【依珠单抗治疗系统性红斑狼疮合并血栓性微血管病变1例】。

Q3 Medicine
Heng Liu, Pan-Li Liao, Xiao-Wen Wang
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引用次数: 0

摘要

患者为女童,年龄10岁零10个月,身体虚弱,面色苍白,首发表现为皮疹。患者表现为贫血、血小板减少、血尿蛋白尿伴肾功能不全、补体不足、多血清炎、抗核抗体、抗dsdna抗体阳性。该女孩最初被诊断为系统性红斑狼疮和狼疮肾炎。她对甲基强的松龙脉冲治疗、静脉注射免疫球蛋白和治疗性血浆交换反应不佳。患者有持续性贫血、血小板减少、肾功能异常、乳酸脱氢酶升高、补体因子H和I降低、C3转化酶抗体升高、ADAMTS13活性正常。她被诊断为继发于系统性红斑狼疮的补体介导的溶血性血栓性微血管病。患者在接受两剂eculizumab(每剂600mg)治疗后病情得到改善。系统性红斑狼疮合并血栓性微血管病变患者病程严重,预后差;因此,早期识别和积极干预对改善预后至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Eculizumab in the treatment of systemic lupus erythematosus complicated by thrombotic microangiopathy: a case report].

The patient was a girl aged 10 years and 10 months, with weakness, pale complexion, and rash as the initial presentation. She had the manifestations of anemia, thrombocytopenia, hematuria-proteinuria with renal insufficiency, hypocomplementemia, polyserositis, and positive anti-nuclear antibody and anti-dsDNA antibody. The girl was initially diagnosed with systemic lupus erythematosus and lupus nephritis. She demonstrated a suboptimal response to methylprednisolone pulse therapy, intravenous immunoglobulin administration, and therapeutic plasma exchange. She had persistent anemia, thrombocytopenia, abnormal renal function, elevated lactate dehydrogenase, decreased complement factors H and I, increased antibodies to C3 converting enzyme, and normal ADAMTS13 activity. She was diagnosed with complement-mediated hemolytic thrombotic microangiopathy secondary to systemic lupus erythematosus. The patient's condition improved after treatment with two doses of eculizumab (600 mg per dose). Patients with systemic lupus erythematosus complicated by thrombotic microangiopathy often have a severe disease course and poor prognosis; therefore, early recognition and aggressive intervention are crucial for improving outcomes.

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来源期刊
中国当代儿科杂志
中国当代儿科杂志 Medicine-Pediatrics, Perinatology and Child Health
CiteScore
1.50
自引率
0.00%
发文量
5006
期刊介绍: The Chinese Journal of Contemporary Pediatrics (CJCP) is a peer-reviewed open access periodical in the field of pediatrics that is sponsored by the Central South University/Xiangya Hospital of Central South University and under the auspices of the Ministry of Education of China. It is cited as a source in the scientific and technological papers of Chinese journals, the Chinese Science Citation Database (CSCD), and is one of the core Chinese periodicals in the Peking University Library. CJCP has been indexed by MEDLINE/PubMed/PMC of the American National Library, American Chemical Abstracts (CA), Holland Medical Abstracts (EM), Western Pacific Region Index Medicus (WPRIM), Scopus and EBSCO. It is a monthly periodical published on the 15th of every month, and is distributed both at home and overseas. The Chinese series publication number is CN 43-1301/R;ISSN 1008-8830. The tenet of CJCP is to “reflect the latest advances and be open to the world”. The periodical reports the most recent advances in the contemporary pediatric field. The majority of the readership is pediatric doctors and researchers.
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