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系统性红斑狼疮中的血管炎。

Vasculitis in systemic lupus erythematosis.

作者信息

Calamia Kenneth T, Balabanova Maria

机构信息

Division of Rheumatology, Mayo Clinic, Jacksonville, Florida 32224, USA.

出版信息

Clin Dermatol. 2004 Mar-Apr;22(2):148-56. doi: 10.1016/j.clindermatol.2003.12.022.

Abstract

Vasculitis in connective tissue diseases is not an uncommon complication. Vasculitis complicates both rheumatoid arthritis and systemic lupus erythematosis (SLE) in about 4% of cases. Cutaneous lesions, representing small-vessel involvement, are most common; however, widespread, necrotizing visceral medium-and large-vessel involvement, mimicking primary vasculitic syndromes, may also occur. Connective tissue disease-associated vasculitis is separated from primary vasculitis syndromes in classification schemes. Granulomatous large-vessel disease does not occur in connective tissue diseases, suggesting a different pathogenesis. In most disorders, the etiology of vascular inflammation in not completely understood, but basic pathogenic mechanisms can often be distinguished. The role of immune complexes in the inflammatory manifestations of SLE is recognized, and other pathogenic factors such as antineutrophil cytoplasmic antibodies, common in other vasculitides, are infrequent. A diverse spectrum of clinical features, due to inflammatory involvement of arterial and venous vessels of all sizes, characterize several connective tissue diseases including Behçet's disease and SLE. The recognition of disease manifestations due to vasculitis in these disorders has important implications for treatment and may be critical to reduce morbidity and mortality.

摘要

血管炎是结缔组织病中并不少见的并发症。血管炎使类风湿关节炎和系统性红斑狼疮(SLE)的并发症发生率约为4%。皮肤病变代表小血管受累,最为常见;然而,也可能出现广泛的、坏死性的内脏中、大血管受累,类似于原发性血管炎综合征。在分类方案中,结缔组织病相关血管炎与原发性血管炎综合征是分开的。肉芽肿性大血管疾病在结缔组织病中不会出现,提示发病机制不同。在大多数疾病中,血管炎症的病因尚未完全明确,但基本的致病机制通常可以区分。免疫复合物在SLE炎症表现中的作用已得到认可,而其他血管炎中常见的抗中性粒细胞胞浆抗体等致病因素则不常见。由于各种大小的动脉和静脉血管发生炎症,多种临床特征是包括白塞病和SLE在内的几种结缔组织病的特点。认识到这些疾病中血管炎引起的疾病表现对治疗具有重要意义,对于降低发病率和死亡率可能至关重要。

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