Abstract: SA-PO689
Clinical Characteristics and Risk Factors for Thrombotic Microangiopathy in Children with Lupus Nephritis
Session Information
- Pediatric Glomerular Disease
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1700 Pediatric Nephrology
Authors
- Yin, Lei, Shanghai children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
- Zhou, Wei, Shanghai children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
- Mao, Youying, Boston children''s hospital, Boston, United States
- Jin, Yanliang, Shanghai children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China
Background
Thrombotic microangiopathy (TMA) presents with the most severe clinical manifestations and high mortality. The aim of this study is to find the risk factors for TMA in children with lupus nephritis (LN) after analyzing their clinical characteristics.
Methods
We retrospectively reviewed the clinical data of 12 LN patients complicated with TMA hospitalized from January 2006 to December 2018．
Results
TMA occurred in 8.28% (12/145) of the hospitalized patients with LN. Four patients were boys and 8 patients were girls. One boy was 6-year-old and the other 11 patients were from 11 to 18-year-old. Their SLEDAI scores ranged from 14 to 31, 92% of them were in severe activity. Renal biopsy was performed in 11 patients, and the results of renal pathology showed all of them had type IV or type IV plus type V LN (type IV, n=8; type IV+V, n=3). All the patients were diagnosed with TMA within 1 week to 2 months after admission. At the beginning of the hospitalization, seven of them (58%) had both anemia and thrombocytopenia, while 5 patients (42%) only had moderate anemia. All of them had obvious hypocomplementemia. Especially in all of the 8 patients with first onset of SLE, there serum levels of C3 were lower than 0.18g/L and C4 were lower than 0.07g/L. Moreover, GFR of them were much lower than normal range. Most of the patients had heavy proteinuria (11/12) and positive anti-ds-DNA antibody (10/12). All of the patients accepted the treatment of methylprednisolone pulse therapy, ten of the them were treated with cyclophosphamide, five patients were treated with plasmapheresis (PE). After 6 months of treatment, five patients obtained complete response, five patients obtained partial response. One patient relieved after 3 times of PE but died during the fourth PE may because of intracranial hemorrhage. The other patient was unwilling to accept renal biopsy and the diagnosis of TMA was not confirmed until 2 months after onset. Although she was treated with PE for 10 times and two doses of Rituximab, she deteriorated continuously.
Conclusion
Children with LN complicated with TMA are mostly in severe disease activity, and the main pathological change of them is type IV LN. Anemia and extremely low level of serum complement occur simultaneously maybe the risk factors for TMA in children with LN whether they have thrombocytopenia or not.