误诊为肺结核的抗中性粒细胞胞浆抗体相关性小血管炎

目的探讨抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎(AASV)的诊治要点。方法对我科收治的AASV 1例的临床资料进行回顾性分析。结果本例因发热、咳嗽、痰中带血伴腹痛1个月入院,在外院诊断为肺结核,予抗结核治疗10 d病情无缓解到我院就诊。痰查抗酸杆菌、结核抗体、癌细胞均(-);尿红细胞、尿蛋白均(+),血肌酐345.7μmol/L;行肾脏穿刺活检示新月体性肾小球肾炎,ANCA及相关抗体(+),结合患者有因甲状腺功能亢进症长期口服丙硫氧嘧啶(PTU)史,确诊为PTU诱发AASV。予大剂量甲泼尼龙冲击治疗、抗感染及对症支持治疗后病情缓解,出院。出院后继续予泼尼松口服维持并逐步减量,1年后随访痊愈。结论 AASV临床较少见,早期症状不典型,易误诊。对有肾功能及多系统损害症状者应及时行ANCA、肾活检等相关检查,同时重视采集病史、用药史,以提高确诊率。     

抗中性粒细胞胞浆抗体与变应性血管炎

应用间接免疫荧光法（ＩＩＦ）对１１例变应性血管炎进行了抗中性粒细胞胞浆抗体（ＡＮＣＡ）检测，结果伴有肾损寄的３例老年男性阳性，均为核周型ＡＮＣＡ。激素和免疫抑制剂治疗效果好。认为ＡＮＣＡ阳性的变应性血管炎可能有系统损害。     

抗中性粒细胞胞浆抗体相关性血管炎研究进展

抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)是一类以小血管壁炎症和纤维素样坏死为特征,累及全身多系统的自身免疫性疾病。亚洲地区AAV以显微镜下多血管炎(MPA)为主,最常累及肾和肺。近几年发现补体,例如C5a,通过替代途径在AAV的发病机制中发挥着重要作用,所以了解AAV补体与凝血过程之间的联系有利于从发病机制出发找到治疗疾病的靶点。本综述总结AAV发病机制中凝血及纤溶系统变化及与补体系统相互作用的研究进展。     

抗中性粒细胞胞浆抗体相关性血管炎2例误诊分析

<正>系统性血管炎是一组由血管炎性病变引起的疾病的总称,可以累及动脉、静脉、毛细血管甚至淋巴管。因其受累血管的部位、大小、类型及病理等各不相同,使其临床呈现多样化的特点。目前已经认识的系统性血管炎至少有20种。按照受累血管的大小进行分类,包括小血管(毛细血管和毛细血管后微静     

抗中性粒细胞胞浆抗体相关性小血管炎合并血栓栓塞的危险因素分析

目的分析抗中性粒细胞胞浆抗体相关性小血管炎(AAV)合并血栓栓塞事件的临床特征以及相关危险因素。方法对我院2013年1月至2018年12月期间确诊为AAV患者177例,其中28例合并动静脉血栓栓塞,149例未合并动静脉血栓栓塞。采用多因素logistic回归分析AAV合并血栓栓塞的高危因素。结果栓塞组发生在AAV活动期23例(82.1%),发生在缓解期5例(17.9%),50.0%发生在疾病诊断后2个月。栓塞组合并急性感染率、BVAS评分、血清肌酐、ESR水平高于未栓塞组,但血小板计数、C3水平低于未栓塞组(P0.05)。多因素回归分析显示,合并急性感染、ESR、C3水平是AAV合并血栓栓塞的独立危险因素。结论 AAV患者活动期,尤其在疾病诊断后的2个月,发生血栓栓塞的风险较高。合并急性感染、ESR升高、C3降低的AAV患者可能更易合并血栓栓塞。     

抗中性粒细胞胞浆抗体相关性血管炎的免疫抑制剂治疗进展

原发性小血管炎是目前病因尚未明确的一类小血管炎,以血管壁炎性反应、纤维素样坏死为病理特征的自身免疫性疾病。在原发性小血管炎中抗中性粒细胞胞浆抗体(ANCA)的检出率高因而又称之为ANCA相关小血管炎(AAV),包括     

抗中性粒细胞胞浆抗体相关性血管炎

1 引言 抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody,ANCA)是第一个被证实与血管炎相关的自身抗体,与其相关的血管炎包括韦格纳肉芽肿(Wegener granulomatosis,WG)、变应性肉芽肿血管炎[又称Churg-Strauss综合征(Churg-Strauss syndrome,CSS)]、显微镜下多血管炎(microscopic polyangiitis,MPA)等.而WG、CSS和MPA具有相似的病理改变(主要累及小血管)、临床特点(具有肺、肾小球损害)和实验室检查(ANCA阳性),因此有学者将这3种小血管炎合称为ANCA相关性血管炎.     

1例抗中性粒细胞胞浆抗体相关性血管炎肾损害患儿的护理体会

目的 总结1例抗中性粒细胞胞浆抗体相关性血管炎肾损害患儿的护理体会。方法 对我科收治的1例抗中性粒细胞胞浆抗体相关性血管炎肾损害患儿的护理方法进行分析、总结、制定针对性的护理措施。结果 经过对症治疗和综合护理干预,该患儿病情得到了控制,逐渐好转,促进了疾病的康复。     

老年抗中性粒细胞胞浆抗体相关性血管炎诊治8例并文献复习

回顾分析8例以肺部感染为首发症状的抗中性粒细胞胞浆抗体相关性血管炎(antineutrophil cytoplasmic autoantibodyassociated vasculitides,ANCA-AAV)老年患者的临床资料,探讨以肺部感染症状为主要表现的ANCA-AAV的临床特征与诊治方法。8例中6例患者接受甲泼尼龙1 mg/(kg·d)治疗4~6周,病情控制后改为泼尼松,逐渐减量,其中3例同时接受免疫抑制剂环磷酰胺2 mg/(kg·d)口服1年;1例加用雷公藤1 mg/(kg·d)治疗3个月;2例因出现肾功能衰竭病死,未用糖皮质激素治疗。8例患者中,除2例因肾功能衰竭死亡外,1例因呼吸功能衰竭死亡,5例患者经治疗后咳嗽、咳痰、贫血、乏力、肌肉酸痛等症状明显好转,体温恢复正常。因此,对于临床表现缺乏特异性且抗感染等疗效不佳的患者应尽早行ANCA检测。糖皮质激素治疗能改善ANCA-AAV患者的预后。     

抗中性粒细胞胞浆抗体相关性小血管炎肺部表现二例

例1,患者男,62岁,因慢性咳嗽、咳痰、呼吸困难3个月、加重伴痰中带血10d入院。既往：双侧肘关节风湿性关节炎病史7年。入院查体：一般状态差,胸廓对称,双肺呼吸运动度对称,听诊双肺呼吸音粗,双肺可闻及散在湿啰音,少量干啰音,未闻及胸膜摩擦音。     

肺血管炎361例临床分析

目的探讨肺血管炎的临床特点。方法回顾性分析361例肺血管炎患者的临床资料,观察疾病特点及在民族、性别等方面的差异。结果肺血管炎女性发病率（85.6%）高于男性（14.4%）（P〈0.05）,汉族、维吾尔族、哈萨克族和回族肺血管炎发病率（54.8%、28.3%、11.9%、4.99%）比较差异无统计学意义（P〉0.05）;咳嗽、发热为肺血管炎常见临床症状,其中121例有多脏器受累,可继发于系统性红斑狼疮、类风湿性关节炎、干燥综合征等疾病;肺部高分辨率CT可见网状改变、肺泡病变、囊状改变及结节改变;抗中性粒细胞胞质抗体阳性63例（17.5%）。结论肺血管炎发生率在民族间未发现存在差异,多见于女性,临床症状不典型,有多脏器受累的特点;动态检测抗中性粒细胞胞质抗体或行组织活检病理检查有助于疾病的诊断。     

Serum progranulin as a predictive marker for high activity of antineutrophil cytoplasmic antibody‐associated vasculitis

BackgroundThis study investigated whether serum progranulin could act as a predictive marker for high disease activity of antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis (AAV).MethodsFifty‐eight AAV patients were included in this study. Clinical and laboratory data were obtained at blood collection. The Short‐Form 36‐Item Health Survey Physical and Mental Component Summaries (SF‐36 PCS and SF‐36 MCS), Birmingham Vasculitis activity score (BVAS), Five‐Factor Score (FFS), and Vasculitis Damage Index (VDI) were assessed as AAV‐specific indices. Whole blood was collected and serum samples were isolated and stored at −80°C. Serum progranulin concentration was quantified by ELISA kits.ResultsThe median age of patients was 63.0 years (19 men). The median BVAS was 11.0, and the median serum progranulin level was 49.0 ng/ml. Serum progranulin was significantly correlated with BVAS, FFS, erythrocyte sedimentation rate, C‐reactive protein level, SF‐36 PCS, haemoglobin, and serum albumin. Severe AAV was arbitrarily defined as the highest tertile of BVAS (BVAS ≥16). When the cut‐offs of serum progranulin were set as 55.16 ng/ml and 43.01 ng/ml for severe AAV, AAV patients with serum progranulin ≥55.16 and 43.01 ng/ml had significantly higher risks of severe AAV than those without (relative risk (RR) 4.167 and 4.524, respectively).ConclusionsProgranulin might play an anti‐inflammatory role in AAV pathogenesis and serum progranulin could be used as a predictive marker for high activity of AAV.     

抗中性粒细胞胞质抗体相关性血管炎心脏受累影像学所见

抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)为全身坏死性小血管炎,可累及全身多个器官,肾脏和肺最常受累,心脏受累则相对少见,但多与预后不良相关.本文对AAV累及心脏的机制及其常见心电图、超声心动图及心脏M RI表现进行综述.     

抗中性粒细胞胞浆抗体检测在肺纤维化疾病中应用研究

目的 探讨肺纤维化疾病患者的抗中性粒细胞胞浆抗体（ANCA）与血清胱抑素C（CysC）和高敏C反应蛋白等指标之间的变化规律.方法 检测69例肺间质纤维化伴感染患者和30例健康对照组的ANCA及血尿素氮、肌酐、CysC和高敏C反应蛋白等指标.ANCA检测先应用间接免疫荧光法,并用免疫印迹法确定靶抗原.生化指标用全自动生化分析仪测定.白细胞和血小板用血细胞分析仪测定.结果 肺纤ANCA（＋）组CysC水平明显高于肺纤ANCA（-）组与健康对照组（P〈0.05）,而肺纤ANCA（-）组与健康对照组的CysC比较差异无统计学意义（P〉0.05）;肺纤ANCA（＋）组与肺纤ANCA（-）组CRP水平明显高于与健康对照组（P〈0.01）,其他指标各组比较差异无统计学意义（P〉0.05）.结论 肺纤ANCA（＋）可能伴有肾功能损伤,应注重肺纤ANCA（＋）患者的肾功能监测.     

Fibrinogen to albumin ratio reflects the activity of antineutrophil cytoplasmic antibody‐associated vasculitis

BackgroundWe investigated whether fibrinogen to albumin ratio (FAR) at diagnosis could reflect the cross‐sectional activity and predict poor outcomes in patients with antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis (AAV).MethodsThis cross‐sectional study included 54 immunosuppressant drug‐naïve patients with AAV who had the results of plasma fibrinogen and serum albumin at diagnosis. Clinical and laboratory data at diagnosis were collected, and all‐cause mortality, cerebrovascular accident, cardiovascular disease, end‐stage renal disease occurrences were assessed as poor outcomes. FAR was calculated by the following equation: FAR = plasma fibrinogen (g/dl)/serum albumin (g/dl).ResultsThe median age was 65.5 years, and 59.3% of patients were men (33 MPA, 13 GPA and 8 EGPA). FAR was significantly correlated with Birmingham vasculitis activity score (BVAS; r = 0.271), erythrocyte sedimentation rate (ESR; r = 0.668) and C‐reactive protein (CRP; r = 0.638). High BVAS was defined as BVAS ≥16, and the cut‐off of FAR at diagnosis was set as 0.118. AAV patients with FAR at diagnosis ≥0.118 had a significantly higher risk for the cross‐sectional high BVAS than those without (RR 3.361). In the univariable linear regression analysis, CRP (β = 0.383) and FAR (β = 0.297) were significantly correlated with BVAS at diagnosis. However, in the multivariable analysis, none of them was correlated with the cross‐sectional BVAS. FAR at diagnosis could not predict poor outcomes during follow‐up in AAV patients.ConclusionsFibrinogen to albumin ratio at diagnosis could reflect the cross‐sectional BVAS but could not predict poor outcomes in patients with AAV.     

慢性阻塞性肺疾病合并肺间质纤维化的临床分析

目的 探讨慢性阻塞性肺疾病(COPD)合并肺间质纤维化(PF)的临床特点以及两者之间的关系.方法 对比分析我院2001年7月至2010年10月确诊的PF-COPD患者27例(PF-COPD)组及COPD患者30例(COPD组)的症状、体征、肺功能、动脉血气分析及胸部X线片和CT及高分辨CT(HRCT)结果.结果 PF-COPD组患者临床表现介于此两种疾病之间.肺功能检查示:PF-COPD组以混合性通气功能障碍为主(18与0;x2=17.10,P＜0.01),而COPD组30例均为阻塞性通气功能障碍(30与9;x2 =15.72,P＜0.01).血气分析示:PF-COPD组低氧血症较为明显,COPD组PaCO2升高较多见,但两组比较差异无统计学意义(P＞0.05).胸部X线显示,COPD组30例胸廓前后径均增大,PF-COPD组则为14例(P＜0.05),而PF-COPD组点状、网状或蜂窝状改变24例,COPD组则无此改变(P＜0.01);胸部CT及HRCT示:PF-COPD组肺部磨玻璃状改变、网格结节影均较COPD组高(9与0、19与0;x2=10.85、17.36,P均＜0.01).结论 PF-COPD具有独特的临床特点,兼有二者的特征.胸部HRCT能为临床提供重要诊断依据.     

Lung adenocarcinoma and sequential antineutrophil cytoplasmic antibody-associated vasculitis: a case report

The relationship between antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and lung cancer remains unclear. A 66-year-old man presented with pulmonary nodules. Histological examination of a specimen from computed tomography-guided percutaneous transthoracic biopsy revealed adenocarcinoma. The patient was treated using cryoablation and systemic chemotherapy. Sixteen months later, the patient presented with fever, nasal inflammation, recurrent lung lesions, elevated serum creatinine levels, and high levels of ANCA. Histological examination of a specimen from ultrasound-guided percutaneous renal biopsy revealed pauci-immune necrotizing crescentic glomerulonephritis. The patient responded to treatment, but granulomatosis with polyangiitis recurred and he later died. This case highlights the possibility of sequential AAV with lung cancer. Although this is relatively rare, further research is needed to better understand the association or pathophysiological link between lung cancer and AAV.     

54例原发ANCA相关性小血管炎的肾脏病理及预后的分析

目的探讨原发小血管炎肾脏病理特点及与肾功能预后的关系。方法 54例确诊并行肾活检的原发AN-CA相关小血管炎患者,分别按年龄及性别分组,对比统计各组病理类型和病理特点并随访。结果①肾脏病理类型检出率最高的是硬化性和轻度系膜增生性肾炎各占22.2%,其次是局灶节段硬化性肾炎（18.5%）;60岁以上组轻度系膜增生的患者比例偏高,P=0.05;②肾脏病理急慢性损害检出率较高为：中重度间质炎细胞浸润（48.1%）,中重度间质小管损害（46.3%）,纤维新月体（38.9%）,中重度增生硬化（35.2%）,细胞性新月体（24.1%）,袢坏死检出率（14.8%）;各年龄段相比,中重度增生硬化在30岁以下检出率相对较高,差异有统计学意义,P〈0.05;③随访1年,9例进入尿毒症期（16.7%）。发生尿毒症的相关风险是肾脏慢性指数,P〈0.05。结论肾小球硬化及严重的肾脏病理损害发生率高尤以年轻患者多见;发生终末期肾的相关风险因素是肾脏慢性指数。     

老年ANCA相关性小血管炎肺损害11例临床分析

目的探讨老年抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎肺脏损害的临床特征,提高对其肺损害的认识。方法回顾性分析北京大学第一医院老年科60岁以上的ANCA相关性小血管炎肺损害患者的临床病理资料。结果11例ANCA相关性小血管炎肺脏损害患者平均年龄75.9(60～89)岁,均有慢性基础病。发病至确诊的中位间隔时间为4个月(2周～1年)。小血管炎临床症状多为咳嗽、咯痰(90.9%);喘憋气短或活动耐力下降(63.6%);咯血/痰中带血(36.4%)和发热(45.5%)。胸部影像学9例(91.9%)表现为肺间质病变,其中8例呈显著肺纤维化表现;2例(18.2%)呈两肺大片渗出的弥漫肺泡出血表现(其中1例在肺纤维化基础上发生);1例呈结节性病变(9.1%)。8例(72.7%)患者初诊时无肾损害。确诊主要依靠血清ANCA的检测和组织活检病理诊断。经免疫抑制治疗后,多数患者症状好转,初诊及随访期间5例(45.5%)因呼吸衰竭或严重感染死亡。结论原发性ANCA相关性小血管炎患者常有肺部受累并可以为首发表现。其症状和胸部影像表现多样,肺间质病变突出。正确及时的诊断治疗能改善预后。