Inherited anomalies of innate immune receptors in pediatric-onset inflammatory diseases

Pattern-recognition receptors (PRRs) can detect various pathogen-associated molecular patterns such as carbohydrates, nucleic acids or bacterial peptides and play a major role in both innate and adaptive immunity. In physiological conditions, the engagement of PRRs triggers the production of proinflammatory cytokines and promotes pathogen destruction. Inappropriate stimulation or defective regulation of PRR has been recently evidenced in several inherited inflammatory disorders. This new field of childhood-onset inflammatory diseases encompass the so-called type-I interferon-related diseases and autoinflammatory diseases.