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Description of therapeutic strategies in severe systemic lupus erythematosus-associated immune thrombocytopenia: a retrospective cohort study of response and relapse

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Abstract

Objectives

To describe the response and relapse of severe thrombocytopenia in patients with systemic lupus erythematosus (SLE) with different treatments.

Method

We performed a retrospective cohort study, which included SLE patients who were hospitalized for thrombocytopenia of less than 30,000/µL platelets, from January 2012 to December 2021. Demographic and clinical information was obtained from clinical records. Kaplan–Meier and logrank test were performed.

Results

Forty-seven patients, mostly women (83%) with a median age of 31 years, were included in the study. Eight patients (17%) relapsed within a median period of 35.7 weeks. Initial acute treatment with prednisone at 1 mg/kg/day was as effective as glucocorticoid pulses. However, induction treatment with cyclophosphamide (CYC) had the lowest remission rate (43%, p = 0.034). There was no significant difference in relapse-free survival (RFS) among the acute glucocorticoid treatments. CYC induction was associated with lower RFS compared to rituximab (RTX) (CYC 43.6 weeks vs. RTX 51.8 weeks, p = 0.040) or azathioprine (AZA) (CYC 43.6 weeks vs. AZA 51.2 weeks, p = 0.024). Administration of antimalarials was associated with longer RFS (51.6 weeks vs. 45.0 weeks, p = 0.021). Factors such as antiphospholipid syndrome, IgG anti-β2 glycoprotein I positivity, renal and additional hematologic SLE activity during follow-up significantly reduced RFS.

Conclusions

Despite similar response of acute glucocorticoid regimens, induction therapy with AZA or RTX resulted in a longer RFS compared to CYC. Adding an antimalarial also improved RFS. Our study provides evidence that may help develop better treatment strategies for severe thrombocytopenia in SLE patients.

Key Points

• Induction therapy with azathioprine or rituximab provided longer relapse-free survival in SLE thrombocytopenia compared with cyclophosphamide.

• Antimalarial administration was associated with longer relapse-free survival in SLE thrombocytopenia.

• Antiphospholipid syndrome, IgG anti-β2 glycoprotein I positivity, as well as renal and additional hematologic SLE activity during follow-up, decreased relapse-free survival.

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Data availability

Data are available if requested with Dr. Cimé at cime.erik@gmail.com.

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Acknowledgements

The authors acknowledge to all the patients included in the study.

Funding

No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.

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Authors and Affiliations

  1. Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico

    Erik Cimé-Aké, Martha Ramírez-Alemón & Marina Rull-Gabayet

  2. Department of Medical Education, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico

    Ana Barrera-Vargas

  3. Department of Hematology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico

    Roberta Demichelis-Gómez

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  1. Erik Cimé-Aké
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  2. Ana Barrera-Vargas
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by E. C-A and M. R-A. The first draft of the manuscript was written by E. C-A and M. R-G, A. B-V and R. D-G made contributions to subsequent versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Marina Rull-Gabayet.

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Ethical approval

The study involved human subjects and was approved by the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán Research and Ethics Committee with registration number IRE-4027–22-22–1. This is a retrospective cohort study and the information analyzed comes from a historical data registry, so the requirement for written informed consent was waived by the Committee.

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The authors declare that there is no conflict of interest.

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Part of the data in this manuscript were presented at the ACR Convergence 2022 and were published as an abstract in the corresponding supplementary issue: Abstract Supplement ACR Convergence 2022. Arthritis Rheumatol 74(S9):730-732. https://doi.org/10.1002/art.42355.

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Cimé-Aké, E., Barrera-Vargas, A., Demichelis-Gómez, R. et al. Description of therapeutic strategies in severe systemic lupus erythematosus-associated immune thrombocytopenia: a retrospective cohort study of response and relapse. Clin Rheumatol (2024). https://doi.org/10.1007/s10067-024-07031-1

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