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Systemic lupus erythematosus presenting with atypical hemolytic uremic syndrome: a case report and review of the literature

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Abstract

Systemic lupus erythematosus (SLE) can present with a diverse array of hematologic manifestations, among which atypical hemolytic uremic syndrome (aHUS) is a rare entity. SLE-triggered aHUS has significant morbidity and mortality without timely intervention, yet its frequency remains uncertain and optimal strategies for complement-directed therapies are largely expert-driven. We performed a comprehensive literature review and present a case of a 23-year-old female newly diagnosed with SLE/class IV lupus nephritis who developed aHUS that rapidly responded to the C5 antagonist, eculizumab. Review of the current literature identified forty-nine published cases of SLE with concurrent aHUS and revealed a predilection for aHUS in younger SLE patients, concurrent presentation with lupus nephritis, anti-dsDNA positivity, and complement system abnormalities. Over seventy percent of cases used eculizumab as complement-directed therapy with a trend towards faster time to improvement in laboratory parameters, though reported outcomes were highly variable. Early recognition of aHUS in SLE is pivotal in guiding appropriate therapeutic interventions, and prompt initiation of eculizumab may reduce the potential morbidity associated with plasmapheresis and additional immunosuppression. While eculizumab showcases promising results, its optimal timing and duration remain elusive. An understanding of a patients' complement genetics could aid management strategies, and ongoing research into complement-targeted therapies offers promising avenues for both SLE and aHUS treatment.

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Acknowledgements

We would like to thank Dr. Stephanie Thompson of Nephrology for providing ongoing clinical care for the patient.

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  1. Department of Medicine, University of Alberta, 8-130 Clinical Sciences Building, 11350 83 Avenue NW, Edmonton, AB, T6G 2G3, Canada

    Justin Smith, Varinder Hans, Elaine Yacyshyn & Azin Rouhi

  2. Department of Medicine, Division of Hematology, University of Alberta, Edmonton, Canada

    Monika Oliver

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All authors have completed the “Conflict of Interest Disclosure Form”. The authors JS, VH, EY, and AZ declare no financial relationships with any organizations that might have an interest in the submitted work in the previous three years, no other relationships or activities that could appear to have influenced the submitted work. MO declares advisory board honoraria from Alexion, Takeda, Sobi, Novartis, Sanofi, Roche, travel support from Alexion and Sobi, speaking honoraria from Alexion and Roche, and the following clinical trial involvement: Alexion (ALXN1210-301, ALXN2040-301); Apellis (APL2-307).

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Smith, J., Hans, V., Yacyshyn, E. et al. Systemic lupus erythematosus presenting with atypical hemolytic uremic syndrome: a case report and review of the literature. Rheumatol Int (2024). https://doi.org/10.1007/s00296-024-05558-9

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