Clinical characteristics and early recognition of thrombotic microangiopathy in children with systemic lupus erythematosus
10.3760/cma.j.issn.1673-4912.2021.10.006
- VernacularTitle:儿童系统性红斑狼疮并发血栓性微血管病的临床特点和早期识别
- Author:
Lei YIN
1
;
Youying MAO
;
Zhengyu ZHOU
;
Xiaoyu SHEN
;
Hua HUANG
;
Fei DING
;
Yanliang JIN
;
Wei ZHOU
Author Information
1. 上海交通大学医学院附属上海儿童医学中心肾脏科 200127
- Keywords:
Systemic lupus erythematosus;
Thrombotic microangiopathy;
Hypocomplementemia;
Children
- From:
Chinese Pediatric Emergency Medicine
2021;28(10):868-873
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical characteristics of children diagnosed with systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA)for early recognition.Methods:We retrospectively reviewed the clinical records of 14 SLE patients with TMA hospitalized at Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine from December 2005 to October 2020.Results:The incidence of TMA was 5.65%(14/248)of the hospitalized patients with SLE and 7.87%(14/178)of the hospitalized patients with lupus nephritis.Four patients were boys while ten patients were girls.One boy was six years old and other 13 patients were from 11 to 18 years old.Their SLEDAI scores ranged from 14 to 31, and all of them were severe activity.Renal biopsy of 11 patients during TMA course all revealed lupus nephritis(type Ⅳ, n=8; type Ⅳ+ Ⅴ, n=3). These 14 SLE children were diagnosed with TMA within 3 days to 2 months after admission.At the beginning of the hospitalization, only six patients had both anemia and thrombocytopenia, while eight patients only had moderate anemia.All of the patients had obvious hypocomplementemia.Especially in the patients with first onset of SLE without treatment, their serum levels of C3 were less than 0.17 g/L and C4 were less than 0.07 g/L.Moreover, glomerular filtration rates of these patients were lower than that in normal range.The follow-up time were 0.2-11.3 years(median time was 2.6 years). After treatment, six patients obtained complete remission, and five patients obtained partial remission.One patient had sudden death during the 4th plasmapheresis, and the other two patients deteriorated. Conclusion:Children with SLE and TMA are mostly in severe disease activity, and renal pathology is type Ⅳ lupus nephritis.The SLE children with anemia should be paid special attention to the level of serum complement whether they have thrombocytopenia or not.If the level of serum complements decrease obviously, glomerular filtration rates should be monitored closely and schistocytes should be searched repeatedly in the blood smears of the peripheral blood to facilitate the early recognition of TMA.
