Helmchen U, Kneissler U, Prall F
Institut für Pathologie, Universitäts-Krankenhaus Eppendorf, Hamburg.
Verh Dtsch Ges Pathol. 1996;80:38-45.
Among the various types of primary systemic vasculitides, which were recently newly classified (Chapel Hill Classification, 1994), in two forms of small vessel vasculitis, i.e. in Wegener's granulomatosis and in microscopic polyangiitis, the diagnostic utility of anti-neutrophil cytoplasmic antibodies (ANCA) is now well established. In most cases of Wegener's granulomatosis and microscopic polyangiitis the target antigens of these autoantibodies are proteinase 3 (c-ANCA) or myeloperoxidase (p-ANCA), respectively. Both of these autoantibodies are probably not only diagnostic tools but also contribute to the pathogenic mechanisms causing the vascular damage. In Wegener's granulomatosis and in microscopic polyangiitis the upper and lower respiratory tract and the kidneys are preferentially affected. The lesions of Wegener's granulomatosis show an extremely wide morphologic spectrum. In the oropharynx, nasal sinuses, trachea, and large bronchi, they appear for the most part as ulcerations in which a granulomatous response may or may not be evident. Thus in the absence of granulomas, especially in small biopsy material, the diagnosis of Wegener's granulomatosis should by no means be excluded. In the kidneys a focal and segmental necrotizing glomerulonephritis, often associated with crescent formation, is the typical lesion in Wegener's granulomatosis and in microscopic polyangiitis, as well. Histologically, this glomerulonephritis does not differ from the forms also seen in Schoenlein-Henoch disease, in Goodpasture's syndrome and in some cases of systemic lupus erythematosus, but in Wegener's granulomatosis and in microscopic polyangiitis immune glomerular deposits are uncommon. In fact, by analysis of our biopsy material, these "pauci immune" type of necrotizing glomerulonephritis account for nearly 70%. In the past, Wegener's granulomatosis was usually fatal within 5 months of diagnosis. After introduction of the combined therapy with steroids and cyclophosphamide the prognosis improved considerably. Nevertheless, a further improvement is necessary. This could be reached, without any doubt, by an earlier detection of the disease.
在最近重新分类的（1994年 Chapel Hill 分类法）各种原发性系统性血管炎中，抗中性粒细胞胞浆抗体（ANCA）在两种小血管炎形式，即韦格纳肉芽肿病和显微镜下多血管炎中的诊断效用现已得到充分确立。在大多数韦格纳肉芽肿病和显微镜下多血管炎病例中，这些自身抗体的靶抗原分别是蛋白酶3（c-ANCA）或髓过氧化物酶（p-ANCA）。这两种自身抗体可能不仅是诊断工具，而且还参与导致血管损伤的致病机制。在韦格纳肉芽肿病和显微镜下多血管炎中，上、下呼吸道和肾脏是优先受累部位。韦格纳肉芽肿病的病变表现出极其广泛的形态学谱。在口咽、鼻窦、气管和大支气管中，它们大多表现为溃疡，其中肉芽肿反应可能明显或不明显。因此，在没有肉芽肿的情况下，尤其是在小活检材料中，绝不应排除韦格纳肉芽肿病的诊断。在肾脏中，局灶性节段性坏死性肾小球肾炎，常伴有新月体形成，也是韦格纳肉芽肿病和显微镜下多血管炎的典型病变。从组织学上看，这种肾小球肾炎与 Schönlein-Henoch 病、Goodpasture 综合征以及某些系统性红斑狼疮病例中所见的形式并无不同，但在韦格纳肉芽肿病和显微镜下多血管炎中，免疫性肾小球沉积物并不常见。事实上，通过对我们活检材料的分析，这些“寡免疫”型坏死性肾小球肾炎占近70%。过去，韦格纳肉芽肿病通常在诊断后5个月内死亡。在引入类固醇和环磷酰胺联合治疗后，预后有了显著改善。然而，仍有必要进一步改善。毫无疑问，通过更早地发现疾病可以实现这一点。
