Wegener肉芽肿

1Wegener肉芽肿的临床特点Wegener肉芽肿(Wegener granulomatosis,WG),主要侵犯肺小血管,形成血管坏死性肉芽肿,目前多归属于风湿病范畴,亦为弥漫性结缔组织病之一。WG多侵犯上气道(鼻腔)、下呼吸道及肾脏,故有人称“三联征”。但此症系全身性血管炎,故多器官受累,如咽喉、腭、     

Wegener肉芽肿

病倒报告患者女性,34岁,农民,山东莱荛人.因鼻衄、流臭涕3月余,发热、咳嗽、气短20余天于1966年5月18日入院.患者病初有鼻衄,流脓性臭悌、发热,并有左耳流脓,经耳鼻喉科检查诊断鼻部恶性肉芽肿,给用强地松、土霉素等药物治疗后,体温恢复正常.     

Wegener肉芽肿

Wegener肉芽肿 (WG)是一种以中小动脉、静脉受累为主的系统性坏死性血管炎。 1931年由 Klinge最先提出。Wegener提出经典 WG三联征 ,即上和 (或 )下呼吸道坏死性肉芽肿血管炎、周身性血管炎及局灶性坏死性肾小球肾炎。部分患者最终转变为淋巴瘤。1　病理改变195 4年 Goodman和 Churg描述 WG的病理特点为坏死性血管炎、呼吸道的坏死性肉芽肿性炎症、坏死性肾小球肾炎。 WG的镜下特点为 :1多核巨细胞肉芽肿 :中心为多核巨细胞 ,周围有白细胞和栅栏样排列的组织细胞 ;2血管炎症 :血管有炎细胞浸润、闭塞 ;3炎细胞浸润伴组织坏死。肺脏病…     

Wegener肉芽肿的诊断和治疗

1 引言 1.1 概念 Wegener 肉芽肿(Wegener's granulomatosis,WG)概念最早由Godman和Churg提出[1],是病理学概念,即为一种暴发性系统性疾病,累积多脏器系统,是以呼吸道坏死性肉芽肿、弥漫性血管炎和肾小球肾炎三联症为特征(表1).临床上分2型:(1)局限型或初发性WG,指有呼吸道病变但无肾脏受累,80%后来累及肾脏;(2)暴发型,活动性或广泛性WG,符合Godman和Churg的概念.     

Wegener肉芽肿一例

病例:患者,女,41岁。因发热40余天人院。入院前40 余天无诱因出现发热,体温37.8℃-39.5℃之间,伴鼻塞、咳 嗽、咳少许白痰、全身乏力、食欲不振、体重下降,曾做血常规 检查,白细胞1.2×109／L、血沉60mm／h,胸片示两肺中下肺 野斑片状阴影,按肺感染及肺结核治疗,病情不好转。查体:     

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.     

过敏性肉芽肿血管炎

报告４例过敏性肉芽肿性血管炎，临床主要为表现哮喘、周围血嗜酸细胞增多以及系统性血管炎。Ｘ线胸相表现多种多样。病理所见为全血管炎、嗜酸细胞浸润和肉芽肿形成。对其论断和鉴别诊断进行了讨论。     

变应性肉芽肿血管炎

变应性肉芽肿血管炎（CSS）临床病情以支气管哮喘（哮喘）为主,其归类属嗜酸粒细胞增多症范畴,又属中性粒细胞胞浆抗体（ANCA）相关性血管炎范畴,故类似的疾病和需鉴别的疾病较多而易于误诊,我科最近收治一例亦被误诊为哮喘达6年之久,为此临床医生认识此病甚为重要。     

食道Wegener肉芽肿一例

食道Ｗｅｇｅｎｅｒ肉芽肿一例韩树堂，张志宏患者男，３１岁。因右胸部阵发性胀痛２个月，进食时加重作上消化道钡餐检查：食道中段粘膜皱襞有紊乱改变，但钡剂通过顺利，食道扩张良好，考虑为炎症改变。反复２次内镜检查发现距门齿３１～３６ｃｍ的食道前壁及后壁不规则...     

ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis

Background

The pathogenic significance of antineutrophilic cytoplasmic antibodies (ANCA) in Wegener’s granulomatosis is controversial. Their presence is influenced by the extent, severity, and activity of the disease at the time of sampling. The objective of this study was to determine the frequency of ANCA in patients with active Wegener’s granulomatosis and to assess the influence of disease severity on test results.

Methods

Baseline serum samples from the 180 participants in a multicentric prospective trial were tested for ANCA by indirect immunofluorescence, direct enzyme-linked immunosorbent assay (ELISA), and capture ELISA. Disease activity was measured using the Birmingham Vasculitis Activity Score for Wegener’s granulomatosis. All patients had active disease at enrollment. Patients were categorized as having severe (n = 128) or limited (n = 52) Wegener’s granulomatosis.

Results

When all ANCA detection methods were combined, 166 patients (92%) were ANCA positive, including 96% with severe disease and 83% with limited disease.

Conclusion

ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis, but approximately 1 of 5 patients with active limited disease are ANCA negative. Immunofluorescence and both direct and capture ELISAs are required for optimal detection, suggesting that ANCA are not recognized equally well by all testing methods.     

Wegener's granulomatosis of the penis: diagnosis and management

A 24 year old uncircumcised man presented with a 1 month history of a painful, fungating lesion on his glans penis. Following biopsy, and further clinical developments, a diagnosis of Wegener's Granulomatosis (WG) was made. The penile lesion was treated with surgical debridement, and a penile stump was salvageable. This is only the fourth case of WG initially presenting with a penile lesion to be reported in the literature.     

Pancreatic involvement in Wegener's granulomatosis: a rare and difficult diagnosis

INTRODUCTION: Pancreatic involvement in the course of wegener's granulomatosis is rarely reported. EXEGESIS: We report a case presenting as a pancreatic tumorlike lesion. We also discuss six further cases from the medical literature. Two of them had a tumorlike lesion, three had acute pancreatitis and the last had exocrine pancreatic insufficiency. CONCLUSION: Treatment, association of corticoid and cyclophosphamide, is efficient if it is started quickly. So we think that this unusual manifestation of Wegener's granulomatosis should be recognized.     

Eosinophilia in Wegener's granulomatosis

Significant eosinophilia is a prominent feature in Churg-Strauss syndrome but has only rarely been described in Wegener's granulomatosis (WG). We describe two Wegener's granulomatosis patients with > 30% eosinophilia on their initial presentations. Other etiologies that could account for their eosinophilia were excluded. Both patients had pulmonary alveolar hemorrhage, sinusitis, arthritis, high-titer cytoplasmic antineutrophil cytoplasmic antibodies (cANCA), and proteinase-3 antibodies, but no evidence of renal disease. Herein we discuss eosinophilia, the differential diagnosis of pulmonary infiltrates and eosinophilia, the role of cANCA in vasculitis and autoimmune disease, compare Wegener's granulomatosis and Churg-Strauss syndrome, and review possible pathogenic mechanisms.