{"ok":true,"nota":{"id":"eba5e641e21942de","slug":"nuevas-pautas-internacionales-para-tratar-con-inhibidores-de-jak-a-pac-eba5e6","titulo":"Nuevas pautas internacionales para tratar con inhibidores de JAK a pacientes con errores genéticos inmunes","resumen":"Consenso de expertos europeos que, a partir de una revisión sistemática de la literatura y un método Delphi, establece recomendaciones para el uso de inhibidores de JAK en enfermedades raras del sistema inmune por ganancia de función. Hoy estos fármacos se recetan sin aprobación específica para estas condiciones.","fuente":"zenodo","fuenteNombre":"Zenodo · Investigación abierta","color":"#1682d4","autor":"Olbrich P., Fischer M., Deyà-Martinez Á.","url":"https://doi.org/10.1016/j.jaci.2026.05.005","imagen":"/hoy/portada/eba5e641e21942de","tema":"salud","subtemas":["inmunodeficiencias","inhibidores de jak","enfermedades raras","pautas clínicas","uso fuera de indicación"],"entidades":["European Society for Immunodeficiencies","ERN-RITA"],"formato":"dato","entretenimiento":false,"pubTs":"2026-10-21T13:20:29.000Z","indexTs":"2026-10-23T12:03:18.527Z","hash":"598cee4ab85ac3b8ddabae79074a9764fb43a517d3cebb32ec6ba70625993880","tsa":{"time":"Oct 23 12:04:07 2026 GMT","provider":"freetsa.org","sobre":"sello"},"stats":{"views":0,"likes":0,"clicks":0,"dwell":0},"tipo":"normal","registro":"hallazgo","porQueImporta":"Estos medicamentos se usan fuera de indicación en pacientes con enfermedades muy raras, sin una guía clara sobre dosis ni seguridad. Con este consenso, médicos de Argentina y otros países tienen un marco para decidir con más criterio, aunque la evidencia siga siendo limitada. Para las familias afectadas, significa menos improvisación en tratamientos que pueden cambiar la vida.","quienFirma":"Sociedad Europea de Inmunodeficiencias / Sociedad Europea de Trasplante de Médula Ósea (grupo de errores innatos) y ERN-RITA","confianza":"revisado","consulta":null},"sello":{"hash":"598cee4ab85ac3b8ddabae79074a9764fb43a517d3cebb32ec6ba70625993880","sello":"f72cf81f82c2ebc68a14517bc6e1efd4bfd968cefb9c9b96fbecd0c29cd39bb1","beacon":{"drand":{"cadena":"8990e7a9aaed2ffed73dbd7092123d6f289930540d7651336225dc172e51b2ce","ronda":6577533,"aleatorio":"c4aba0dd838f5aced1ee3ce73b5a8152f120dc443db083e10a01045483b7000d","tiempo":"2026-10-23T12:03:30.000Z"},"nist":{"cadena":2,"pulso":1995961,"salida":"F41B5D0D554B9AA93D86596521336D547B043E4F52A3C280F10F60EAE511ED1743D3E56670D4E53EB431734AC5B1B8CB5DCF96D1DB26F562D058604979415129","tiempo":"2026-10-23T12:03:00.000Z"},"noAntes":"2026-10-23T12:03:30.000Z"},"verificacion":{"drand":true,"nist":true},"tsa":{"time":"Oct 23 12:04:07 2026 GMT","provider":"freetsa.org","sobre":"sello"},"original":{"titulo":"Guidance on JAK inhibitor treatment for inborn errors of JAK-STAT signaling, 2026: International consensus statement on behalf of the European Society for Immunodeficiencies/European Society for Blood and Marrow Transplantation–Inborn Errors Working Party and ERN-RITA","descripcion":"Background: Inborn errors of immunity (IEI) associated with gain of function (GOF) in the Janus kinase (JAK)/signal transducer and activator of transcription (STAT) pathways (JAK-STAT-GOF IEI) are rare disorders characterized by immune dysregulation, autoimmunity, malignancy, and increased infection susceptibility. To date, no JAK inhibitors (JAKi) are licensed for these conditions, but they are frequently prescribed off-label, even though high-quality evidence and treatment guidelines remain limited. Objective: We developed evidence- and consensus-based recommendations for JAKi treatment in JAK-STAT-GOF IEI across all age groups on the basis of a systematic literature review and expert consensus. Methods: An international expert group conducted a systematic literature review and developed recommendations on JAKi treatment in JAK-STAT-GOF IEI using a modified Delphi approach. Recommendat","pubDate":"2026-10-21T13:20:29Z","url":"https://doi.org/10.1016/j.jaci.2026.05.005"},"indexado":"2026-10-23T12:03:18.527Z"}}