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Case Reports
. 2022 May;42(4):901-903.
doi: 10.1007/s10875-022-01236-2. Epub 2022 Mar 19.

A Case of XIAP Deficiency Successfully Managed with Tadekinig Alfa (rhIL-18BP)

Ashley V Geerlinks  1 , Andrea M Dvorak  2 ; XIAP Deficiency Treatment Consortium
Collaborators, Affiliations
Case Reports

A Case of XIAP Deficiency Successfully Managed with Tadekinig Alfa (rhIL-18BP)

Ashley V Geerlinks et al. J Clin Immunol. 2022 May.
No abstract available

Keywords: IL-18; Immune dysregulation; Tadekinig alfa; XIAP deficiency.

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References

    1. Rigaud S, Fondanèche MC, Lambert N, Pasquier B, Mateo V, Soulas P, et al. XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome. Nature. 2006;444(7115):110–4. - DOI
    1. Wada T, Kanegane H, Ohta K, Katoh F, Imamura T, Nakazawa Y, et al. Sustained elevation of serum interleukin-18 and its association with hemophagocytic lymphohistiocytosis in XIAP deficiency. Cytokine. 2014;65(1):74–8. - DOI
    1. Arnold DE, Nofal R, Wakefield C, Lehmberg K, Wustrau K, Albert MH, et al. Reduced-intensity/reduced-toxicity conditioning approaches are tolerated in XIAP deficiency but patients fare poorly with acute GVHD. J Clin Immunol. 2022;42(1):36–45.
    1. Gabay C, Fautrel B, Rech J, Spertini F, Feist E, Kötter I, et al. Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still’s disease. Ann Rheum Dis. 2018;77(6):840–7. - PubMed
    1. Canna SW, Girard C, Malle L, de Jesus A, Romberg N, Kelsen J, et al. Life-threatening NLRC4-associated hyperinflammation successfully treated with IL-18 inhibition. J Allergy Clin Immunol. 2017;139(5):1698–701. - DOI

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