肺脏受累的原发性抗中性粒细胞胞浆抗体相关性小血管炎43例临床分析

目的 探讨肺脏受累的原发性抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎患者的临床特征,为肺部受累的原发性ANCA相关性小血管炎的诊断提供帮助.方法 回顾性分析2009年3月至2013年9月在湘雅二医院住院的符合2012年美国Chapel Hill会议关于系统性小血管炎诊断标准,并血清ANCA阳性的43例肺脏受累的的原发性ANCA相关性小血管炎患者的临床资料.结果 43例患者中,肺部症状首发就诊者22例,常见肺部症状依次为咳嗽咯痰、活动后气促、咯血、哮喘等,肺外受累器官依次为肾脏、神经系统、眼、鼻;肺外症状首发就诊者21例,肾脏为最常见受累器官;显微镜下多血管炎（MPA） 34例,其中抗髓过氧化物酶（MPO）抗体（P-ANCA）阳性率97.1％（33/34）,抗蛋白酶3（PR3）抗体（C-ANCA）阳性率2.9％ （1/34）;肉芽肿性多血管炎（GPA）（韦格纳肉芽肿）8例,抗蛋白酶3（PR3）抗体（C-ANCA）阳性率62.5％（5/8）,抗髓过氧化物酶（MPO）抗体（P-ANCA）阳性率为37.5％（3/8）;嗜酸细胞性肉芽肿性多血管炎（EGPA）1例,为抗髓过氧化物酶（MPO）抗体阳性;胸部影像学表现多为双肺间质病变,如双肺网格样改变、磨玻璃影、蜂窝肺、多发条索状及结节性病变等,或为条索或斑片状病变、支气管扩张、胸腔积液及肿块病变等.经激素和免疫抑制剂治疗多数患者病情可缓解,19例患者（44.2％）于住院及随访期间因血管炎活动并肺部感染、大咯血及肾功能衰竭等原因死亡.结论 肺脏受累的原发性ANCA相关性小血管炎临床表现无特异性,多数患者合并有肾脏受累,影像学多为肺间质病变,具有提示诊断价值,血清ANCA检查有特殊诊断价值,此类患者死亡率高,多为疾病活动并感染致死,应积极控制感染并合理采用抑制免疫治疗.     

误诊为肺间质纤维化的显微镜下多血管炎1例并文献复习

抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎常常表现为多系统损害,以肾脏和肺的受累最为常见,亦常有不规则发热、疲乏、皮疹、关节痛、肌痛、腹痛、神经炎和体质量下降等非特异性症状.由于临床表现复杂,常易误诊、漏诊.我们近期在病房诊断1例首发首诊为特发性肺间质纤维化的显微镜下多血管炎(MPA).     

肺部原发性小血管炎的临床表现和诊断

目的 研究肺脏受累的原发性小血管炎的临床特点和早期诊断要点，减少其误诊率。方法 对26例原发性小血管炎伴肺脏受累患者的临床资料进行回顾性分析。结果 26例中，显微镜下多血管炎(MPA)15例，韦格纳肉芽肿(WG)9例，变应性肉芽肿性血管炎(CCS)2例。抗中性粒细胞胞浆抗体(ANCA)阳性率61．5％。肺脏受累的临床表现有咯血、呼吸困难、干咳、胸痛、胸闷等，胸部X线多表现为多发斑片状或点状阴影、大片高密度阴影或团块影、结节影、网格状影，可有空洞形成。疾病早期多误诊为肺部感染、肿瘤、肺间质病变等。应用肾上腺皮质激素和环磷酰胺治疗肺部病变可明显吸收，临床危重症得以改善。结论 伴肺脏受累的原发性小血管炎临床表现复杂多样，诊断困难，误诊率高，易延误病情，须引起临床医师的重视。     

25例肺部受累的原发性小血管炎的HRCT表现及临床分析

目的研究肺部受累的原发性小血管炎的HRCT表现等临床特点。方法对本院2004年1月~2010年1月收治的肺部受累的19例显微镜下多血管炎（MPA）患者及6例变应性肉芽肿性血管炎（CSS）患者的临床资料进行回顾性分析。结果 19例MPA的肺部HRCT表现有：（1）两肺弥漫浸润性阴影、类似肺间质性改变12例;（2）肺内多发斑片影7例。6例CSS的肺部HRCT均表现为多发斑片影。19例MPA中抗中性粒细胞胞浆抗体（ANCA）阳性18例,阳性率高达94.74%。6例CSS中ANCA阳性1例。结论肺部受累的原发性小血管炎HRCT影像学表现无明显特异性,结合临床及实验室检查,可以提高认识,早期诊治。     

显微镜下多血管炎肺部病变的临床特点

目的 探讨显微镜下多血管炎(microscopic polyangiitis,MPA)肺部病变特点.方法 回顾性分析2008年1月至2009年12月在北京协和医院伟院的50例MPA患者的临床资料,包括临床表现、实验室检查、超声心动图、肺功能、胸部CT及肾脏病理等.结果 男22例,女28例,年龄23～85岁,平均(66±12)岁.病程中存在肺部病变者46例(92%),常见症状有咳嗽(34/46,74%)、咳痰(30/46,65%)、呼吸困难(19/46,41%)和咯血(16/46,35%);14例(14/50,28%)以肺部病变为首发表现,其中5例影像学特点符合普通型问质性肺炎.抗中性粒细胞胞浆抗体(ANCA)中髓过氧化物酶(MPO)-ANCA阳性率96%(48/50),蛋白酶3(PR3)-ANCA阳性率6%(3/50).影像学表现为磨玻璃影者16例,肺间质纤维化16例,浸润影12例,胸腔积液7例.常见肺功能异常为弥散功能障碍(12/15)和限制性通气功能障碍(4/15).肺动脉高压13例,肺动脉收缩压平均为(48±8)mmHg(1 mm Hg=0.133 kPa).结论 MPA肺受累发生率高,约1/3患者以肺受累为首发表现;临床表现无特异性,磨玻璃影、肺间质纤维化、浸润影和胸腔积液为常见影像学表现;激素和免疫抑制剂治疗后短期预后较好,肺部感染是MPA肺受累的主要死亡原因.

Abstract：

Objective To explore the clinical features of pulmonary involvement in patients with microscopic polyangiitis(MPA).Methods We retrospectively investigated the clinical data of 50 patients hospitalized with MPA in Peking Union Medical College Hospital from January 2008 to December 2009,the data included clinical manifestation,laboratory parameters,echocardiography,pulmonary funetion test,chest computed tomography,and histopathology of kidney.Results Pulmonary involvements were observed in 46 patients,common symptoms include cough(34/46),expectoration(30/46),dyspnea(19/46)and hemoptysis(16/46).Pulmonary involvement was the initial manifestation in 14 patients,five cases had radiographic evidences of usaal interstitial pneumonia before MPA was diagnosed.The prevalence of positive MPO-ANCA antibodies in MPA patients was 96%.The prevalence of positive PR3-ANCA antibodies was 6% Radiographic manifestations included ground glass attenuation(16/37),interstitial changes(16/37),infihrates(12/37)and pleural effusion(7/37).The most frequent abnormality in pulmonary function test was reduced carbon monoxide diffusing capacity(12/15)and restrictive ventilation dysfunction(4/15).The incidences of pulmonary hypertension was 33%(13/39),the average pulmonary artery systolic pressure was (48±8)mm Hg(1 mm Hg=0.133 kPa).Conclusion The prevalence of pulmonary involvement in patients with MPA was high,pulmonary involvement was the initial manifestation in 28%patients.The clinical manifestations were nonspecific,radiographic manifestations included ground glass attenuation,interstitial changes,infiltrates and pleural effusion.The short term prognosis was well in patients with pulmonary involvement treated with systemic cortieosteroids and cyclophosphamide,infection was a leading cause of death in patients with pulmonary involvement.     

坏死性肉芽肿性血管炎20例临床分析

目的分析坏死性肉芽肿性血管炎(NGV)的临床资料。方法对南京鼓楼医院收治的20例NGV患者的临床资料进行分析。结果男11例,女9例,发病年龄18~76岁,平均年龄44.7岁。可累及多个系统或器官,以肺脏受累最常见,占100%,其次为鼻部和肾脏,各为85%。血抗中性粒细胞胞质抗体(ANCA)阳性率95%。影像学以肺部多发结节/肿块影伴或不伴空洞病灶(65%)为主。最常见的病理表现为坏死性肉芽肿和小血管炎。结论 NGV临床表现复杂多样,综合ANCA、影像学和组织活检利于早期诊断,早期治疗。     

老年人抗中性粒细胞胞浆抗体相关性小血管炎的临床表现

目的　为了解老年人抗中性粒细胞胞浆抗体 (ANCA)相关性小血管炎所引起的多器官损害的临床表现。方法　分析总结近 5年来检出的 1 4 5例 60岁以上老年ANCA相关性小血管炎患者的临床病理资料。结果　1 4 5例患者 ,男 74例 ,女 71例 ,平均年龄 68.2岁。胞浆型ANCA(c ANCA)阳性 1 7例 ,均识别蛋白酶 3(PR3) ;环核型ANCA (p ANCA)阳性 1 2 8例 ,其中 1 2 0 1 2 8例单独识别髓过氧化物酶 (MPO) ,8 1 2 8例同时识别PR3和MPO。不足 1 2的患者在 3个月内确诊。确诊病例均呈多器官损害 ,其中肾脏受累占 93 .8% ,且 75 %出现肾功能不全 ;肺脏受累占 74.5 % ,半数以上表现为肺出血和 (或 )肺部阴影 ,此外还有不同程度的消化道、神经系统、肌肉关节、眼、耳器官或组织损害及发热、乏力、消瘦等非特异症状。实验室检查 ,90 %以上的患者有中重度贫血、血沉增快和C反应蛋白升高。结论　老年人ANCA阳性小血管炎较为多见 ,临床表现为多器官损害 ,ANCA检测有助于诊断     

ANCA相关性血管炎肺脏受累的临床特点及危险因素分析

目的探讨ANCA相关性血管炎(AASV)肺脏受累的临床特点并分析其危险因素。方法回顾性分析安医大一附院2010年1月至2015年1月住院确诊的201例ANCA相关性血管炎临床资料,其中肺脏受累组93例和非肺脏受累组(对照组)108例,比较两组临床表现及辅助检查资料,运用Logistic回归模型进行危险因素分析。结果 201例患者中,男性75例,女性126例,平均年龄(62.64±14.89)岁,死亡2例,自动出院5例。两组在性别、纳差、乏力、体重下降、关节痛、肌痛差异无统计学意义;在白细胞计数、中性粒细胞比例、血红蛋白水平、血清白蛋白水平、CRP、ANCA滴度差异有统计学意义(P0.05)。Logistic多因素分析结果显示:低蛋白血症(OR=0.593,95%CI 0.385~0.914,P0.05)和C反应蛋白升高(OR=1.302,95%CI 1.087~1.559,P0.05)的患者更易合并肺脏受累。肺脏受累组影像学表现半数以上为间质性改变及斑片状、条索影。结论 AASV患者肺脏受累临床表现缺乏特异性,肺部影像学以间质性改变及斑片状、条索影为主,低白蛋白血症和C反应蛋白升高合并肺脏受累的风险高,临床上应予以重视。     

ANCA相关性血管炎肺部表现

目的 研究抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AASV)在肺部疾病的临床表现及特点.方法 回顾性分析2007年1月至2011年4月于吉林大学第二医院确诊为AASV的30例住院患者肺部病变的临床资料.结果 30例AASV患者发病年龄24～77岁,其中女18例,男12例.30例AASV患者中Wegener肉芽肿(WG)8例,显微镜下多血管炎(MPA) 22例,无变应性肉芽肿血管炎(CSS).结论 所有年龄段均有可能患AASV,MPA的发病率大约是WG的3倍,MPA患病率较多,可能是我国AASV的特点之一.WG患者较MPA患者更易出现呼吸系统症状,MPA患者较WG患者更易累及肾脏,出现肾功改变.影像学表现无特异性改变,但常重于临床表现.AASV肺部病理可表现为特发型肺间质纤维化.随着检验手段的提高,AASV的发病率逐渐增高,其临床表现多变,病变部位广泛,肾脏和肺脏是AASV最易受累的器官.对于长期发热及多器官受损的患者应尽早进行ANCA检查,及时治疗,改善预后.     

以肺部病变为主要表现的ANCA相关性血管炎10例临床分析

目的从呼吸系统疾病的角度分析和认识ANCA相关性血管炎(ANCA-associated Vasculitis,AAV)。方法回顾性分析我院确诊的10例以呼吸系统病变为主要表现的AAV患者的临床资料,对患者的临床表现、实验室检查、影像学表现以及其他特殊检查结果进行汇总分析,采用五因子评分系统评估预后。结果本组患者显微镜下多血管炎(microscopic polyangiitis,MPA)7例,肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)2例,嗜酸性肉芽肿性血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)1例,男性3例,女性7例。咳嗽咳痰咯血及呼吸困难是AAV最常见的呼吸系统症状,部分患者合并其他系统特别是肾脏累及。实验室检查MPA均有p ANCA和MPO-ANCA阳性,GPA和EGPA患者有c ANCA和PR3-ANCA阳性,7例患者有CRP升高(82.18±48.67mg/L),10例患者均有血沉升高(62.9±13.5mm/h);仅有4例患者出现肌酐轻度升高(139±29.3umol/L),3例患者淋巴细胞总数降低,提示继发感染风险大,预后欠佳。影像学表现多表现为双肺磨玻璃斑片影、间质改变以及蜂窝肺表现,对诊断具有提示作用。肺功能检查表现为限制性通气功能障碍,部分患者出现肺动脉高压。结论肺部是AAV的常见累及器官,部分患者以肺部病变为主要表现,呼吸科医生应加强对AAV的认识,提高诊断率,减少误诊和漏诊。     

28例ANCA阳性肺血管炎临床分析

目的总结我科28例ANCA阳性肺血管炎的临床表现,提高对该病认识。方法回顾性分析我科2011-2015年ANCA阳性肺血管炎患者,总结其临床资料、实验室和肺部CT检查表现。结果 28例患者纳入分析,性别比例1∶1.15;平均年龄64.11±13.51岁(36~81岁);大部分患者表现为发热、咳嗽、咳痰;25例患者表现为P-ANCA阳性(89.28%);所有检测ESR的患者均有不同程度升高(21/21),96.30%的患者CRP升高(26/27);17例患者合并有肾受累表现,主要以血尿为主(17/28,60.71%);74.19%的患者有不同程度的贫血,78.57%有中性粒细胞比例升高。肺部CT提示以斑片状渗出影最常见(67.86%),其次为间质病变(28.57%),其他病变包括结节样病变(17.86%),胸腔积液(25.00%),磨玻璃样病变(7.14%)。与单纯肺受累组相比,合并肾受累时实验室检查和肺部影像学改变并无明显差异。结论 ANCA阳性肺血管炎临床和相关检测缺少特异性,诊断上需仔细甄别。     

咳嗽及肺部游走性阴影为主要表现的抗中性粒细胞胞浆抗体相关性血管炎1例

笔者成功诊治1例65岁男性抗中性粒细胞胞浆抗体（ANCA）相关性血管炎患者。该患者主要表现为慢性咳嗽和肺部游走性阴影,曾被诊断为淋巴细胞性间质性肺炎,激素治疗有效,病情复发后查MPO—ANCA阳性,结合肺穿刺活检明确诊断为ANCA相关性血管炎,加用糖皮质激素和环磷酰胺后病情再次缓解。根据文献报道和笔者的临床经验,ANCA相关性血管炎临床表现复杂,缺乏特异性,常被误诊。呼吸科医师对ANCA相关性血管炎肺部受累特点应熟悉,要重视血清ANCA的筛查,以便于本病的早期诊断。     

Vasculitis associated with anti-neutrophil cytoplasmic autoantibodies in rheumatoid arthritis. Report of a case of microscopic polyangiitis and another case of Wegener's granulomatosis

Vasculitis is an uncommon complication of rheumatoid arthritis that is associated with a clear increase in morbidity and mortality, although systemic manifestations such as glomerulonephritis, cerebral vasculitis or pulmonary vasculitis are very rare. Systemic vasculitis with renal involvement is associated with overt polyarthritis in less than 5% and association with rheumatoid arthritis is exceptional. Determination of anti-neutrophil cytoplasmic autoantibodies (ANCA), used in the appropriate clinical context, has become an important diagnostic tool in small-vessel systemic vasculitides. We present two patients with rheumatoid arthritis who subsequently developed systemic vasculitis. ANCA determination was decisive in the early diagnosis of these patients.     

Antineutrophil cytoplasmic autoantibody-associated vasculitis in older patients

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is increasingly recognized in older patients. The differences in disease presentation and outcome between older and younger patients remain controversial. We conducted the current study to analyze the characteristics of patients aged over 65 years with AAV and to compare the younger and older cohorts. We recruited 234 consecutive Chinese patients with AAV. We compared clinical and pathologic characteristics as well as outcomes between younger and older patients. Among the 234 patients with AAV, 99 were older than 65 years. Compared with the 135 younger patients, the older patients had a significantly higher proportion of positive myeloperoxidase-ANCA (94.9% vs. 80.0%, p < 0.01) and a higher proportion of microscopic polyangiitis (79.8% vs. 50.4%, chi = 11.8, p < 0.001), but had a lower proportion of Wegener granulomatosis (18.2% vs. 37.8%, p < 0.01) and renal-limited vasculitis (0% vs. 11.1%, p < 0.001).Older patients had more prevalent and severe pulmonary involvement than younger patients, including pulmonary infiltration, interstitial fibrosis, and mechanical ventilation dependence at presentation (47.5% vs. 31.9%, p < 0.05; 37.4% vs. 18.5%, p < 0.01; and 9.1% vs. 1.5%, p < 0.05, respectively). Older patients were less likely to respond to treatment (p < 0.01) and had worse survival than younger patients (p = 0.000). During follow-up, older patients had a higher risk of secondary pulmonary infection (p < 0.001), and those with pulmonary interstitial fibrosis were more likely to develop secondary pulmonary infections (p < 0.05). In conclusion, compared with younger patients, older patients with AAV had more severe and more prevalent pulmonary lesions, which might contribute to subsequent pulmonary infections after the initiation of immunosuppressive therapy. Age and pulmonary infection were independent predictors of death.     

426例抗中性粒细胞胞浆抗体相关性小血管炎患者多系统临床表现和肾脏病理分析

目的 分析426例抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎患者多系统的临床和病理表现。方法 回顾性分析我院1997年-2004年6月检测并明确诊断的426例ANCA相关性小血管炎患者的临床病理资料。结果 426例患者中,70例胞浆型ANCA（cANCA）阳性,均识别蛋白酶3（PR3）;354例环核型ANCA（pANCA）阳性,均识别髓过氧化物酶（MPO）。201例（47．2％,201／426）患者是在发病后3个月内确诊。临床表现呈多器官受累,其中cANCA阳性者皮疹、关节痛、眼、鼻受累的发生率显著高于pANCA阳性者,而pANCA阳性者。肾脏受累和乏力的发生率显著高于cANCA阳性者。多数患者有贫血,血沉增快,C反应蛋白增高。采用糖皮质激素联合环磷酰胺进行强化免疫抑制治疗,诱导缓解期的缓解率为88．5％。结论 ANCA相关性小血管炎在我国并非少见,临床表现呈多器官受累,ANCA检测有助于早期诊断。     

ANCA-associated lung fibrosis: analysis of 17 patients

In this retrospective study, we analyzed 17 patients presenting with pulmonary fibrosis and a positive ANCA testing. This group was compared with a control group of 12 patients with IPF and negative ANCA testing. Patients were 15 males and 2 females, with a mean age of 66 years. Eight patients were past smokers, 3 current smokers and 6 non-smokers. Lung function tests at diagnosis were as follows (% predicted): total lung capacity 73%+/-18, vital capacity 82%+/-23, forced expiratory volume in 1s (FEV(1)) 88%+/-24, carbon monoxide diffusion capacity of the lung 49%+/-2 (% predicted). Bronchoalveolar lavage results showed an increased cellularity with increased neutrophils counts. High resolution computed tomography of the chest showed prominent fibrosis with some degree of ground-glass attenuation in all patients. These characteristics were similar to the control group. Microscopic polyangiitis (MPA) was a major complicating event in ANCA-positive patients, occurring in 7 patients (anti-myeloperoxidase specificity in 5 patients). Pulmonary fibrosis predated occurrence of MPA in 6 patients and was diagnosed concomitantly with MPA in 1 patient. During the follow-up, 10/17 patients died. The death was directly related to vasculitis in 3 patients. We conclude that patients with pulmonary fibrosis should be evaluated for the presence of ANCA. Patients with positive ANCA testing, particularly if anti-myeloperoxidase, should be carefully monitored to detect the occurrence of microscopic polyangiitis.     

以肺部表现首发的抗中性粒细胞胞浆抗体相关性小血管炎临床分析

目的探讨以肺部表现首发的抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎的临床特征。方法回顾分析10例以肺部症状首发的ANCA相关性小血管炎患者的临床资料。结果10例患者均有肺病变,肾脏损害5例,皮肤损害1例。所有患者ANCA阳性。双肺病变8例,单侧肺部病变2例。大部分患者可见血白细胞升高、贫血、血沉明显增快、C反应蛋白升高等。应用糖皮质激素及免疫抑制剂治疗,死亡3例,完全缓解7例。结论ANCA相关性小血管炎临床表现缺乏特异性,ANCA检查有助于该病的早期诊断。糖皮质激素及免疫抑制剂治疗能改善预后。