Eculizumab in Systemic Lupus Erythematosus Complicated with Thrombotic Microangiopathy

Authors

  • Azar Nickavar Professor of Pediatric Nephrology, Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran. Author
  • Nafiseh Mortazavi Assistant Professor of Pathology, Department of Pathology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran Author
  • Shima Salehi Assistant Professor of Pediatric Rheumatology, Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran. Author

DOI:

https://doi.org/10.61882/ijkd.3.03.9466

Keywords:

Systemic lupus erythematosus, Antiphospholipid antibody, Thrombotic microangiopathy, Eculizumab

Abstract

Thrombotic microangiopathy (TMA) is a rare but serious complication of systemic lupus erythematosus (SLE) in children, which is often resistant to different medical treatments. This is the report of a 13 years old female with a newly diagnosed SLE, complicated with a refractory course of SLE-TMA. Eculizumab had an effective therapeutic impact with clinical and laboratory improvement of TMA. 

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References

1. Kello N, Khoury LE , Marder G, Furie R, Zapantis E, Horowitz DL. Secondary thrombotic microangiopathy

in systemic lupus erythematosus and antiphospholipid syndrome, the role of complement and use of eculizumab: Case series and review of literature. Semin Arthritis Rheum 2019;49:74-83.

2. Holanda MI, Pôrto LC, Wagner T, Christiani LF, Palma LM. Use of eculizumab in a systemic lupus erythemathosus patient presenting thrombotic microangiopathy and heterozygous deletion in CFHR1-CFHR3. A case report and systematic review. Clin Rheumatol. 2017;36:2859-2867.

3. Cervera R, Rodríguez-Pintó I, Espinosa G. The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. J Autoimmun. 2018;92:1-11.

4. Aigner C, Gaggl M, Stemer G, Eder M, Böhmig G, Kain R, Prohászka Z et al. Eculizumab use in a tertiary care nephrology center: data from the Vienna TMA cohort. J Nephrol. 2022;35(2):451-461.

5. Sciascia S, Radin M, Bazzan M, Roccatello D. Novel diagnostic and therapeutic frontiers in thrombotic anti-phospholipid syndrome. Intern Emerg Med. 2017;12:1-7.

6. Islabão AG, Trindade VC, Henrique da Mota LM, Oliveira Andrade DC, Silva CA. Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects. Paediatr Drugs. 2022;24:13-27.

7. Alhamoud I, Freiberg SA. Successful Discontinuation of Eculizumab in a Pediatric Patient With Atypical Hemolytic Uremic Syndrome and Underlying Systematic Lupus Erythematosus. Cureus. 2022;18:e25117.

8. Ono M, Ohashi N, Namikawa A, Katahashi N, Ishigaki

S, Tsuji N et al. A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome. Intern Med. 2018;1:1617-1623.

9. Kim MJ, Lee H, Kim YH, Jin SY, Kim H-J, Oh D. Eculizumab therapy on a patient with co-existent lupus nephritis and C3 mutation-related atypical haemolytic uremic syndrome: a case report. BMC Nephrol. 2021,10:86.

10. Yamaguchi M, Mizuno M, Kitamura F, Iwagaitsu Sh, Nobata H, Kinashi H et al. Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory

to conventional treatment successfully treated with eculizumab. Front Med (Lausanne). 2023; 9:9:1097528.

11. Wright RD, Bannerman F, Beresford MW, Oni L.A systematic review of the role of eculizumab in systemic lupus erythematosus-associated thrombotic microangiopathy. BMC Nephro. 2020; 30;21:245.

12. Smith J, Hans V, Yacyshyn E, Rouhi A, Oliver M. Systemic lupus erythematosus presenting with atypical hemolytic uremic syndrome: a case report and review of the literature. Rheumatol Int. 2024; 44:2213-2225.

13. Sciascia S, Radin M, Yazdany J, Tektonidou M, Cecchi I, Roccatello D et al. Eculizumab in refractory catastrophic antiphospholipid syndrome: a case report and systematic review of the literature. Clin Exp Med. 2019; 19:281-288.

14. Chiara Pala Ch, Elisabetta Parenti E, Giuseppe Vizzini G, Davide Gianfreda D. Thrombotic microangiopathy due to primary antiphospholipid syndrome: successful treatment with eculizumab. J Nephrol. 2024; 37:1141-1145.

15. Marinho A, Alves JD, Fortuna J, Faria R, Almeida I, Alves G et al. Biological therapy in systemic lupus erythematosus, antiphospholipid syndrome, and Sjögren’s syndrome: evidence- and practice-based guidance. Front Immunol. 2023; 17:14:1117699.

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Published

2026-05-31

Data Availability Statement

The authors have not made their research available.

Issue

Section

CASE REPORT | Dialysis

How to Cite

Eculizumab in Systemic Lupus Erythematosus Complicated with Thrombotic Microangiopathy. (2026). Iranian Journal of Kidney Diseases, 20(03), 175-179. https://doi.org/10.61882/ijkd.3.03.9466

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