Papini M, Quaglino P, La Placa M, Marzano A V
Terni Dermatological Clinic, Department of Surgical and Biomedical Sciences, University of Perugia, Perugia, Italy -
G Ital Dermatol Venereol. 2015 Apr;150(2):165-7.
Vasculitides are a challenge to the clinician, in terms of both diagnosis and therapy. Multiple classification systems have been implemented and the numerous classification schemes reflect the complexity of establishing a simple classification that could be functional for daily care. Although vasculitis classification has become increasingly elaborated, some areas remain ill defined. Some forms of vasculitis are still difficult to assign to a specific disease entity. Generally accepted operational criteria are available for many vasculitides, but for some entities there are no effective criteria. Moreover, diagnostic criteria for vasculitis with sufficient strength and/or confidence that can be universally accepted are not yet available. The need for diagnostic criteria validated and agreed upon is particularly relevant in the context of cutaneous vasculitis. The project of the SIDeMaST Italian Group of Immunopathology on cutaneous vasculitis is a national prospective observational study designed to develop and validate diagnostic criteria and to improve and validate classification criteria for cutaneous small vessel vasculitis also known as leukocytoclastic vasculitis (CLV). Primary objective of the study will also be that of developing the CUtaneous VAsculitis Severity Index (CUVASI). Secondary objectives of the project will be: 1) definition of the etiological agents that are most frequently associated with CLV; 2) search for possible correlations between causative agent and peculiar clinical and/or histopathological aspects; 3) evaluation of immunofluorescence pattern observed in this specific group of primitive cutaneous vasculitis in order to characterize the diagnostic sensitivity and specificity of this technique; 4) identification of a set of clinical investigations and laboratory tests to be performed for a correct CLV assessment. Actually 15 Italian dermatological clinics are contributing to the project and anticipated recruiting >100 patients with CLV. A pilot retrospective study to assess the feasibility of the project is going to be launched and if its results are positive then the prospective study will be started and it promises to be a unique opportunity to evaluate a large database on CLV in Italian population.
血管炎对临床医生而言，在诊断和治疗方面都是一项挑战。已经实施了多种分类系统，众多的分类方案反映出建立一个对日常护理有用的简单分类的复杂性。尽管血管炎分类已日益详尽，但有些领域仍定义不清。某些形式的血管炎仍难以归为特定的疾病实体。许多血管炎有普遍接受的实用标准，但对某些实体尚无有效的标准。此外，尚无具有足够强度和/或可信度且能被普遍接受的血管炎诊断标准。在皮肤血管炎背景下，对经过验证并达成共识的诊断标准的需求尤为迫切。意大利免疫病理学SIDeMaST小组关于皮肤血管炎的项目是一项全国性前瞻性观察研究，旨在制定和验证诊断标准，并改进和验证皮肤小血管血管炎（也称为白细胞破碎性血管炎，CLV）的分类标准。该研究的主要目标还将是制定皮肤血管炎严重程度指数（CUVASI）。该项目的次要目标将是：1）确定与CLV最常相关的病原体；2）寻找病原体与特殊临床和/或组织病理学方面之间可能的相关性；3）评估在这组原发性皮肤血管炎中观察到的免疫荧光模式，以表征该技术的诊断敏感性和特异性；4）确定一套用于正确评估CLV的临床检查和实验室检测。实际上，15家意大利皮肤科诊所正在参与该项目，预计将招募100多名CLV患者。一项评估该项目可行性的初步回顾性研究即将启动，如果结果呈阳性，则将开始前瞻性研究，这有望成为评估意大利人群中CLV大型数据库的独特机会。
