摘要
目的探讨巩膜炎与肉芽肿性多血管炎（GPA）的临床表现之间的关系。方法收集2012年3月至2016年7月就诊于首都医科大学附属北京同仁医院风湿科的资料完整的GPA 102例，其中43例GPA伴巩膜炎，对患者临床资料进行回顾性病例分析。定量资料采用独立样本t检验和Mann-Whitney U检验，分类变量资料采用χ2检验。结果GPA的眼部表现以巩膜炎为主，GPA合并巩膜炎与未合并巩膜炎组相比，肾损害的发生率（37.2%和11.9%，χ2=9.148，P=0.002）、疾病活动度（BVAS/GPA评分8.9和5.1，t=7.847，P=0.000）、疾病累及范围（DEI评分7和4，Z=5.919，P=0.000）及炎症指标（ESR 56 mm/1 h和36 mm/1 h，Z=2.456，P=0.016；CRP 51 mg/L和8 mg/L，Z=3.784，P=0.000）、中性粒细胞百分比（70%和61%，t=3.993，P=0.000）、补体C3水平（1.2 g/L和1.0 g/L，Z=2.652，P=0.009）、血浆纤维蛋白原（3.5 g/L和3.3 g/L，Z=3.947，P=0.000）及D-二聚体水平（0.5 mg/L FEU和0.3 mg/L FEU，Z=2.371，P=0.018）、ANCA阳性率较高（90.7%和57.6%，χ2=13.369，P=0.000），预后较差（2009-FFS 0.4和0.1，Z=2.600，P=0.009），而声门下狭窄发生率明显低于未合并巩膜炎组（4.7%和20.3%，χ2=5.169，P=0.023）。广义的巩膜炎分为表层巩膜炎和巩膜炎，2组之间在临床表现、诊断时疾病活动度、炎症指标及预后方面差异无统计学意义。结论伴巩膜炎的GPA在疾病诊断时具有较高的疾病活动度及疾病累及范围，预后较差，常需要积极的治疗，而声门下累及少见。巩膜炎类型与疾病活动度及严重程度无关。
Objective To investigate the clinical characteristics of granulomatosis with polyangiitis （GPA） patients with scleritis.
MethodsThe completed clinical data of 102 GPA patients from March 2012 to July 2016 in Capital Medical University, Tong Ren Hospital were collected. Altogether 43 GPA patients with scleritis were enrolled, and the clinical data were analyzed respectively. The t test, Mann-Whitney U test andχ2 testwere used to analyze the data.ResultsThe most common ocular manifestation in GPA was scleritis. The incidence of renal damage （37.2% vs 11.9%, χ2=9.148, P=0.002）, disease activity （BVAS/GPA score 8.9 vs 5.1, Z=7.847, P=0.000）, DEI score （7 vs 4, Z=5.919, P=0.000）, inflammation index （56 mm/1 h vs 36 mm/1 h, Z=2.456, P=0.016; CRP 51 mg/L vs 8 mg/L, Z=3.748, P=0.000）, neutrophil percent （70% vs 61%, t=3.993, P=0.000）, complement 3（1.2 g/L vs 1.0 g/L, t=2.652, P=0.009）, plasma fibrinogen （3.5 g/L vs 3.3 g/L, t=3.947, P=0.000）, D-dimmer （0.5 mg/L FEU vs 0.3 mg/L FEU, Z=2.371, P=0.018） and ANCA positive rate （90.7% vs 57.6%, χ2=13.369, P=0.000） were higher in patients with scleritis, as well as a poor prognosis （2009-FFS 0.4 vs 0.1, Z=2.600, P=0.009）. However, a lower rate of subglottic involvement was detected in scleritis group （4.7% vs 20.3%, χ2=5.169, P=0.023）. There were no significant differences between GPA patients with scleritis and episcleritis in clinical feature, disease activity, inflammatory index and prognosis. Aggressive immunosuppres-sive treatment was appropriate for GPA with scleritis. Tumor necrosis factor （TNF）-аinhibitor was an effective treatment.ConclusionGPA with scleritis usually suffered from higher disease activity index, broader DEI, as well as poorer prognosis. Aggressive treatment should be initiated in order to achieve disease remission. Subglottic involvement is less common in patients with scleritis. The phenotype of scleritis is not associated with disease severity.
出处
《中华风湿病学杂志》
CSCD
北大核心
2017年第10期673-679,共7页
Chinese Journal of Rheumatology
基金
首都医科大学附属北京同仁医院科研骨干基金（2015-YJJ-GGL-016）
关键词
巩膜炎
肉芽肿性多血管炎
生物制剂
Scleritis
Granulomatosis with polyangiitis
Biological agents
