Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic Microangiopathy

Introduction: Thrombotic microangiopathy (TMA) is a rare but severe complication of systemic lupus erythematosus (SLE); however, the overall patient and renal survival rates of patients with SLE-associated TMA (SLE-TMA) remain controversial. This study aimed to evaluate the prognosis of and identify...

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Main Authors: Yixuan Gai, Mucong Li, Zhenye Zhu, Yangzhong Zhou, Can Huang, Wei Bai, Jiuliang Zhao, Mengtao Li
Format: Article
Language:English
Published: Elsevier 2025-07-01
Series:Kidney International Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2468024925002463
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author Yixuan Gai
Mucong Li
Zhenye Zhu
Yangzhong Zhou
Can Huang
Wei Bai
Jiuliang Zhao
Mengtao Li
author_facet Yixuan Gai
Mucong Li
Zhenye Zhu
Yangzhong Zhou
Can Huang
Wei Bai
Jiuliang Zhao
Mengtao Li
author_sort Yixuan Gai
collection DOAJ
description Introduction: Thrombotic microangiopathy (TMA) is a rare but severe complication of systemic lupus erythematosus (SLE); however, the overall patient and renal survival rates of patients with SLE-associated TMA (SLE-TMA) remain controversial. This study aimed to evaluate the prognosis of and identify potential prognostic factors in patients with SLE-TMA. Methods: Literature search was performed in MEDLINE, EMBASE, Web of Science, Cochrane Central Register of Controlled Trial, CINAHL, PsycINFO, Scopus, CNKI, and Sinomed for studies published before December 2023. Studies that encompassed no less than 5 patients with SLE-TMA and followed well-recognized diagnostic criteria were included, whereas studies with a median follow-up < 6 months or that provided incomplete outcomes were excluded. Results: Fourteen eligible studies encompassing a total of 446 patients with SLE-TMA were included in the meta-analysis. The pooled 1-, 3-, and 5-year patient survival rates were 83% (95% confidence interval [CI]: 0.65–0.96), 81% (95% CI: 0.59–0.96), and 77% (95% CI: 0.52–0.95), respectively. The pooled 1-, 3-, and 5-year renal survival rates were 85% (95% CI: 0.81–0.89), 75% (95% CI: 0.63–0.85), and 56% (95% CI: 0.31–0.79). The pooled 1-, 3-, and 5-year renal relative risks in patients with SLE-TMA compared with patients with SLE without TMA were 4.01 (95% CI: 2.10–7.65), 2.53 (95% CI: 1.83–3.51), and 2.58 (95% CI: 2.01–3.29). Univariate Cox regression indicated that high baseline serum creatinine (SCr) levels, high renal chronicity index and acute hemodialysis were independent prognostic factors of renal survival. Conclusion: Patients with SLE-TMA exhibit lower long-term patient and renal survival rates. High baseline SCr levels, high renal chronicity index or a need for acute hemodialysis at baseline are associated with poorer renal outcomes.
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spelling doaj-art-9198fc4ed43847bea045bb3a39a7f7db2025-08-20T02:35:19ZengElsevierKidney International Reports2468-02492025-07-011072243225410.1016/j.ekir.2025.04.022Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic MicroangiopathyYixuan Gai0Mucong Li1Zhenye Zhu2Yangzhong Zhou3Can Huang4Wei Bai5Jiuliang Zhao6Mengtao Li7Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Beijing, China; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology, Beijing, ChinaDepartment of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Beijing, China; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology, Beijing, ChinaDepartment of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Beijing, China; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology, Beijing, ChinaDepartment of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Beijing, China; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology, Beijing, ChinaDepartment of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Beijing, China; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology, Beijing, ChinaDepartment of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Beijing, China; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology, Beijing, ChinaDepartment of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Beijing, China; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology, Beijing, China; Correspondence: Mengtao Li and Jiuliang Zhao, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College &amp; Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science &amp; Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science &amp; Technology, No.1 Shuaifuyuan, Wangfujing Ave, 100730, Beijing, China.Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science and Technology, Beijing, China; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China; State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science and Technology, Beijing, China; Correspondence: Mengtao Li and Jiuliang Zhao, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College &amp; Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science &amp; Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science &amp; Technology, No.1 Shuaifuyuan, Wangfujing Ave, 100730, Beijing, China.Introduction: Thrombotic microangiopathy (TMA) is a rare but severe complication of systemic lupus erythematosus (SLE); however, the overall patient and renal survival rates of patients with SLE-associated TMA (SLE-TMA) remain controversial. This study aimed to evaluate the prognosis of and identify potential prognostic factors in patients with SLE-TMA. Methods: Literature search was performed in MEDLINE, EMBASE, Web of Science, Cochrane Central Register of Controlled Trial, CINAHL, PsycINFO, Scopus, CNKI, and Sinomed for studies published before December 2023. Studies that encompassed no less than 5 patients with SLE-TMA and followed well-recognized diagnostic criteria were included, whereas studies with a median follow-up < 6 months or that provided incomplete outcomes were excluded. Results: Fourteen eligible studies encompassing a total of 446 patients with SLE-TMA were included in the meta-analysis. The pooled 1-, 3-, and 5-year patient survival rates were 83% (95% confidence interval [CI]: 0.65–0.96), 81% (95% CI: 0.59–0.96), and 77% (95% CI: 0.52–0.95), respectively. The pooled 1-, 3-, and 5-year renal survival rates were 85% (95% CI: 0.81–0.89), 75% (95% CI: 0.63–0.85), and 56% (95% CI: 0.31–0.79). The pooled 1-, 3-, and 5-year renal relative risks in patients with SLE-TMA compared with patients with SLE without TMA were 4.01 (95% CI: 2.10–7.65), 2.53 (95% CI: 1.83–3.51), and 2.58 (95% CI: 2.01–3.29). Univariate Cox regression indicated that high baseline serum creatinine (SCr) levels, high renal chronicity index and acute hemodialysis were independent prognostic factors of renal survival. Conclusion: Patients with SLE-TMA exhibit lower long-term patient and renal survival rates. High baseline SCr levels, high renal chronicity index or a need for acute hemodialysis at baseline are associated with poorer renal outcomes.http://www.sciencedirect.com/science/article/pii/S2468024925002463pooled survival rateprognostic factorsystemic lupus erythematosusthrombotic microangiopathy
spellingShingle Yixuan Gai
Mucong Li
Zhenye Zhu
Yangzhong Zhou
Can Huang
Wei Bai
Jiuliang Zhao
Mengtao Li
Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic Microangiopathy
Kidney International Reports
pooled survival rate
prognostic factor
systemic lupus erythematosus
thrombotic microangiopathy
title Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic Microangiopathy
title_full Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic Microangiopathy
title_fullStr Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic Microangiopathy
title_full_unstemmed Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic Microangiopathy
title_short Systematic Review of the Outcomes and Prognostic Factors of Patients With Systemic Lupus Erythematosus-Associated Thrombotic Microangiopathy
title_sort systematic review of the outcomes and prognostic factors of patients with systemic lupus erythematosus associated thrombotic microangiopathy
topic pooled survival rate
prognostic factor
systemic lupus erythematosus
thrombotic microangiopathy
url http://www.sciencedirect.com/science/article/pii/S2468024925002463
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