Francès C, Boisnic S, Blétry O
Rev Prat. 1989 Oct 11;39(23):2029-36.
The diagnosis of necrotizing vasculitis rests on histology which shows parietal fibrinoid necrosis, vascular and perivascular infiltrates and intraluminal thrombosis in varying degrees of association. However, histopathological classifications of necrotizing vasculitis according to the size of the diseased vessels or the appearance of vascular infiltrates are not satisfactory, since microvasculitis may successively to simultaneously involve vessels of different calibers and result (generally in succession) in leukoclastic or lymphocytic vasculitis. For this reason we give preference to an aetiological classification which includes five major entities, some of which were described over a century ago (periarteritis nodosa, Churg-Strauss syndrome, Wegener's disease, rheumatoid purpura and Mac Duffie syndrome); drug-induced vasculitis, infective vasculitis, vasculitis associated with blood diseases; vasculitis of connective tissue diseases; vasculitis that may accompany some system diseases (e.g. Kawasaki disease and Cogan's syndrome); and vasculitis that may complicate cryoglobulinaemia or congenital deficiencies in complement fraction or in alpha-1 antitrypsin. It can be seen that this is not the ideal classification, since the same causative agent may be found in several chapters (e.g. HB virus in periarteritis nodosa, infective vasculitis and cryoglobulinaemia). We describe in detail only those types of vasculitis which are not treated in other in this issue of the Revue du Praticien, notably Wegener's disease, Mac Duffie syndrome and Kawasaki disease.
坏死性血管炎的诊断依赖于组织学检查,其表现为壁层纤维素样坏死、血管及血管周围浸润以及不同程度相关的管腔内血栓形成。然而,根据病变血管大小或血管浸润表现对坏死性血管炎进行组织病理学分类并不令人满意,因为微血管炎可能先后或同时累及不同管径的血管,并(通常先后)导致白细胞破碎性或淋巴细胞性血管炎。因此,我们更倾向于病因学分类,它包括五个主要类型,其中一些在一个多世纪前就已被描述(结节性多动脉炎、变应性肉芽肿性血管炎、韦格纳肉芽肿病、类风湿性紫癜和麦克达菲综合征);药物性血管炎、感染性血管炎、与血液疾病相关的血管炎;结缔组织病性血管炎;可能伴随某些系统性疾病的血管炎(如川崎病和科根综合征);以及可能并发冷球蛋白血症或补体成分或α-1抗胰蛋白酶先天性缺乏的血管炎。可以看出这并非理想的分类,因为同一病原体可能出现在几个类别中(如结节性多动脉炎、感染性血管炎和冷球蛋白血症中的乙肝病毒)。我们仅详细描述本期《实用医师杂志》中未在其他地方论述的那些类型的血管炎,特别是韦格纳肉芽肿病、麦克达菲综合征和川崎病。