Isa Hazlita, Lightman Sue, Luthert Philip J, Rose Geoffrey E, Verity David H, Taylor Simon R J
Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London W12 0NN, UK.
Int J Clin Exp Pathol. 2012;5(7):684-9. Epub 2012 Sep 5.
The limited form of Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's Granulomatosis (WG) primarily involves the head and neck region, including the orbit, but is often a diagnostic challenge, particularly as it commonly lacks positive anti-neutrophil cytoplasm antibody (ANCA) titres or classical features on diagnostic orbital biopsies. The purpose of this study was to relate biopsy findings with clinical outcome and to determine which histopathological features are predictive of a clinical diagnosis of GPA.
Retrospective case series of 234 patients identified from the database of the UCL Institute of Ophthalmology Department of Eye Pathology as having had orbital biopsies of orbital inflammatory disorders performed between 1988 and 2009. Clinical records were obtained for the patients and analysed to see whether patients had GPA or not, according to a standard set of diagnostic criteria (excluding any histopathological findings). Biopsy features were then correlated with the clinical diagnosis in univariate and multivariate analyses to determine factors predictive of GPA.
Of the 234 patients, 36 were diagnosed with GPA and 198 with other orbital pathologies. The majority of biopsies were from orbital masses (47%). Histology showed a range of acute and chronic inflammatory pictures in all biopsies, but the presence of neutrophils (P<0.001), vasculitis (P<0.001), necrosis (P<0.001), eosinophils (P<0.02) and macrophages (P=0.05) were significantly associated with a later clinical diagnosis of GPA. In a multivariate analysis, only tissue neutrophils (OR=3.6, P=0.01) and vasculitis (OR=2.6, P=0.02) were independently associated with GPA, in contrast to previous reports associating eosinophils and necrosis with the diagnosis.
Neutrophil, eosinophil and macrophage infiltration of orbital tissues, together with vasculitis and necrosis, are all associated with a clinical diagnosis of GPA, but only neutrophil infiltration and vasculitis are independently associated with this diagnosis. These features may assist in the establishing the diagnosis of limited GPA among patients with early orbital disease, particularly in the absence of positive serum ANCA titres.
局限性肉芽肿性多血管炎（GPA），以前称为韦格纳肉芽肿（WG），主要累及头颈部区域，包括眼眶，但通常是一个诊断难题，尤其是因为它通常缺乏抗中性粒细胞胞浆抗体（ANCA）阳性滴度或诊断性眼眶活检的典型特征。本研究的目的是将活检结果与临床结局相关联，并确定哪些组织病理学特征可预测GPA的临床诊断。
从伦敦大学学院眼科研究所眼病理科数据库中识别出234例患者的回顾性病例系列，这些患者在1988年至2009年间接受了眼眶炎性疾病的眼眶活检。获取患者的临床记录，并根据一组标准诊断标准（不包括任何组织病理学发现）分析患者是否患有GPA。然后在单变量和多变量分析中将活检特征与临床诊断相关联，以确定GPA的预测因素。
在234例患者中，36例被诊断为GPA，198例患有其他眼眶疾病。大多数活检来自眼眶肿块（47%）。组织学显示所有活检中均有一系列急性和慢性炎症表现，但中性粒细胞的存在（P<0.001）、血管炎（P<0.001）、坏死（P<0.001）、嗜酸性粒细胞（P<0.02）和巨噬细胞（P=0.05）与GPA的后期临床诊断显著相关。在多变量分析中，与先前将嗜酸性粒细胞和坏死与诊断相关联的报告相反，只有组织中性粒细胞（OR=3.6，P=0.01）和血管炎（OR=2.6，P=0.02）与GPA独立相关。
眼眶组织中的中性粒细胞、嗜酸性粒细胞和巨噬细胞浸润，以及血管炎和坏死，均与GPA的临床诊断相关，但只有中性粒细胞浸润和血管炎与该诊断独立相关。这些特征可能有助于在早期眼眶疾病患者中确立局限性GPA的诊断，特别是在血清ANCA滴度为阴性的情况下。
