CAJ | 학술논문

目的分析韦格纳肉芽肿病(Wegener's granulomatosis,WG)患者的临床诊疗资料,提高对本病的认识.方法回顾性研究分析2002年1月至2012年3月在北京协和医院确诊的34例WG患者的临床表现、实验室检查、影像特点、病理特点、治疗及疗效.结果 34例患者中,男18例,女16例,发病年龄(45±15)岁(18 ～81岁)；确诊时间是(140±72)个月,3个月以内确诊率29.4％；首发临床表现多为肺(41.1％)、鼻(38.2％)、眼(11.7％)、头痛(11.7％)、耳(8.8％),非特异症状如发热(26.4％)等亦不少见；全部临床表现多为肺部(76.4％)、鼻(73.5％)、肾脏(67.6％)、眼(58.8％)、耳(47.0％)；神经系统及口腔症状亦不少见,分别是41.1％、20.5％.实验室检查C-ANCA及PR3阳性率61.7％,P-ANCA及MPO阳性率20.5％,ANCA阴性率11.7％,P-ANCA阳性及MPO阴性1例(2.9％),C-ANCA阳性并PR3及MPO均阳性1例(2.9％).肺部影像表现复杂多样,多为结节或肿块(34％)、索条影(20.5％)、空洞(17.6％)、斑片影(17.6％)；典型病理三联征表现1例(4.3％),出现典型病理中二联表现10例(43.4％)；在入院前误诊为肺结核、肺癌、肺脓肿,误诊率为20.5％；肺部感染发生率高达50％；入院后给予激素和环磷酰胺正规诱导缓解及维持治疗,难治型给予血浆置换、静脉输注丙种球蛋白(IVIG)和抗CD20单克隆抗体利妥昔单抗,治疗疗效好.结论 WG的临床表现复杂多样且变化迅速,容易误诊误治；早期积极运用糖皮质激素和环磷酰胺疗效好.
목적 분석위격납육아종병(Wegener's granulomatosis,WG)환자적림상진료자료,제고대본병적인식.방법 회고성연구분석2002년1월지2012년3월재북경협화의원학진적34례WG환자적림상표현、실험실검사、영상특점、병리특점、치료급료효.결과 34례환자중,남18례,녀16례,발병년령(45±15)세(18 ～81세)；학진시간시(140±72)개월,3개월이내학진솔29.4％；수발림상표현다위폐(41.1％)、비(38.2％)、안(11.7％)、두통(11.7％)、이(8.8％),비특이증상여발열(26.4％)등역불소견；전부림상표현다위폐부(76.4％)、비(73.5％)、신장(67.6％)、안(58.8％)、이(47.0％)；신경계통급구강증상역불소견,분별시41.1％、20.5％.실험실검사C-ANCA급PR3양성솔61.7％,P-ANCA급MPO양성솔20.5％,ANCA음성솔11.7％,P-ANCA양성급MPO음성1례(2.9％),C-ANCA양성병PR3급MPO균양성1례(2.9％).폐부영상표현복잡다양,다위결절혹종괴(34％)、색조영(20.5％)、공동(17.6％)、반편영(17.6％)；전형병리삼련정표현1례(4.3％),출현전형병리중이련표현10례(43.4％)；재입원전오진위폐결핵、폐암、폐농종,오진솔위20.5％；폐부감염발생솔고체50％；입원후급여격소화배린선알정규유도완해급유지치료,난치형급여혈장치환、정맥수주병충구단백(IVIG)화항CD20단극륭항체리타석단항,치료료효호.결론 WG적림상표현복잡다양차변화신속,용역오진오치；조기적겁운용당피질격소화배린선알료효호.
Objective To analyze the clinical data of diagnosis and treatment of Wegener' s granulomatosis (WG) in order to understand the nature of this disease. Methods The clinical data including clinical manifestations,laboratory findings imaging features,pathological changes and efficacy of treatment of 34 patients with WG admitted from January 2002 to March 2012 were analyzed.Results Of the 34 patients,male to female ratio was 18 to 16,and the average age subjected to WG onset was (45 ± 15)years old ranged from 18 to 81 years old.The average duration before the diagnosis confirmed was ( 140 ±72) mouths,while 29.4％ was diagnosed within 3 months. The presenting symptoms included initial involvements in lung (41.1％),nose (38.2％ ),eyes ( 11.7％ ),ear (8.8％ ) and constitutional symptoms such as fever (26.4％ ). Throughout the whole disease course,the incidence of systemic impairments were as follows:lung (76.4％),nose (73.5％),kidney (67.6),eyes (58.5％),ear (47.0％),nervous system (41.1％),oral ulcer (20.5％ ).Of laboratory findings,C - ANCA/PR3 -ANCA was positive in 61.7％ patients,P - ANCA/MPO - ANCA positive in 20.5％ patients,ANCA negative in 11.7％ patients,P- ANCA positive with MPO- ANCA negative in one patient (2.9 ％ ) and C -ANCA positive with PR3 and MPO positive in one patient (2.9％ ).Of imaging findings,nodules or masses were most commonly observed (34％), followed by fibrotic lesions (20.5％ ), cavitations ( 17.6％ )、opacities ( 17.6％ ).Typically pathological triad ( vasculitis,necross and granuloma formation)was found in one patient ( 4.3％ ),while two pathological changes of the triad occurred in 10 patients (43.4％). Pulmonary infection was highly prevalent (50％), and of them,20.5％ patients were misdiagnosed as tuberculosis,malignancy or abscess.After admission,high dose of corticosteroids and cyclophosphamide were administered as bolus and maintenance therapy. Plasma exchange,intravenous immunoglobulin,and rituximab were added for refractory cases and optimal response were obtained in most cases.Conclusions Wegeners granulomatosis is a clinically complicated entity and sometimes can have progression,leading to misdiagnosis. Early and prompt use of steroids and cyclophosphamide may confer good therapeutic efficacy and avoid life -threatening complications.