Pyrin-associated autoinflammatory diseases (PAADs) are a group of IRDs caused by the over-activation of the pyrin inflammasome by MEFV gene mutation, which leads to a series of symptoms, such as familial Mediterranean fever (FMF), pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND), chronic aseptic osteomyelitis, and ulcerative dermatitis. The gene discussed is MEFV; the disease is respiratory distress syndrome in premature infants.