Criopirinopatías: ¿qué son?, ¿cómo detectarlas? y ¿cuál es el enfoque de su tratamiento?
DOI:
https://doi.org/10.29176/2590843X.983Palabras clave:
síndromes periódicos asociados con criopirinas, síndrome CINCA, síndrome articular y cutáneo neurológico infantil crónico, criopirinopatía, síndrome autoinflamatorio familiar por frío, urticaria familiar por frío, enfermedad inflamatoria multisistémica de inicio en la infancia (IOMID), síndrome de Muckle-Wells, enfermedad inflamatoria multisistémica de inicio en el periodo neonatal (NOMID), proteína con dominio pirina 3 de la familia NLRResumen
Los síndromes periódicos asociados con criopirinas (Cryopyrin Associated Periodic Syndromes, CAPS), o criopirinopatías, son enfermedades raras que hacen parte de los síndromes autoinflamatorios, caracterizados por episodios de fiebre recurrente e inflamación sin etiología autoinmunitaria, neoplásica ni infecciosa. Entre estos síndromes, los CAPS incluyen tres entidades: el síndrome autoinflamatorio familiar por frío (Familial Cold Autoinflammatory Syndrome, FCAS), el síndrome de Muckle-Wells y la enfermedad multisistémica inflamatoria de inicio neonatal (Neonatal Onset Multisystem Inflammatory Disease, NOMID), también conocida como síndrome articular, cutáneo y neurológico crónico infantil (Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome).
Se han identificado mutaciones en el gen NLRP3 como etiología de estos síndromes, con un patrón de herencia autosómico dominante en la mayoría de los casos. El diagnóstico suele hacerse por sus manifestaciones clínicas, con apoyo de la biopsia de piel, y se confirma con el estudio genético. Hasta la fecha, el tratamiento basado en el bloqueo de la IL-1β, ha mostrado una mejoría satisfactoria en la mayoría de los pacientes.
Biografía del autor/a
Maribel Gallego
Médico, dermatólogo, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
Catalina Jaramillo
Médico, dermatólogo, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
Jaime Sierra
Médico, dermatólogo, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
Margarita María Velásquez
Médica dermatóloga, doctora en Ciencias Básicas Biomédicas con énfasis en Inmunología; profesora, Sección de Dermatología, Centro de Investigaciones Dermatológicas CIDERM, Facultad de Medicina, Universidad de Antioquia, Medellín, Colombia
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