Danve Abhijeet, Grafe Marjorie, Deodhar Atul
Division of Arthritis & Rheumatic Diseases (OP-09), Oregon Health & Science University, 3181, SW Sam Jackson Park Road, Portland, OR 97239.
Department of Pathology, Oregon Health & Science University, Portland, OR.
Semin Arthritis Rheum. 2014 Aug;44(1):86-92. doi: 10.1016/j.semarthrit.2014.02.001. Epub 2014 Feb 11.
Amyloid Beta-Related Angiitis (ABRA) is a rare cause of central nervous system vasculitis complicating cerebral amyloid angiopathy. Data regarding its prevalence, clinical features, management, and outcomes are scant.
To describe a patient with ABRA and discuss clinical features and management of ABRA.
A case report and review of literature were conducted of all reported cases of ABRA in the English literature.
The exact etiology of ABRA is not clear, though it is thought to be secondary to an inflammatory response to beta amyloid (Aβ) in the walls of blood vessels. Role of ApoE e4/e4 genotype and its association with autoimmune diseases have been reported. ABRA shares many clinical features with primary CNS vasculitis. Patients with ABRA are relatively younger than those with non-inflammatory cerebral amyloid angiopathy (CAA), but older than patients with primary central nervous system vasculitis (PCNSV). Acute-onset cognitive behavioral abnormalities, focal neurological deficits, seizures, or unusual headaches are the most common presentations of ABRA. Majority have elevated CSF proteins. Up to 70% of patients have ApoE e4/e4 genotype. MRI is the most important diagnostic tool and is almost always abnormal. Characteristically, MRI shows hyperintensities on T2-weighted (T2W) or fluid-attenuation inversion recovery (FLAIR) images with minimal gadolinium enhancement. On susceptibility-weighted images (SWI), a majority of the patients have the presence of microbleeds at cortico-subcortical junction. It may be possible to diagnose typical patients based on clinical features and MRI findings alone, obviating the need for brain biopsy. Brain biopsy is the gold standard and shows transmural granulomatous vasculitis superimposed on CAA. ABRA responds well to steroids in majority. Patients usually need additional immunosuppressants, especially to prevent relapse. MRI abnormalities resolve with treatment and recur with the relapse.
ABRA is a rare but treatable cause of progressive dementia and should be considered in the differential diagnosis of rapid-onset CNS dysfunction in patients older than 60 years. It has characteristic MRI findings and responds well to steroids and other immunosuppressant therapy.
淀粉样β相关血管炎（ABRA）是中枢神经系统血管炎的一种罕见病因，常并发脑淀粉样血管病。关于其患病率、临床特征、治疗及预后的数据较少。
描述一名ABRA患者，并探讨ABRA的临床特征及治疗方法。
对英文文献中所有已报道的ABRA病例进行病例报告及文献综述。
ABRA的确切病因尚不清楚，不过一般认为它继发于血管壁中β淀粉样蛋白（Aβ）的炎症反应。已有报道载脂蛋白E（ApoE）e4/e4基因型的作用及其与自身免疫性疾病的关联。ABRA与原发性中枢神经系统血管炎有许多共同的临床特征。ABRA患者相对比非炎症性脑淀粉样血管病（CAA）患者年轻，但比原发性中枢神经系统血管炎（PCNSV）患者年长。急性起病的认知行为异常、局灶性神经功能缺损、癫痫发作或不寻常头痛是ABRA最常见的表现。大多数患者脑脊液蛋白升高。高达70%的患者具有ApoE e4/e4基因型。磁共振成像（MRI）是最重要的诊断工具，几乎总是显示异常。典型的是，MRI在T2加权（T2W）或液体衰减反转恢复（FLAIR）图像上显示高信号，钆增强极少。在磁敏感加权成像（SWI）上，大多数患者在皮质-皮质下交界处存在微出血。仅根据临床特征和MRI表现可能就能诊断典型患者，无需进行脑活检。脑活检是金标准，显示CAA基础上的透壁性肉芽肿性血管炎。大多数ABRA患者对类固醇反应良好。患者通常需要额外的免疫抑制剂，尤其是为了预防复发。MRI异常在治疗后消退，复发时再次出现。
ABRA是进行性痴呆的一种罕见但可治疗的病因，对于60岁以上快速起病的中枢神经系统功能障碍患者，在鉴别诊断时应予以考虑。它具有特征性的MRI表现，对类固醇及其他免疫抑制治疗反应良好。
