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Autoinflammatory disease

Targeting G-CSF to treat autoinflammation

Nature Immunology volume 24, pages 736–737 (2023)Cite this article

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APLAID is a very rare autoinflammatory disease thought to be caused by mutations in PLCG2. A mouse model of APLAID recapitulates clinical features of the disease, and identifies a crucial function for G-CSF that might be targeted therapeutically.

Inflammation is a complex and strictly regulated defense mechanism, triggered to protect an organism from harmful stimuli. However, an excessive innate immune response can lead to self-directed inflammation and autoinflammatory disease — a distinct type of immune dysregulation first described nearly 25 years ago, in which typical features of broadly defined autoimmune diseases are often lacking1. Since then, advances in genetic sequencing have revealed that, in many cases, these diseases have a strong genetic background, with mutations in single genes being linked to autoinflammation. The current list of monogenic autoinflammatory disorders includes more than 40 conditions, extending our understanding of the range of genes, pathway perturbations and clinical features involved2. The monogenic nature of many autoinflammatory diseases has also prompted the generation of mouse models, which have provided important insights into autoinflammatory pathology3. In this issue of Nature Immunology, Mulazzani et al.4 describe a new mouse model of a monogenic autoinflammatory disease, showing that such models can identify unexpected drivers of inflammation and lead to feasible targets for therapeutic intervention.

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Fig. 1: A new model of autoinflammation and emerging treatment options.

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  1. Institute of Structural and Molecular Biology, Division of Biosciences, University College London, London, UK

    Tom D. Bunney & Matilda Katan

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  1. Tom D. Bunney
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  2. Matilda Katan
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Correspondence to Matilda Katan.

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The authors declare no competing interests.

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Bunney, T.D., Katan, M. Targeting G-CSF to treat autoinflammation. Nat Immunol 24, 736–737 (2023). https://doi.org/10.1038/s41590-023-01474-5

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  • DOI: https://doi.org/10.1038/s41590-023-01474-5

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