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系统性红斑狼疮:新观点。第2部分:皮肤和关节疾病、抗磷脂抗体综合征、妊娠与激素治疗、发病率与死亡率以及发病机制。

Systemic lupus erythematosus: emerging concepts. Part 2: Dermatologic and joint disease, the antiphospholipid antibody syndrome, pregnancy and hormonal therapy, morbidity and mortality, and pathogenesis.

作者信息

Boumpas D T, Fessler B J, Austin H A, Balow J E, Klippel J H, Lockshin M D

机构信息

National Institutes of Health, Bethesda, Maryland, USA.

出版信息

Ann Intern Med. 1995 Jul 1;123(1):42-53. doi: 10.7326/0003-4819-123-1-199507010-00007.

DOI:10.7326/0003-4819-123-1-199507010-00007
PMID:7762914
Abstract

PURPOSE

To review 1) advances in the pathogenesis, diagnosis, and management of dermatologic and joint disease and the antiphospholipid antibody syndrome in patients with systemic lupus erythematosus; 2) controversies related to pregnancy and hormonal therapy and to morbidity and mortality in these patients; and 3) current views on the pathogenesis of systemic lupus erythematosus.

DATA SOURCES AND STUDY SELECTION

Review of the English-language medical literature with emphasis on articles published within the last 5 years. More than 400 articles were reviewed.

DATA SYNTHESIS

Despite considerable overlap, cutaneous lesions specific to lupus erythematosus may be divided into subsets with distinct clinical, histologic, and immunofluorescent features. A recent short-term, prospective, uncontrolled trial found hydroxychloroquine and retinoids to be of similar efficacy in the treatment of cutaneous lupus erythematosus. Optimal treatment for patients with lupus and the anticardiolipin antibody syndrome remains to be defined; uncontrolled, retrospective, and treatment-withdrawal studies suggest that warfarin may be more protective than aspirin. Whether pregnancy induces lupus flares has not yet been established; existing data suggest both that it does and that it does not. Oral contraceptive use and postmenopausal estrogen replacement therapy appear not to cause clinical deterioration in patients with lupus. Recent studies have documented a substantial improvement in the survival of patients with systemic lupus erythematosus; they found 5-year survival rates of 90% or more and 10-year survival rates of more than 80%. Most data suggest that systemic lupus erythematosus results from the activation of self-reactive T cells and B cells by genetic or environmental factors.

CONCLUSIONS

The optimal treatment for dermatologic disease and the antiphospholipid antibody syndrome in patients with systemic lupus erythematosus remains unknown. Although mortality has decreased substantially, the morbidity related to the disease itself and to complications of therapy is still considerable. More studies are needed to further elucidate the effects of pregnancy on this condition and the pathogenetic mechanisms responsible for the development of this disease.

摘要

目的

回顾1)系统性红斑狼疮患者皮肤和关节疾病以及抗磷脂抗体综合征的发病机制、诊断和管理方面的进展;2)与这些患者的妊娠和激素治疗以及发病率和死亡率相关的争议;3)目前对系统性红斑狼疮发病机制的观点。

数据来源和研究选择

回顾英文医学文献,重点关注过去5年内发表的文章。共查阅了400多篇文章。

数据综合

尽管有相当多的重叠,但狼疮特异性皮肤病变可分为具有不同临床、组织学和免疫荧光特征的亚组。最近一项短期、前瞻性、非对照试验发现,羟氯喹和维甲酸在治疗皮肤型红斑狼疮方面疗效相似。狼疮和抗心磷脂抗体综合征患者的最佳治疗方法仍有待确定;非对照、回顾性和撤药研究表明,华法林可能比阿司匹林更具保护作用。妊娠是否会诱发狼疮发作尚未确定;现有数据表明既会诱发也不会诱发。口服避孕药的使用和绝经后雌激素替代疗法似乎不会导致狼疮患者的临床病情恶化。最近的研究记录了系统性红斑狼疮患者生存率的显著提高;他们发现5年生存率为90%或更高,10年生存率超过80%。大多数数据表明,系统性红斑狼疮是由遗传或环境因素激活自身反应性T细胞和B细胞所致。

结论

系统性红斑狼疮患者皮肤疾病和抗磷脂抗体综合征的最佳治疗方法尚不清楚。尽管死亡率已大幅下降,但与疾病本身及治疗并发症相关的发病率仍然相当高。需要更多研究来进一步阐明妊娠对这种疾病的影响以及导致该疾病发生的发病机制。

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