Specks U, DeRemee R A
Division of Thoracic Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota.
Rheum Dis Clin North Am. 1990 May;16(2):377-97.
Wegener's granulomatosis and the Churg-Strauss syndrome are both syndromes that appear to begin with a phase of regionally limited symptomatology before they progress at unpredictable rate to a generalized phase characterized by symptoms of systemic vasculitis. The clinical features of atopy, peripheral blood eosinophilia, and tissue eosinophilia distinguish CSS from WG, with its typical necrotizing granulomatous respiratory tract lesions. Whereas in generalized WG with renal involvement the use of cyclophosphamide usually cannot be avoided, the generalized systemic vasculitis phase of CSS appears to respond well to glucocorticoids alone. For the more limited forms of WG, adapted therapy regimens including trimethoprim-sulfamethoxazole have been reported to be successful. Anticytoplasmic autoantibodies (c-ANCA = ACPA) are a new diagnostic serum test with high specificity for WG. Serial determinations of c-ANCA are a promising tool to monitor disease activity.
韦格纳肉芽肿病和变应性肉芽肿性血管炎综合征似乎都始于局部症状受限阶段，之后以不可预测的速度进展至全身性阶段，其特征为系统性血管炎症状。特应性、外周血嗜酸性粒细胞增多症和组织嗜酸性粒细胞增多症的临床特征将变应性肉芽肿性血管炎综合征与韦格纳肉芽肿病区分开来，后者具有典型的坏死性肉芽肿性呼吸道病变。在伴有肾脏受累的全身性韦格纳肉芽肿病中，通常无法避免使用环磷酰胺，而变应性肉芽肿性血管炎综合征的全身性血管炎阶段似乎对单独使用糖皮质激素反应良好。对于局限性韦格纳肉芽肿病，据报道包括甲氧苄啶 - 磺胺甲恶唑在内的适应性治疗方案是成功的。抗细胞质自身抗体（c - ANCA = ACPA）是一种对韦格纳肉芽肿病具有高特异性的新型诊断血清检测方法。连续测定c - ANCA是监测疾病活动的一种有前景的工具。
