Journal of Clinical Pediatrics >
Diagnosis, treatment, and reflection on pediatric systemic lupus erythematosus complicated by thrombotic microangiopathy
Received date: 2024-12-30
Accepted date: 2025-01-15
Online published: 2025-02-12
A 12-year-old female SLE patient with lupus nephritis (class Ⅳ) and TMA was admitted to our hospital. After treatments including blood purification, methylprednisolone and CTX pulse therapy, even belimumab infusion, she still exhibited renal dysfunction and abnormal blood tests including decreased levels in platelet count, hemoglobin and fragmented red blood cells. Further tests showed normal activity of the von Willebrand factor-cleaving protease, negative antiphospholipid antibodies, and significantly elevated soluble c5-9 levels. After initiating treatment with eculizumab, the patient's condition improved. The patient remained stable during a three-month follow-up. This case provides clinicians with insights into the treatment of SLE complicated by TMA. Early identification of complement involvement and prompt initiation of eculizumab treatment can significantly improve the patient's long-term prognosis.
NI Jiajia , ZHU Yaju , JIN Jin , LI Jiaoyu , GUO Guimei . Diagnosis, treatment, and reflection on pediatric systemic lupus erythematosus complicated by thrombotic microangiopathy[J]. Journal of Clinical Pediatrics, 2025 , 43(2) : 150 -156 . DOI: 10.12372/jcp.2025.24e1397
| [1] | Aringer M. EULAR/ACR classification criteria for SLE[J]. Semin Arthritis Rheum, 2019, 49(3S): S14-S17. |
| [2] | Brocklebank V, Wood KM, Kavanagh D. Thrombotic microangiopathy and the kidney[J]. Clin J Am Soc Nephrol, 2018, 13(2): 300-317. |
| [3] | Bayer G, von Tokarski F, Thoreau B, et al. Etiology and outcomes of thrombotic microangiopathies[J]. Clin J Am Soc Nephrol, 2019, 14(4): 557-566. |
| [4] | Freedman SB, van de Kar N, Tarr PI. Shiga toxin-producing escherichia coli and the hemolytic-uremic syndrome[J]. N Engl J Med, 2023, 389(15): 1402-1414. |
| [5] | Mitsuiki N, Tamanuki K, Sei K, et al. Severe neonatal CMV infection complicated with thrombotic microangiopathy successfully treated with ganciclovir[J]. J Infect Chemother, 2017, 23(2): 107-110. |
| [6] | Timmermans S, Abdul-Hamid MA, Vanderlocht J, et al. Patients with hypertension-associated thrombotic microangiopathy may present with complement abnormalities[J]. Kidney Int, 2017, 91(6): 1420-1425. |
| [7] | National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents. The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents[J]. Pediatrics, 2004, 114(2Suppl 4th Report): 555-576. |
| [8] | Bamidele OF, Akintayo RO, Bojuwoye MO, et al. Throm-botic thrombocytopenic purpura as the first presentation in systemic lupus erythematosus[J]. Reumatologia, 2018, 56(4): 268-270. |
| [9] | Riancho-Zarrabeitia L, Martinez-Taboada V, Rua-Figueroa I, et al. Antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE) implies a more severe disease with more damage accrual and higher mortality[J]. Lupus, 2020, 29(12): 1556-1565. |
| [10] | Davin JC, van de Kar NC. Advances and challenges in the management of complement-mediated thrombotic microangiopathies[J]. Ther Adv Hematol, 2015, 6(4): 171-185. |
| [11] | Groot N, de Graeff N, Avcin T, et al. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative[J]. Ann Rheum Dis, 2017, 76(11): 1788-1796. |
| [12] | Fujimura Y, Matsumoto M. Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008[J]. Intern Med, 2010, 49(1): 7-15. |
| [13] | Schmidt CQ, Schrezenmeier H, Kavanagh D. Complement and the prothrombotic state[J]. Blood, 2022, 139(13): 1954-1972. |
| [14] | Kidney Disease: Improving Global Outcomes Glomerular Diseases Work G. KDIGO 2021 clinical practice guideline for the management of glomerular diseases[J]. Kidney Int, 2021, 100(4S): S1-S276. |
| [15] | Muff-Luett M, Sanderson KR, Engen RM, et al. Eculizumab exposure in children and young adults: indications, practice patterns, and outcomes-a Pediatric Nephrology Research Consortium study[J]. Pediatr Nephrol, 2021, 36(8): 2349-2360. |
| [16] | 中国罕见病联盟儿童非典型溶血尿毒综合征专业委员会. 中国儿童非典型溶血尿毒综合征诊治专家共识(2023版)[J]. 中华实用儿科临床杂志, 2023, 38(6): 401-412. |
| China Alliance for Rare Diseases Pediatric Atypical Hemolytic Uremic Syndrome Committee. Consensus on the diagnosis and treatment of atypical hemolytic uremic syndrome in Chinese children(2023 edition)[J]. Zhonghua Shiyong Erke Linchuang Zazhi, 2023, 38(6): 401-412. | |
| [17] | Kello N, Khoury LE, Marder G, et al. Secondary thrombotic microangiopathy in systemic lupus erythematosus and antiphospholipid syndrome, the role of complement and use of eculizumab: case series and review of literature[J]. Semin Arthritis Rheum, 2019, 49(1): 74-83. |
| [18] | Fakhouri F, Fila M, Hummel A, et al. Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study[J]. Blood, 2021, 137(18): 2438-2449. |
| [19] | Ardissino G, Possenti I, Tel F, et al. Discontinuation of eculizumab treatment in atypical hemolytic uremic syndrome: an update[J]. Am J Kidney Dis, 2015, 66(1): 172-173. |
| [20] | Merrill SA, Brittingham ZD, Yuan X, et al. Eculizumab cessation in atypical hemolytic uremic syndrome[J]. Blood, 2017, 130(3): 368-372. |
| [21] | Fakhouri F, Fila M, Provot F, et al. Pathogenic Variants in complement genes and risk of atypical hemolytic uremic syndrome relapse after eculizumab discontinuation[J]. Clin J Am Soc Nephrol, 2017, 12(1): 50-59. |
| [22] | Fakhouri F, Schwotzer N, Fremeaux-Bacchi V. How I diagnose and treat atypical hemolytic uremic syndrome[J]. Blood, 2023, 141(9): 984-995. |
| [23] | de Souza RM, Correa BHM, Melo PHM, et al. The treatment of atypical hemolytic uremic syndrome with eculizumab in pediatric patients: a systematic review[J]. Pediatr Nephrol, 2023, 38(1): 61-75. |
/
| 〈 |
|
〉 |
