Publication:
SARS-CoV-2 infection in a pediatrics STAT1 GOF patient under Ruxolitinib therapy-a matter of balance?

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Date

2021-06-09

Authors

Guisad-Hernandez, Paloma
Blanco-Lobo, Pilar
Villaoslada, Isabel
de-Felipe, Beatriz
Lucena, Jose M
Martin-Gutierrez, Guillermo
Castro, María Jose
Gutierrez-Valencia, Alicia
Sanchez-Codez, Maria Isabel
Gaboli, Mirella

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Springer New York LLC
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Abstract

Recently, 94 inborn errors of immunity (IEI) patients suffering from COVID-19 have been described, overall demonstrating a mild phenotype [1] although more severe disease manifestations have been suggested for patients with alterations in the interferon (IFN) signaling pathway, including auto-antibodies against type I IFN [2]. Patients with STAT1 GOF mutations show a complex and often severe phenotype, combining an increased susceptibility of fungal, (myco-) bacterial and viral infections as well as autoimmune and autoinflammatory manifestations [3]. Characteristically, in response to type I and type II IFN stimulation, these patients show STAT1 hyperphosphorylation [3, 4]. Whether in the context of SARS-CoV-2 infection, the hyperactivation of the IFN-JAK1/2-STAT1 pathway would be protective (antiviral effect) or deleterious (hyperinflammation) is unclear. Ruxolitinib (a selective JAK1/2 inhibitor) has been successfully used in STAT1 GOF patients controlling many disease manifestations [5] and also resulted in improved pulmonary function and faster recovery from lymphopenia in previously healthy individuals suffering from severe COVID-19

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MeSH Terms

Child
Female
Humans
Janus Kinases
Mutation
Nitriles
Pyrimidines
SARS-CoV-2
Severity of Illness Index

DeCS Terms

COVID-19
Mutación
SARS-CoV-2
Linfopenia
Virosis
Interferones
Insuficiencia multiorgánica
Antineoplásicos inmunológicos
Diabetes mellitus tipo 2

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Keywords

COVID-19, Interferon-Stimulated Gene Factor 3, Pyrazoles, COVID-19 Drug Treatment

Citation

Guisado Hernández P, Blanco Lobo P, Villaoslada I, de Felipe B, Lucena JM, Martín Gutierrez G, et al. SARS-CoV-2 infection in a pediatrics STAT1 GOF patient under Ruxolitinib therapy-a matter of balance? J Clin Immunol. 2021 Oct;41(7):1502-1506.