干燥综合症合并血管炎的特点
发表者：张警丰
日期：2015-03-08
浏览次数：1016
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摘要（美国）：干燥综合征是一种主要累及外分泌腺进而可导致眼干，口干的自身免疫性疾病。干燥综合征同时可累及其他脏器。腺体外器官受累的最常见表现之一为血管炎。临床上主要表现为以紫癜为特征的皮肤血管炎，病理上主要表现为白细胞破碎性血管炎。尽管约有一半合并皮肤血管炎的患者仅有一次发作，但皮肤血管炎可能与更严重的疾病合并。干燥综合征的患者可出现类似结节性多动脉炎累及中等大小血管的坏死性血管炎。目前治疗干燥综合征合并血管炎的经验仍较少，但静脉应用免疫球蛋白可能有效。新近文献指出，视神经脊髓炎与干燥综合征相关。干燥综合症合并视神经炎或横贯性脊髓炎的患者抗水通道蛋白4抗体阳性，而抗水通道蛋白4抗体为视神经脊髓炎的特异性抗体。视神经脊髓炎患者唾液腺的淋巴细胞浸润类似于干燥综合征的表现。
附原文：Abstract Sjögren's syndrome is a chronic autoimmune disease that is commonly manifested by immune attack on the exocrine glands with resultant dry eyes and dry mouth. Sjögren's syndrome patients also have disease in other organs. One of the most common extraglandular manifestations is vasculitis. Skin vasculitis, with palpable purpura clinically and leukocytoclastic vasculitis on pathological examination, is common. Although half of those individuals with subcutaneous vasculitis have only a single episode, skin vasculitic involvement is associated with more severe disease. Necrotizing vasculitis of medium-sized vessels resembling polyarteritis nodosa can occur in Sjögren's syndrome patients. Experience in therapy for vasculitis is limited, but intravenous IgG may be effective. Recent data support a relationship between neuromyelitis optica (Devic disease) and Sjögren's syndrome. Sjögren's syndrome patients with optic neuritis or transverse myelitis have anti-aquaporin-4, which are characteristic of Devic disease. Devic disease patients have salivary lymphocytic infiltration similar to that found among Sjögren's syndrome patients.
引自：Scofield RH. Vasculitis in Sjögren's Syndrome. Curr Rheumatol Rep. 2011 Aug 26. [Epub ahead of print] （注：干燥综合症病人或亲属可加QQ群交流，群号： 118194945 ，本网站站长私人微信号： ssgzz88 ）
附原文：Abstract Sjögren's syndrome is a chronic autoimmune disease that is commonly manifested by immune attack on the exocrine glands with resultant dry eyes and dry mouth. Sjögren's syndrome patients also have disease in other organs. One of the most common extraglandular manifestations is vasculitis. Skin vasculitis, with palpable purpura clinically and leukocytoclastic vasculitis on pathological examination, is common. Although half of those individuals with subcutaneous vasculitis have only a single episode, skin vasculitic involvement is associated with more severe disease. Necrotizing vasculitis of medium-sized vessels resembling polyarteritis nodosa can occur in Sjögren's syndrome patients. Experience in therapy for vasculitis is limited, but intravenous IgG may be effective. Recent data support a relationship between neuromyelitis optica (Devic disease) and Sjögren's syndrome. Sjögren's syndrome patients with optic neuritis or transverse myelitis have anti-aquaporin-4, which are characteristic of Devic disease. Devic disease patients have salivary lymphocytic infiltration similar to that found among Sjögren's syndrome patients.
引自：Scofield RH. Vasculitis in Sjögren's Syndrome. Curr Rheumatol Rep. 2011 Aug 26. [Epub ahead of print] （注：干燥综合症病人或亲属可加QQ群交流，群号： 118194945 ，本网站站长私人微信号： ssgzz88 ）