The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. We conducted a retrospective chart review in a Western Spain multicentre survey. A total of 115 adult patients diagnosed with AAV from 2002 to 2013 and followed for at least 3 years were included. They were classified according to (1) Chapel Hill Consensus Conference (CHCC), (2) European Medicines Agency algorithm and (3) French Vasculitis Study Group/European Vasculitis Society phenotypes. Fifty-three patients (46%) had neither distinctive histopathological data of a single AAV definition nor any surrogate markers for granulomatous inflammation and thus did not fulfill any diagnostic criteria. Ocular, ear, nose, throat, skin, and lung involvement were more frequent with proteinase 3 (PR3) antibodies, whereas peripheral neuropathy was more frequent with myeloperoxidase (MPO) antibodies. When the disease was severe at diagnosis, the HR for mortality was 10.44. When induction treatment was not given in accordance with the guidelines, the HR for mortality was 4.00. For maintenance treatment, the HR was 5.49 for mortality and 2.48 for relapse. AAV classification is difficult because many patients had neither specific clinical data nor distinctive histological features of a single CHCC definition. A structured clinical assessment of patient severity is the best tool to guide the management of AAV.

中文翻译：

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在实际临床中，将ANCA相关的小血管血管炎分类的复杂性：来自多中心回顾性调查的数据。

诊断或分类抗中性粒细胞胞浆抗体相关血管炎（AAV）的不同标准准则在临床实践中导致大量重叠和重新分类的诊断。我们设计了这项研究，以评估对AAV患者进行分类的困难。作为次要目标，测试了不同的变量以预测预后。我们在西班牙西部的多中心调查中进行了回顾性图表审查。从2002年至2013年，共115名被诊断患有AAV的成年患者，并随访了至少3年。根据（1）教堂山共识会议（CHCC），（2）欧洲药品管理局的算法和（3）法国血管炎研究组/欧洲血管炎协会的表型对它们进行了分类。53名患者（46％）既没有单一AAV定义的独特组织病理学数据，也没有肉芽肿性炎症的任何替代指标，因此不符合任何诊断标准。蛋白酶3（PR3）抗体对眼，耳，鼻，喉，皮肤和肺的侵害更为频繁，而髓过氧化物酶（MPO）抗体对周围神经病变的侵害更为频繁。当疾病在诊断时严重时，死亡率的HR为10.44。如果未按照指南进行诱导治疗，则死亡率的HR为4.00。对于维持治疗，HR死亡率为5.49，复发率为2.48。AAV分类很困难，因为许多患者既没有特定的临床数据，也没有单一CHCC定义的独特组织学特征。