无尿、突盲、意识障碍：一例不寻常的“成人斯蒂尔斯病”

乐偲; 刘岩; 秦岩; 焦禹豪; 苏龙翔; 徐军; 刘炜; 赵森; 杨璐; 王迁; 李宁宁; 陈有信; 韩冰; 张波; 李雪梅; 陈丽萌

doi:10.12376/j.issn.2097-0501.2022.01.009

无尿、突盲、意识障碍：一例不寻常的“成人斯蒂尔斯病”

doi: 10.12376/j.issn.2097-0501.2022.01.009

乐偲^{1, 2},
刘岩^{1, 2},
秦岩^{1, 2},
焦禹豪³,
苏龙翔⁴,
徐军⁵,
刘炜⁶,
赵森⁷,
杨璐⁸,
王迁⁹,
李宁宁¹⁰,
陈有信^{2, 11},
韩冰^{2, 12},
张波¹³,
李雪梅^{1, 2},
陈丽萌^{1, 2, ,}

1.
中国医学科学院北京协和医院肾内科，北京 100730
2.
中国医学科学院北京协和医院疑难重症及罕见病国家重点实验室，北京 100730
3.
中国医学科学院北京协和医院内科，北京 100730
4.
中国医学科学院北京协和医院重症医学科，北京 100730
5.
中国医学科学院北京协和医院急诊科，北京 100730
6.
中国医学科学院北京协和医院放射科，北京 100730
7.
中国医学科学院北京协和医院骨科，北京 100730
8.
中国医学科学院北京协和医院皮肤科，北京 100730
9.
中国医学科学院北京协和医院风湿免疫科，北京 100730
10.
中国医学科学院北京协和医院肿瘤科，北京 100730
11.
中国医学科学院北京协和医院眼科，北京 100730
12.
中国医学科学院北京协和医院血液科，北京 100730
13.
中国医学科学院北京协和医院药剂科，北京 100730

基金项目:

中国医学科学院医学与健康科技创新工程项目 2017-I2M-2-001

中国医学科学院医学与健康科技创新工程项目 2021-I2M-1-003

详细信息

通信作者:
陈丽萌，E-mail: chenlimeng@pumch.cn

中图分类号: R559;R692.9
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- 文章访问数: 540
- HTML全文浏览量: 281
- PDF下载量: 97
- 被引次数: 0
出版历程
- 收稿日期: 2021-12-13
- 修回日期: 2022-01-05

Anuria, Sudden Blindless, Confusion: A Case of Unusual "Adult Still Disease"

YUE Cai^{1, 2},
LIU Yan^{1, 2},
QIN Yan^{1, 2},
JIAO Yuhao³,
SU Longxiang⁴,
XU Jun⁵,
LIU Wei⁶,
ZHAO Sen⁷,
YANG Lu⁸,
WANG Qian⁹,
LI Ningning¹⁰,
CHEN Youxin^{2, 11},
HAN Bing^{2, 12},
ZHANG Bo¹³,
LI Xuemei^{1, 2},
CHEN Limeng^{1, 2
, ,}

1.
Department of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
2.
State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
3.
Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
4.
Intensive Care Unit, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
5.
Department of Emergency Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
6.
Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
7.
Department of Orthopedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
8.
Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
9.
Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
10.
Department of Oncology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
11.
Department of Ophthalmology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
12.
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
13.
Department of Pharmacy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

Funding:

CAMS Innovation Fund for Medical Sciences 2017-I2M-2-001

CAMS Innovation Fund for Medical Sciences 2021-I2M-1-003

More Information

Corresponding author: CHEN Limeng, E-mail: chenlimeng@pumch.cn

摘要

摘要: 一例青年女性，因发热、关节痛、皮疹诊断为成人斯蒂尔斯病，激素治疗过程中出现无尿、突盲、意识障碍，病情凶险，诊疗难度大。经多学科协作及团队共同讨论，考虑诊断为不典型溶血尿毒症综合征，经依库珠单抗治疗，获得良好的治疗效果。不典型溶血尿毒症综合征属于罕见疾病，依库珠单抗属于孤儿药。对该例患者的诊治体现了多学科协作在疑难罕见病诊疗过程中的重要意义。
- 成人斯蒂尔斯病 /
- 血栓性微血管病 /
- 不典型溶血尿毒症综合征 /
- 依库珠单抗
Abstract: A young female patient presented with fever, arthralgia, and rash was diagnosed with adults still's disease. When treated with glucocorticoid steroid, the above patient progressed to anuria, sudden, and confusion. After a teamwork involving different departments, the patient was finally diagnosed with atypical hemolytic uremic syndrome (aHUS) and treated with good outcome. aHUS is a rare disease, while Eculizumab is an orphan drug. The diagnosis and treatment of the patient reveals the importance of multidisciplinary team on the diagnosis and treatment of rare and difficult diseases.
- adult still's diasease /
- thrombotic microangiopathies /
- atypical hemolytic uremic syndrome /
- eculizumab
注释:

作者贡献：论文选题与设计：陈丽萌；论文整合与修订：陈丽萌；撰稿：乐偲，刘炜，赵森，杨璐，王迁，李宁宁，陈有信，韩冰，张波，陈丽萌；临床诊治：乐偲，刘岩，秦岩，焦禹豪，苏龙翔，徐军；科研经费来源及平台支持：李雪梅，陈丽萌。

利益冲突：所有作者均声明不存在利益冲突

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图 1 皮疹及皮肤组织病理学变化

A.右上肢屈侧皮疹; B.左下肢屈侧皮疹; C.腹壁皮疹; D~E.2021年9月27日皮肤活检，显示真皮浅中层血管周围和胶原纤维束间有淋巴细胞、中性粒细胞和较多嗜酸性粒细胞浸润

下载: 全尺寸图片幻灯片

参考文献(2)

[1]	Goodship TH, Cook HT, Fakhouri F, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference[J]. Kidney Int, 2017, 91: 539-551.
[2]	乐偲, 陈丽萌. 非典型溶血尿毒症综合征[M]//张抒扬. 中国第一批罕见病目录释义. 北京: 人民卫生出版社, 2018: 24-26.

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无尿、突盲、意识障碍：一例不寻常的“成人斯蒂尔斯病”

doi: 10.12376/j.issn.2097-0501.2022.01.009

通信作者: 陈丽萌，E-mail: chenlimeng@pumch.cn

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出版历程

Anuria, Sudden Blindless, Confusion: A Case of Unusual "Adult Still Disease"

Corresponding author: CHEN Limeng, E-mail: chenlimeng@pumch.cn

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通信作者:
陈丽萌，E-mail: chenlimeng@pumch.cn