数字缺血事件的复发。
结果:在纳入INSPIRE队列的2503例SLE患者中,我们确定了75(2.9%)的CPI患者,72(96%)妇女和6(8%)儿童。其中,55例(73.3%)患有数字坏疽,21例(28%)患有数字梗塞。大多数数字坏疽导致末端指骨远端截肢(63.6%)。多因素分析显示肺动脉高压AOR[6.34(1.99,20.2)],共存血栓形成AOR[27.8(15.7,48.7)],三联抗磷脂抗体阳性AOR[5.36(1.67,16.9)]和存在抗Scl-70抗体AOR[5.59(1.86,16.7)]的可能性更大,而患有3类或4类狼疮性肾炎的患者AOR[0.37(0.15,0.95)]和抗核小体抗体AOR[0.47(0.23,0.99)]的患者与CPI相关的可能性更低.病例和对照组之间的SLEDAI和短期死亡率相似。
结论:与早期报告相比,INSPIRE队列中SLE患者的CPI发生率更高(2.9%)。血栓前状态和血管病变均有助于其发生。
BACKGROUND: There is limited literature on digital ischemia in systemic Lupus erythematosus (SLE). We report the prevalence, associations and outcome of digital infarcts and gangrene from the Indian SLE inception cohort (INSPIRE).
METHODS: From the web-based database of INSPIRE, we extracted information for patients with \'Digital Infarct\' and \'Digital gangrene\' at enrolment into cohort, together considered as critical peripheral ischemia (CPI); all others were controls. We describe the associations of CPI with SLE clinical phenotype, autoantibodies, and disease activity at enrolment. We also report short term outcomes viz. Digital tissue loss and early mortality up to 6 months and recurrence of digital ischemic events in cases till date.
RESULTS: Of 2503 SLE patients enrolled into the INSPIRE cohort, we identified 75 (2.9%) patients with CPI, 72 (96%) women and 6 (8%) children. Of them, 55 (73.3%) had digital gangrene and 21 (28%) patients had digital infarcts. Majority of digital gangrene resulted in amputation distal to terminal phalanx (63.6%). Multivariable analysis showed that pulmonary hypertension AOR [6.34 (1.99, 20.2)], coexistent thrombosis AOR [27.8 (15.7, 48.7)], triple antiphospholipid antibody positivity AOR [5.36 (1.67, 16.9)] and the presence of anti-Scl-70-antibody AOR [5.59 (1.86, 16.7)] were more likely while patients with class 3 or 4 lupus nephritis AOR [0.37 (0.15, 0.95)] and anti-nucleosome antibodies AOR [0.47 (0.23, 0.99)] were less likely to be associated with CPI. SLEDAI and short-term mortality were similar between cases and controls.
CONCLUSIONS: CPI occurred in a higher proportion (2.9%) of SLE patients in the INSPIRE cohort as compared to earlier reports. Both prothrombotic state and
vasculopathy contribute to its occurrence.