韦格纳肉芽肿病临床分析

目的 探讨韦格纳肉芽肿病的临床特点,以提高对本病的认识.方法 回顾性分析1992年4月至2012年5月确诊的50例韦格纳肉芽肿病患者的临床资料,包括临床表现,实验室、影像学及病理检查等.结果 男22例,女28例,发病年龄为13～74岁,平均43.8岁.确诊时间为14 d～180个月,平均16.7个月.临床以眼(38％)、耳(20％)、鼻(64％)、口咽(26％)等上呼吸道及肺部(58％)和肾(44％)受累为主.胞质型抗中性粒细胞胞质抗体(cANCA)阳性率96％,核周型抗中性粒细胞胞质抗体(pANCA)阳性率12％.病理表现为坏死性肉芽肿性炎症、炎细胞浸润的血管炎.治疗以糖皮质激素和免疫抑制剂为主.结论 韦格纳肉芽肿病的临床表现复杂多样,缺乏特异性体征,靶器官受损有多系统或器官病变的症候群,尤以上、下呼吸道及肾脏受累最多见,病理特点为坏死性肉芽肿和血管炎.     

韦格纳肉芽肿病18例临床分析

韦格纳肉芽肿病 (Wegener′sgranulomatosis,WG)是一种病因未明的系统性炎症性疾病 ,发病率低。临床表现多种多样 ,常累及上下呼吸道、肾脏、皮肤、眼部、生殖器等多器官 ,现将我院自 1997年 6月至 2 0 0 2年 12月收治的WG患者的临床情况进行回顾性分析。1　资料与方法1 1　临床资料1 1 1　一般资料 :WG患者 18例 ,符合 1990年美国WG诊断标准。男性 13例 ,女性 5例 ,年龄 19～ 6 1岁 ,平均 (39±6 )岁 ,排除复发性多软骨炎等其他疾病。1 1 2　临床表现 :全部病例均具有乏力、消瘦、周身不适、肌痛、低～中度发热等全身非特异性表现。此…     

韦格纳肉芽肿病100例临床分析

目的 总结分析100例韦格纳肉芽肿病(WG)患者的临床持点方法回顾性分析北京协和医院1998年1月至2009年12月确诊的100例WG患者的临床资料结果100例患者的男女比例1.04∶1,发病年龄4～72岁,平均(39±17)岁.上呼吸道(86%)、肺脏(82%)、肾脏(70%)和眼(53%)受累常见,神经系统(12%)及心脏(11%)等相对少见.胞质型抗中性粒细胞胞质抗体(cANCA)阳性率为77%,ANCA阴性率为8%.影像学以肺部多发结节/肿块影伴或不伴空洞病灶(59%,48/81)及鼻窦炎(57%,44/77)最常见.具有诊断意义的病理为坏死性肉芽肿和(或)小血管炎,鼻黏膜/肿中物及肺活检分别占78%及75%,肾活检以局灶节段坏死性肾小球肾炎(59%)最有诊断意义.WG亚组以全身型(49%)最常见,其次为局限型(22%)、早期全身型(15%)、重症肾脏病型(9%)及难治型(5%).激素联合环磷酰胺治疗,诱导缓解期总缓解率为78%,其中早期全身型最高(73%),病死率为4%.随访1～145个月,16例(22%)合并感染,9例(12%)合并慢性肾功能不全,8例(11%)并发深静脉血栓5例(8%)死亡,其中2例死于感染结论WG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,减少并发症的发生,改善预后.     

韦格纳肉芽肿

韦格纳肉芽肿又称韦格纳肉芽肿病 ( WG)。 193 6年首先由 Wegener描述。主要表现为呼吸道肉芽肿性坏死性血管炎及弥漫性坏死性血管炎 ,肾小球肾炎常见 ,为一种少见严重的多系统受累疾病。WG原因不明 ,早期病变内常有急性白细胞破碎性血管炎的改变 ,以后发现 Ig G、Ig A及 CIC异常。近年发现抗中性粒细胞浆抗体与 WG相关 ,这些都支持本病为一种自身免疫性疾病。WG在白种人多见 ,在美国估计其发病率为百万分之三。多见于男性 ,常起病于 3 0～ 4 0岁。少数患者起病急骤 ,多数呈现慢性病程。本病无特异的前驱症状 ,病情呈进行性发展。常…     

韦格纳肉芽肿一例

患者,男性,58岁,因咳嗽、咳痰2个月,右胸痛,关节痛1个月入院.2个月前受凉后出现咳嗽、咳少量黄痰,自服感冒药等疗效欠佳.1个月前无明显诱因出现右侧胸背部疼痛,伴肩、肘、腕、踝等关节疼痛,到外院诊治,行肺CT检查发现两肺多发性团块、结节,考虑炎性假瘤可能,予抗感染及对症支持治疗半个月,病情无明显好转,为求进一步诊治而到我院呼吸科就诊.     

韦格纳肉芽肿一例

患者 :男性 ,36岁。因咳嗽、胸痛 2个月 ,加重伴胸闷 3ｄ于 1999年 9月 7日收入院。患者 2个月前无明显诱因出现咳嗽 ,咯少量白黏痰 ,偶尔痰中带血丝 ,并感双侧胸部隐痛 ,发热 ,体温达 37 5～ 38 5℃ ,无盗汗、乏力 ,在当地医院就诊为“上呼吸道感染” ,口服感冒药无效 ,8月 7日摄胸片示右下肺 2ｃｍ× 1ｃｍ结节影 ,按炎症给予希普欣静点治疗 1周 ,上述症状无好转 ,再次复查胸片示两肺多发性结节影 ,遂到省某医院住院 ,给予左旋氧氟沙星治疗半个月 ,并经皮肺穿刺 2次 ,拟诊韦格纳肉芽肿。入院前 3ｄ ,咳嗽、胸痛加重 ,伴胸闷、憋气 ,转入我…     

重视韦格纳肉芽肿病的诊断与治疗

血管炎病作为一大类风湿性疾病 ,在我国很长一段时间内未被大多数临床医师所认识 ,其临床的复杂性和诊治的困难性也一直是困扰我们风湿病专科医师的一个问题。近几年由于风湿病学在我国的普及发展 ,血管炎病已逐渐引起人们的重视。张乃峥教授于本刊 1998年第 3期发表了“由三个个案报道引起的思考———论血管炎病” ,提出要重视血管炎病的诊断与治疗。去年中华医学会风湿病学分会召开了全国血管炎的专题研讨会 ,这些促进了人们对血管炎病的认识。韦格纳肉芽肿病 (Wegener′sgranulomatosis ,WG)是一种坏死性肉芽肿性血管炎 ,近几年在我国…     

韦格纳肉芽肿病二例

本文报道 2例经病理证实为韦格纳肉芽肿病 (Ｗｅｇｅｎｅｒｇｒａｎｕｌｏｍａｔｏｓｉｓ,ＷＧ)的患者并分析如下。1　病历摘要例 1:男性 ,72岁。因咳嗽、咯血痰、鼻出血、脓涕、胸痛、发热 10ｄ ,于 2 0 0 0年 10月 11日入院。体检 :体温 38 4℃ ,脉搏 14 0次 /ｍｉｎ ,呼吸 2 0次 /ｍｉｎ ,血压 12 0 / 80ｍｍＨｇ(1ｍｍＨｇ =0 133ｋＰａ) ,消瘦 ,双侧鼻腔黏膜充血 ,有脓痂 ,部分黏膜糜烂 ,双肺呼吸音粗 ,心率 14 0次 /ｍｉｎ ,律齐 ,心音低钝 ,肝脾未触及。实验室检查 :血白细胞 2 2× 10 9/Ｌ、中性0 76、淋巴 0 16、血小板 375× 1…     

以皮肤黏膜损害为突出表现的韦格纳肉芽肿病二例

~~以皮肤黏膜损害为突出表现的韦格纳肉芽肿病二例@茹晋丽!030001太原$山西医科大学第二医院风湿免疫科 @李小峰!030001太原$山西医科大学第二医院风湿免疫科 @李军$霍州煤电集团公司总医院内科 @王秀哲!030001太原$山西医科大学第二医院风湿免疫科 @王来远!030001太原$山西医科大学第二医院风湿免疫科~~     

抗蛋白酶-3抗体在韦格纳肉芽肿病和其他血管炎中的临床应用研究

目的探讨抗蛋白酶-3(PR3)抗体在韦格纳肉芽肿病(WG)和其他血管炎患者中的表达和临床意义。方法选取2001年3月至2006年7月山西医科大学第二医院确诊的住院患者576例。系统性血管炎组111例,其中9例WG(包含21份跟踪随访血清)；结缔组织病(CTD)组403例；各型肾小球疾病患者62例及健康对照30名,均采用酶联免疫吸附试验(ELISA)检测抗PR3抗体、抗髓过氧化物酶(MPO)抗体；采用间接免疫荧光法(IIF)检测抗中性粒细胞胞质抗体(ANCA),观察抗PR3与ANCA在WG和其他血管炎中的阳性率,且追踪WG治疗前后抗PR3吸光度值和ANCA的滴度变化。结果588例血清中抗PR3抗体阳性23例,分别为WG 15例(15/21,71．4％)；系统性红斑狼疮(SLE)6例(6/213,2．8％)：类风湿关节炎(RA)1例(1/135,0．7％)；混合结缔组织病(MCTD)1例。大动脉炎、白塞病,过敏性紫癜等常见的原发性系统性血管炎、肾病组、健康对照组未发现抗PR3抗体阳性。抗PR3抗体和胞质型ANCA(cANCA)在WG中阳性率最高,差异有统计学意义(P＜0．05)。抗PR3对WG诊断的敏感性71．42％,特异性98．58％。联合应用抗PR3与cANCA诊断WG的敏感性61．90％,特异性99．82％。抗PR3吸光度值、ANCA的滴度及伯明翰血管活动度评分(BVAS)随治疗好转下降。结论抗PR3抗体是诊断WG的一种敏感、特异标记抗体,抗PR3抗体和ANCA同时在临床应用,有利于WC和其他系统性血管炎的早期诊断和鉴别诊断。抗PR3抗体还可作为临床疗效观察指标。     

Wegener's granulomatosis: clinical and laboratory results of a university hospital study of 20 patients from Turkey

The aim of this study was to evaluate the clinical and laboratory features, the treatment approaches, and the long-term outcome of patients with Wegener's granulomatosis (WG) who were followed up in our hospital. The hospital files of the patients with the diagnosis of WG who were followed up between the years 1985 and 2003 in Hacettepe University Hospital were retrospectively evaluated. Male/female ratio was 12:8. The mean age was 39 years (range 20–65 years). Constitutional symptoms and upper and lower airway involvement were seen in 95% of all patients. Renal and musculoskeletal symptoms were seen in 90 and 80% of the patients, respectively. Five patients were treated with oral monotherapy (three with methylprednisolone and two with cyclophosphamide). Three patients were given a combination of orally administered cyclophosphamide and methylprednisolone. Ten patients were treated with pulse cyclophosphamide and methylprednisolone combination together with oral alternate-day methylprednisolone therapy. The remaining two resistant patients were treated with pulse cyclophosphamide, methylprednisolone, and intravenous immunoglobulin combination together with oral alternate-day methylprednisolone. Four patients died because of the disease activity. Intravenous pulse therapies with oral, alternate-day methylprednisolone were well tolerated. Sixteen patients experienced long-term remission after immunosuppressive treatment. Eleven patients have been asymptomatic for more than 12 months. In five patients, residual symptoms persisted: constitutional symptoms and renal and respiratory tract symptoms in varying combinations. The demographic and laboratory findings in this trial were similar with those of the previous results. Alternate-day glucocorticoids plus cyctotoxic drugs may be beneficial in patients with WG.     

韦格纳肉芽肿病合并肺部感染的临床分析

目的 分析韦格纳肉芽肿病合并肺部感染的临床特点和危险因素.方法 回顾性分析北京协和医院1998-2009年收治的韦格纳肉芽肿病合并肺部感染患者的临床资料.采用t检验和Fisher检验进行统计分析.结果 92例韦格纳肉芽肿病患者中合并肺部感染27例(29％).其中首次就诊即合并肺部感染7例占26％,确诊韦格纳肉芽肿病0～6个月内发生肺部感染12例(44％),确诊韦格纳肉芽肿病6个月以上发生肺部感染8例(30％).肺部感染表现为咳嗽、咯痰(89％),咯血(63％),发热、乏力(56％),胸闷、胸痛(33％).主要为细菌感染(59％),真菌感染(37％),结核分枝杆菌感染(37％).WG合并肺部感染组有鼻窦感染( P=0.01)、低蛋白血症(P=0.03)、低免疫球蛋白血症(P=0.007)、接受激素冲击治疗(P=0.002)的比例高于无肺部感染组.结论 韦格纳肉芽肿病合并肺部感染0～6个月内发生率高,临床上表现为咳嗽、咯痰,病原学检查细菌、真菌、结核感染多见,与鼻窦感染、低蛋白血症、低免疫球蛋白血症、接受激素冲击治疗有关.     

韦格纳肉芽肿病合并静脉血栓临床分析

目的 分析总结韦格纳肉芽肿病(WG)合并静脉血栓(VTE)的发病率、与疾病活动度的关系及其临床特点,寻找发生VTE的危险因素.方法 回顾性分析2000年2月至2008年4月我院WG住院患者临床资料.结果 总计71例WG患者中合并VTE 7例(10%),2例肺栓塞(PE),5例深静脉血栓(DVT),全部发生在疾病活动期.临床表现各异.合并VTE组与未合并VTE组比较,男性(P=0.012)、尿蛋白定量(24 h)≥3.0 g(P=0.001)及肌酐增高(P=0.004)2组间差异有统计学意义,外伤、肿瘤、药物等常见的危险因素2组之间差异无统计学意义.结论 WG患者VTE发病率增高,尤其在WG活动期.男性、尿蛋白定量(24 h)≥3.0 g及肌酐增高为危险因素,对存在危险因索的WG患者应警惕VTE的可能.     

Wegener's granulomatosis complicated with intestinal ulceration

We report the case of a 32-year-old man who developed Wegener's granulomatosis complicated with refractory intestinal ulceration. In August 2001, he presented with a high fever, nasal bleeding, and bilateral leg numbness. These symptoms worsened, which prompted him to consult his home doctor on February 18, 2002. In spite of treatment with antibiotics, his symptoms did not improve. Furthermore, abdominal pain and melena occurred as additional symptoms in March 2002. He was admitted to our hospital on April 5, 2002. A deformed nose condition (the so-called saddle nose) was observed at this time. Laboratory data showed a high erythrocyte sedimentation rate (103 mm/h) and a high level of serum C-reactive protein (14.98 mg/dl), and hematuria and proteinuria were also observed. The patient was positive for an antineutrophil cytoplasmic antibody specific for proteinase-3 (PR3-ANCA). A chest computed tomography (CT) scan revealed multiple pulmonary nodules in the lung field. A biopsied-specimen from the nasal mucosa showed necrotizing granulomatosis with giant cells. Together with his symptoms and the laboratory and pathological findings, the patient was diagnosed as having Wegener's granulomatosis. A colon fiberscopy showed multiple ulcerations with bleeding from the terminal ileum to the ascending colon, and nodular lesions at the terminal ileum. We started a combination therapy of prednisolone (60 mg/day) and cylophosphamide (100 mg/day) orally. The patient's gastrointestinal symptoms disappeared and abnormal serological indicators improved. Although Wegener's granulomatosis complicated with refractory intestinal ulceration is rare, this case indicates that the gastrointestinal region is also a target organ of Wegener's granulomatosis.     

Limited Wegener's granulomatosis with central nervous system involvement and fatal outcome

Nordmark G, Boquist L, Rönnblom L (Boden Hospital and University Hospital, Umeå, Sweden). Limited Wegener's granulomatosis with central nervous system involvement and fatal outcome (Case Report). J Intern Med 1997; 242 : 433–36.
A 74-year-old woman was admitted to the medical department with vertigo and confusion, and suffered seizures three days later. Clinical, radiological and histopathological examination revealed limited Wegener's granulomatosis (WG) with central nervous system (CNS) involvement. Initially she was treated successfully with prednisolone and cyclophosphamide, but she relapsed after 25 months and died four weeks later. Autopsy showed widespread granulomas with vasculitis in the cerebrum. These findings emphasize the importance of considering WG in CNS disease of unclear origin, and demonstrate the aggressive nature of the disease.     

Wegener’s granulomatosis and giant cell tumor of tendon sheaths: casual or causal combination? First case report

Giant cell tumor of tendon sheath (GCTTS) and Wegener’s granulomatosis (WG) are rare conditions both characterized by polyclonal cellular proliferation and multinucleated giant cells formation. Here, we report the case of a 27-year-old Caucasian woman affected by WG who experienced the metachrone appearance of two different GCTTSs at the right hand within a time of 3 years. To our knowledge, the combination of GCTT with WG is exceptional and this could probably be the first case reported. The subsequent appearance of two rare diseases both characterized by giant cell formation apparently points to similarities in their pathogenesis. However, at present no pathogenic relationship between GCTTS and WG is demonstrable and their simultaneous occurrence has to be considered coincidental. Actually, an emerging opinion is to consider GCTTS as a mixed lesion in which both tumoral and non-tumoral inflammatory cells play a central pathogenic action. On this view, the proposed case could support the evidence about the crucial role of a chronic inflammatory injury in enhancing GCTTS appearance.     

Colitis as the initital presentation of Wegener's granulomatosis.

Abstract. Schneider A, Menzel J, Gaubitz M, Keller R, Domschke W (University of Munster, Munster, Germany). Colitis as the initial presentation of Wegener's granulomatosis (Case Report).
Wegener's granulomatosis is a vasculitis which commonly presents with symptoms referable to the upper airway system, lungs, joints, eyes and kidneys. We present a case in which colitis combined with sinusitis could retrospectively be attributed as the initial manifestations of Wegener's disease.     

Wegener's granulomatosis—use of cyclosporin-A: a case report

Wegener's granulomatosis is a chronic relapsing condition requiring long term immunosuppressive treatment. Periodic exacerbations may require increased dosage of steroids and/or cyclophosphamide. We present a case of a patient with end-stage renal failure who had repeated pulmonary cavitating lesions but was unable to tolerate high dose standard therapy. After only a few months with cyclosporin A there was almost complete resolution of his lung lesions.