Holl-Ulrich K, Reinhold-Keller E, Müller A, Feller A C
Institut für Pathologie, Medizinische Universität Lübeck.
Verh Dtsch Ges Pathol. 2002;86:83-90.
The morphological diagnosis of vasculitis is a challenge for the pathologist for many reasons. Primary systemic vasculitides are rare diseases (ann. incidence 4-5/100,000 according to the Vaskulitis-Register Schleswig-Holstein), pathognomonic histopathological findings are hard to obtain in many patients even with multiple biopsies, and the nomenclature of vasculitis has long been confusing and unequivocal. The Chapel-Hill Consensus Conference (1992) is credited with the establishment of clinico-pathological definitions for primary systemic vasculitides. For the discrimination of primary systemic vasculitis from secondary vasculitis as well as pseudovasculitic syndromes, correlation with clinical data is essential; it has been facilitated by the American College of Rheumatology (ACR) classification criteria (1990). Differential diagnostic schemes for vasculitis are based on the predominant type and size of the blood vessel involved, as well as the histopathological type of inflammation and the presence or absence of immune complex deposits. However, differential diagnostic problems remain: even with the clarified nomenclature of Chapel-Hill, considerable overlap still occurs. A clinical diagnosis of vasculitis is now often established in an earlier disease stadium, in part of the cases facilitated by ANCA testing; the pathologist may therefore at first be confronted with a more subtle or abortive morphology, while in the course of disease, histopathology is modulated by immunosuppressive therapy. A number of immunopathogenic mechanisms prevailing in vasculitis can be verified with immunohistochemical and molecular biological methods, although it is frequently not known how they are initiated and perpetuated. ANCA-associated vasculitides serve as a good example to demonstrate the central role of morphology, its potentials but also its limitations in the differential diagnosis of vasculitis.
血管炎的形态学诊断对病理学家来说是一项挑战，原因众多。原发性系统性血管炎是罕见疾病（根据石勒苏益格 - 荷尔斯泰因血管炎登记处的数据，年发病率为4 - 5/10万），即使对许多患者进行多次活检，也很难获得具有诊断意义的组织病理学发现，而且血管炎的命名长期以来一直混乱且不明确。1992年的 Chapel - Hill 共识会议确立了原发性系统性血管炎的临床病理定义。为了区分原发性系统性血管炎与继发性血管炎以及假性血管炎综合征，与临床数据的关联至关重要；美国风湿病学会（ACR）1990年的分类标准对此提供了便利。血管炎的鉴别诊断方案基于受累血管的主要类型和大小，以及炎症的组织病理学类型和免疫复合物沉积的有无。然而，鉴别诊断问题依然存在：即使有了Chapel - Hill明确的命名，仍存在相当大的重叠。血管炎的临床诊断现在通常在疾病的早期阶段确立，部分病例借助抗中性粒细胞胞浆抗体（ANCA）检测得以实现；因此，病理学家最初可能面对的是更细微或不典型的形态，而在疾病过程中，组织病理学受到免疫抑制治疗的影响。血管炎中存在的一些免疫致病机制可以通过免疫组织化学和分子生物学方法得到证实，尽管通常不清楚它们是如何启动和持续存在的。以ANCA相关血管炎为例，可以很好地说明形态学在血管炎鉴别诊断中的核心作用、潜力及其局限性。
