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Interleukin-12 and Interleukin-23 Blockade in Leukocyte Adhesion Deficiency Type 1

2017; Massachusetts Medical Society; Volume: 376; Issue: 12 Linguagem: Inglês

10.1056/nejmoa1612197

1533-4406

Niki M. Moutsopoulos, Christa S. Zerbe, Teresa Wild, Nicolás Dutzan, Laurie Brenchley, Giovanni DiPasquale, Gülbû Uzel, Karen C. Axelrod, Andrea Lisco, Lucia Dora Notarangelo, George Hajishengallis, Luigi D. Notarangelo, Steven M. Holland,

Autoimmune Bullous Skin Diseases

A patient with leukocyte adhesion deficiency type 1 (LAD1) had severe periodontitis and an intractable, deep, nonhealing sacral wound. We had previously found a dominant interleukin-23-interleukin-17 signature at inflamed sites in humans with LAD1 and in mouse models of the disorder. Blockade of this pathway in mouse models has resulted in resolution of the immunopathologic condition. We treated our patient with ustekinumab, an antibody that binds the p40 subunit of interleukin-23 and interleukin-12 and thereby blocks the activity of these cytokines, inhibiting interleukin-23-dependent production of interleukin-17. After 1 year of therapy, our patient had resolution of his inflammatory lesions without serious infections or adverse reactions. Inhibition of interleukin-23 and interleukin-17 may have a role in the management of LAD1. (Funded by the National Institute of Allergy and Infectious Diseases and others.).