In addition to FMF, Pyrin mutations have been found in patients with Pyrin-associated autoinflammation with neutrophilic dermatosis (PAAND), Pyrin-associated dominant disease (PADD), pyrogenic arthritis, pyoderma gangrenosum and acne (PAPA), mevalonate kinase deficiency (MKD), and autoinflammatory periodic fever, immunodeficiency and thrombocytopenia (PFIT) [542, 556–558]. Here, MEFV is linked to immunodeficiency disease.