A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature

A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature
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DOI:
10.1136/ard.59.6.478
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发表时间:
2000-06-01
影响因子:
27.4
通讯作者:
Petersen, J
Petersen, J
中科院分区:
医学1区
文献类型:
--
作者:
Sorensen, SF;Slot, O;Petersen, J

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测试的有用性的查佩尔山命名法,补充替代参数,作为原发性vasculitides. Methods的诊断标准,以前瞻性地评估血管炎患者根据标准化的临床和准临床程序。根据查佩尔山出版物,使用替代参数:蛋白尿、血尿和红细胞。蒙上(肾小球肾炎),血管造影或超声显示动脉瘤或狭窄(动脉炎)、放射性肺浸润或空洞持续时间超过1个月(肺部肉芽肿)、血性鼻涕或结痂、慢性鼻窦炎、耳炎和/或乳突炎、骨和/或软骨破坏和急性听力损失结果-诊断出以下实体:巨细胞动脉炎(n=14),大动脉炎(n=1),结节性多动脉炎(n=2),韦格纳肉芽肿病(n=27),Churg-Strauss综合征(n=2),显微镜下多血管炎(n=12)、过敏性紫癜(n=2)、皮肤白细胞破碎性血管炎(n=37)和继发性血管炎(n=21)。巨细胞。所有病例均经活检确诊为动脉炎和皮肤白细胞破碎性脉管炎。使用补充替代参数的查佩尔山命名法,27名患者中只有8名被诊断出韦格纳肉芽肿病,12名患者中只有3名被诊断出显微镜下多血管炎。其余诊断实体的患者人数被认为是少数evaluation.Conclusions-The查佩尔山命名法,补充替代参数,未能作为韦格纳肉芽肿和显微镜下多血管炎的诊断标准。提出了以下韦格纳肉芽肿病的诊断标准:(1)呼吸系统肉芽肿性炎症的活检或替代参数;(2)活检证实的小至中等血管坏死性血管炎或肾小球肾炎的活检/替代参数或阳性PR 3-ANCA检测;(3)血液和活检样本中缺乏嗜酸性粒细胞增多。以下是显微镜下多血管炎的诊断标准:(1)活检证实的小血管中的坏死性血管炎和/或肾小球肾炎,几乎没有或没有免疫沉积物,和(2)如活检证实的小至中型血管中的血管炎或肾小球肾炎的替代参数所示,累及一个以上的器官系统,和(3)缺乏呼吸系统肉芽肿性炎症的活检和替代参数。使用这些标准,所有韦格纳的患者和9 12例显微镜下多血管炎可以诊断。
Objective-To test the usefulness of the Chapel Hill nomenclature, supplemented with surrogate parameters, as diagnostic criteria for primary vasculitides.Methods-To prospectively evaluate vasculitis patients according to a standardised clinical and para-clinical programme. In accordance with the Chapel Hill publication surrogate parameters were used: proteinuria, haematuria and red blood cell. casts (glomerulonephritis), angiographic or ultrasonic demonstration of aneurysms or stenoses (arteritis), radiological lung infiltrates or cavitations of more than one month's duration (granuloma in the lungs), bloody nasal discharge or crusts, chronic sinusitis, otitis and/or mastoiditis, bone and/or cartilage destruction, and acute hearing loss (granuloma in upper airways).Results-The following entities were diagnosed: giant cell arteritis (n=14), Takayasu arteritis (n=1), polyarteritis nodosa (n=2), Wegener's granulomatosis (n=27), Churg-Strauss syndrome (n=2), microscopic polyangiitis (n=12), Henoch-Schonlein purpura (n=2), cutaneous leucocytoclastic angiitis (n=37), and secondary vasculitis (n=21). Giant cell. arteritis and cutaneous leucocytoclastic angiitis were in all cases diagnosed by biopsy. Using the Chapel Hill nomenclature supplemented with surrogate parameters, only 8 of 27 patients were diagnosed with Wegener's granulomatosis, and 3 of 12 cases with microscopic polyangiitis. The number of patients in the remaining diagnostic entities were considered to few to evaluate.Conclusions-The Chapel Hill nomenclature, supplemented with surrogate parameters, failed to act as diagnostic criteria in Wegener's granulomatosis and microscopic polyangiitis. The following diagnostic criteria are proposed for Wegener's granulomatosis: (1) Biopsy or surrogate parameter for granulomatous inflammation in the respiratory system and (2) Biopsy verified necrotising vasculitis in small to medium sized vessels or biopsy/surrogate parameter for glomerulonephritis or positive PR3-ANCA test and (3) Lack of eosinophilia in blood and biopsy samples. The following diagnostic criteria are proposed for microscopic polyangiitis: (1) Biopsy verified necrotising vasculitis in small vessels and/or glomerulonephritis with few or no immune deposits and (2) Involvement of more than one organ system as indicated by biopsy verified vasculitis in small to medium sized vessels or surrogate parameter for glomerulonephritis and (3) Lack of biopsy and surrogate parameter for granulomatous inflammation in the respiratory system. Using these criteria all Wegener's patients and 9 of 12 patients with microscopic polyangiitis could be diagnosed.