目的分析总结狼疮肾炎(lupus nephritis,LN)患者的临床特征及初始治疗选择,提高对中国LN患者的认识。方法基于中国系统性红斑狼疮(systemic lupus erythematosus,SLE)研究协作组(Chinese SLE Treatment and Research group,CSTAR)建立的...目的分析总结狼疮肾炎(lupus nephritis,LN)患者的临床特征及初始治疗选择,提高对中国LN患者的认识。方法基于中国系统性红斑狼疮(systemic lupus erythematosus,SLE)研究协作组(Chinese SLE Treatment and Research group,CSTAR)建立的SLE起始队列,分析2009年2月至2021年3月登记注册的确诊时间在3个月内的8713例SLE患者及其中LN患者的基线人口学特征、临床表现、实验室检查以及治疗选择。结果在初始诊断的SLE患者中,LN发生率为33.3%(2900/8713)。LN患者肾脏病理活检以Ⅳ型LN最多见(35.1%)。LN组患者男性比例更高(12.0%vs.7.7%,P<0.001),SLEDAI评分更高(11.7±8.5 vs.6.6±6.2,P<0.001),SLICC/ACR脏器损伤评分≥1分的比例更高(34.0%vs.16.6%,P<0.001)。单因素及多因素分析结果显示血小板减少(23.2%vs.16.6%,P<0.001)、浆膜炎(19.2%vs.8.4%,P<0.001)、神经精神狼疮(7.6%vs.5.5%,P=0.001)、肌炎(3.3%vs.2.1%,P=0.006)、低补体血症(66.0%vs.53.1%,P<0.001)、抗dsDNA阳性(54.4%vs.42.4%,P<0.001)与LN相关,而LN患者关节炎发生比例降低(26.8%vs.30.4%,P<0.001)。与非LN组相比,LN组患者初始治疗方案中糖皮质激素(90.7%vs.84.1%,P<0.001)及激素冲击治疗(9.1%vs.3.4%,P<0.001)、环磷酰胺(27.0%vs.11.7%,P<0.001)、霉酚酸酯(21.6%vs.10.1%,P<0.001)、他克莫司(4.8%vs.2.3%,P<0.001)应用比例显著增高,而羟氯喹(67.4%vs.73.1%,P<0.001)、环孢素(4.3%vs.5.9%,P=0.002)应用比例显著降低。结论SLE中LN组较非LN组患者男性比例更高,病情活动度高,脏器损伤重。LN与浆膜炎、血小板减少等提示SLE病情活动的临床表现及实验室指标相关,初始治疗更积极。展开更多
We describe a case of systemic sclerosis (SSc) complicated with portal hypertensive ascites which did not improve with diuretics and ascitic drainage. When corticosteroid added, her ascites diminished dramatically. ...We describe a case of systemic sclerosis (SSc) complicated with portal hypertensive ascites which did not improve with diuretics and ascitic drainage. When corticosteroid added, her ascites diminished dramatically. Though portal hypertension can be imputed to other causes, such as polycystic liver in this case, it can occur in limited SSc with positive anti-centromere antibody and respond to corticosteroid treatment.展开更多
文摘目的分析总结狼疮肾炎(lupus nephritis,LN)患者的临床特征及初始治疗选择,提高对中国LN患者的认识。方法基于中国系统性红斑狼疮(systemic lupus erythematosus,SLE)研究协作组(Chinese SLE Treatment and Research group,CSTAR)建立的SLE起始队列,分析2009年2月至2021年3月登记注册的确诊时间在3个月内的8713例SLE患者及其中LN患者的基线人口学特征、临床表现、实验室检查以及治疗选择。结果在初始诊断的SLE患者中,LN发生率为33.3%(2900/8713)。LN患者肾脏病理活检以Ⅳ型LN最多见(35.1%)。LN组患者男性比例更高(12.0%vs.7.7%,P<0.001),SLEDAI评分更高(11.7±8.5 vs.6.6±6.2,P<0.001),SLICC/ACR脏器损伤评分≥1分的比例更高(34.0%vs.16.6%,P<0.001)。单因素及多因素分析结果显示血小板减少(23.2%vs.16.6%,P<0.001)、浆膜炎(19.2%vs.8.4%,P<0.001)、神经精神狼疮(7.6%vs.5.5%,P=0.001)、肌炎(3.3%vs.2.1%,P=0.006)、低补体血症(66.0%vs.53.1%,P<0.001)、抗dsDNA阳性(54.4%vs.42.4%,P<0.001)与LN相关,而LN患者关节炎发生比例降低(26.8%vs.30.4%,P<0.001)。与非LN组相比,LN组患者初始治疗方案中糖皮质激素(90.7%vs.84.1%,P<0.001)及激素冲击治疗(9.1%vs.3.4%,P<0.001)、环磷酰胺(27.0%vs.11.7%,P<0.001)、霉酚酸酯(21.6%vs.10.1%,P<0.001)、他克莫司(4.8%vs.2.3%,P<0.001)应用比例显著增高,而羟氯喹(67.4%vs.73.1%,P<0.001)、环孢素(4.3%vs.5.9%,P=0.002)应用比例显著降低。结论SLE中LN组较非LN组患者男性比例更高,病情活动度高,脏器损伤重。LN与浆膜炎、血小板减少等提示SLE病情活动的临床表现及实验室指标相关,初始治疗更积极。
文摘We describe a case of systemic sclerosis (SSc) complicated with portal hypertensive ascites which did not improve with diuretics and ascitic drainage. When corticosteroid added, her ascites diminished dramatically. Though portal hypertension can be imputed to other causes, such as polycystic liver in this case, it can occur in limited SSc with positive anti-centromere antibody and respond to corticosteroid treatment.