Síndromes autoinflamatorios monogénicos

Definition

Autoinflammatory diseases are characterized by apparently unprovoked generalized inflammation in the absence of infection or high titre autoantibodies [39]. The better defined diseases within this group are monogenic disorders, especially the hereditary periodic fever syndromes. These genetic diseases have provided insight in innate immune mechanisms and in the pathogenesis of autoinflammatory diseases in general. The common pathophysiologic feature for many of these disorders appears to be excessive interleukin- (IL-)1 signaling [No translation available]

5 subordinate codes

• 4A60.0Fiebre mediterránea familiar
• 4A60.1Síndromes periódicos asociados a la criopirina
• 4A60.2Síndrome periódico asociado al receptor 1 del factor de necrosis tumoral (FNT)
• 4B4YOtras enfermedades especificadas del sistema inmunitario
• 1H0ZAlgunas enfermedades infecciosas o parasitarias, sin especificación

Nearby in Trastornos autoinflamatorios

• 4A61Síndrome SAPHO
• 4A62Enfermedad de Behçet
• 4B4YOtras enfermedades especificadas del sistema inmunitario
• 1H0ZAlgunas enfermedades infecciosas o parasitarias, sin especificación