Primary Angiitis of the Central Nervous System and Its Mimics: A Pattern-Based Approach to Biopsies for Central Nervous System Vasculitis.

Ryan Cecchi, Nicole Becker, Kyle S Conway
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Abstract

Context.—: Primary angiitis of the central nervous system is a rare vasculitis that affects small parenchymal and leptomeningeal vessels in the brain and spinal cord. As brain biopsy remains the gold standard in diagnosis, the diagnostic approach to brain biopsies for vasculitis is well described. However, a significant minority of brain biopsies for central nervous system (CNS) vasculitis identify alternative diagnoses. Therefore, it is critical to be aware of the spectrum of infectious, inflammatory, and neoplastic diseases that may enter into the differential diagnosis of a biopsy for potential CNS vasculitis and recognize the pathologic patterns that characterize these diseases.

Objective.—: To review the broad diagnostic considerations in evaluating a biopsy for potential CNS vasculitis, with an emphasis on a pattern-based approach and diagnostic tools used to evaluate other entities in the differential diagnosis.

Data sources.—: This review is based on the clinical experience of the authors and a review of the literature.

Conclusions.—: A diagnosis of CNS vasculitis is dependent on the identification of transmural inflammation and vascular wall damage, preferentially affecting leptomeningeal and superficial cortical vessels. Where a diagnosis of vasculitis is not made, granulomatous, lymphocytic, and necrotizing patterns of inflammation present alternative differential diagnoses. The appropriate application of immunohistochemistry, special stains, and other diagnostic tools is critical in resolving these diagnoses.

原发性中枢神经系统血管炎及其模拟:一种基于模式的中枢神经系统血管炎活检方法。
上下文。-:原发性中枢神经系统脉管炎是一种罕见的血管炎,影响脑和脊髓的小实质和小脑膜血管。由于脑活检仍然是诊断的金标准,因此对血管炎的脑活检诊断方法进行了很好的描述。然而,在中枢神经系统(CNS)血管炎的脑活检中,有少数人发现了其他诊断。因此,了解传染性、炎症性和肿瘤性疾病的谱系是至关重要的,这些疾病可能会进入潜在中枢神经系统血管炎的活检鉴别诊断,并识别这些疾病的病理模式。-:回顾评估潜在中枢神经系统血管炎活检的广泛诊断考虑,重点是基于模式的方法和用于评估鉴别诊断中其他实体的诊断工具。数据源。-:本综述基于作者的临床经验和文献综述。-:中枢神经系统血管炎的诊断依赖于跨壁炎症和血管壁损伤的识别,优先影响薄脑膜和皮层浅血管。当没有血管炎的诊断时,肉芽肿性、淋巴细胞性和坏死性的炎症表现为可选的鉴别诊断。适当应用免疫组织化学、特殊染色和其他诊断工具是解决这些诊断的关键。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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