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系统性红斑狼疮(SLE)和抗磷脂综合征(APS)中的肾上腺功能不全:系统评价。

Adrenal insufficiency in systematic lupus erythematosus (SLE) and antiphospholipid syndrome (APS): A systematic review.

机构信息

Department of Pediatrics, Yonsei University College of Medicine, Seoul, Republic of Korea; Division of Pediatric Nephrology, Severance Children's Hospital, Seoul, Republic of Korea.

Yonsei University College of Medicine, Seoul, Republic of Korea.

出版信息

Autoimmun Rev. 2019 Jan;18(1):1-8. doi: 10.1016/j.autrev.2018.06.014. Epub 2018 Nov 5.

Abstract

BACKGROUND

Adrenal insufficiency (AI) is associated with high morbidity and mortality. The aim of this systematic review was to enhance diagnostic approaches and summarize therapeutic strategies in the management of AI in patients with systematic lupus erythematosus (SLE) or antiphospholipid syndrome (APS).

METHODS

A literature search of PubMed and Medline databases was performed and 91 publications containing 105 cases were included for the final analysis.

RESULTS

The following frequency of clinical signs and symptoms was noted: abdominal pain (39.04%) was the leading symptom, followed by fever (33.33%), vomiting (23.81%), and nausea (19.05%). APS was present in 73%, SLE in 17% of the patients, while 2% had a diagnosis of both, SLE and APS. ACTH stimulation test (ACTHst) was performed in 18% of cases and 76.6% of them were unresponsive towards stimulation. Variable treatment approaches were used: hydrocortisone was most commonly used (38.09%), followed by fludrocortisone (26.67%), prednisolone (20.00%) and volume replacement treatment (11.43%), respectively.

CONCLUSIONS

This analysis highlights the importance of an early diagnosis and initiation of therapeutic management when AI is suspected. In line, signs and symptoms related to autoimmune diseases in patients with AI should be reviewed crtitically.

摘要

背景

肾上腺功能不全(AI)与高发病率和死亡率相关。本系统评价的目的是增强诊断方法,并总结系统性红斑狼疮(SLE)或抗磷脂综合征(APS)患者 AI 管理中的治疗策略。

方法

对 PubMed 和 Medline 数据库进行文献检索,纳入最终分析的 91 篇文献,共包含 105 例病例。

结果

注意到以下临床症状和体征的出现频率:腹痛(39.04%)为主要症状,其次是发热(33.33%)、呕吐(23.81%)和恶心(19.05%)。73%的患者存在 APS,17%的患者存在 SLE,而 2%的患者同时诊断为 SLE 和 APS。在 18%的病例中进行了促肾上腺皮质激素刺激试验(ACTHst),其中 76.6%的病例对刺激无反应。使用了不同的治疗方法:最常用的是氢化可的松(38.09%),其次是氟氢可的松(26.67%)、泼尼松龙(20.00%)和容量替代治疗(11.43%)。

结论

本分析强调了在怀疑 AI 时早期诊断和开始治疗管理的重要性。同样,应仔细审查 AI 患者与自身免疫性疾病相关的症状和体征。

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