抗中性粒细胞胞浆抗体相关性小血管炎肺脏受累12例临床分析  

Clinical analysis of antineutrophil cytoplasmic-antibodies-associated small vessel vasculitis with lung involvement:report of cases and review of literature

作　　者：段敏超[1,2] 钟小宁[1] 邓静敏[1] 何志义[1] 张建全[1] 

机构地区：[1]广西医科大学第一附属医院呼吸内科,南宁530021 [2]南宁市第一人民医院呼吸内科

出　　处：《中国实用内科杂志》2009年第1期56-59,共4页Chinese Journal of Practical Internal Medicine

摘　　要：目的提高对抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎肺脏受累的认识。方法对2004年11月至2007年12月广西医科大学第一附属医院住院的12例ANCA相关性小血管炎肺脏受累病例的临床特点进行回顾性分析。结果(1)本组患者以中老年多见,年龄平均(59.17±16.85)岁。(2)主要临床表现为:①呼吸道症状为咳嗽(91.7%)、呼吸困难(50.0%)、咯血(16.7%);②常伴发热(83.3%)和体重下降(41.7%)等全身非特异性表现,肺外表现有贫血(100%)、关节肌肉痛(50.0%)及皮疹(50.0%)等;③泌尿系统症状:血尿(83.3%)、蛋白尿(75.0%);④所有患者均有3个或3个以上器官受累。(3)实验室检查:红细胞沉降率增快(100%),C-反应蛋白增高(83.3%);血肌酐及尿素氮均明显增高(83.3%),ANCA阳性。(4)肺部影像学表现:①肺部多发性病变,可单侧、双侧,以双侧(66.7%)受累多见;②病变多样:主要表现为不规则散在斑片状、斑点条索状致密影、多发结节影及胸腔积液;③经糖皮质激素联合免疫抑制剂治疗病灶明显吸收或消失。(5)12例行经皮肾穿刺活检,病理上主要为少或无免疫复合物沉积的局灶坏死性肾小球肾炎或新月体性肾小球肾炎。(6)12例患者首诊全部误诊,最常误诊疾病为肺炎(50.0%)、肺结核(25.0%);确诊后经糖皮质激素联合免疫抑制剂治疗有效;3例患者肺部病变复发,1例患者死于呼吸衰竭;复发及死亡病例与病理类型有关。结论该病临床上以发热和呼吸系统、泌尿系统及其他多器官受累为特点,影像学表现形式多样,临床误诊率高,诊断需结合ANCA检测及病理检查,糖皮质激素联合免疫抑制剂治疗有效,预后与病理类型有关。Objective To investigate the clinical characteristics of ANCA-associated small vessel vasculitis with lung involvement. Methods Twelve cases of ANCA-associated small vessel vasculitis with lung involvement, admitted to our hospital from November 2004 to December 2007,were retrospectively studied. Results （ 1 ） The mean age of patients was （59. 17 ± 16.85）years. （2）The clinical manifestations： （1）The main symptoms of respiratory system were cough （91.7%） ,dyspnea（ 50. 0% ）and hymoptysis （16. 7% ）. （2）The common accompanying manifestations included systemic symptoms like fever （ 83.3% ） and weight loss （ 41.7% ） as well as extra-pulmonary symptoms, including anaemia （ 100% ）, arthralgia（ 50. 0% ） and skin rashes （ 50.0% ）. （3）The symptoms of renal system are haematuria （ 83.3% ） and proteinuria（ 75. 0% ）. （4）At least three organ involvements were shown in each case. （3）Elevated ESR, CRP, Cr and BUN, and ANCA were revealed in all cases. （4）①The chest radiograph demonstrated that multiple lung fields either unilateral or bilateral could be involved and bilateral involvement（66.7% ）was more common. ②The types of lesions included patchy consolidations, multiple nodules and pleural effusion. ③The treatments with corticosteroid and CTX were effective in these cases, wherein pulmonary lesions were ameliovated obviously. （5）Percutaneous renal biopsy was performed in 12 patients,which typically showed focal segmental recrotizny glomerulonephritis or a cresentic glomerulonephritis, with few or no immune complex deposition. （6）All 12 patients were all misdiagnosed as pneumonia （50. 0% ）or pulmonary tuberculosis （25.0%）at the first diagnosis. Corticosteroid and immune suppressors treatment appeared to be effective. Three out of twelve had relapses of lung involvement ; one patient died of respiratory failure. Assumably, the relapses and death of the patients were related to different pathological changes on renallesion. Con

关 键 词：血管炎 小血管炎 肺脏 抗中性粒细胞胞浆抗体 

分 类 号：R5[医药卫生—内科学]