Auto-inflammatory diseases are a group of rare hereditary periodic diseases that have become increasingly recognized in the last decade. In the past, they were also called “hereditary periodic fever”, “auto-inflammatory fever syndrome”, and so on. In fact, as a member of the family of auto-inflammatory diseases, familial Mediterranean fever (FMF) was first reported in the early 20th century, but the actual discovery of the causative gene, the Mediterranean fever gene MEFV, took about a century and was only reported in 1997. This new understanding of the ancient disease of familial Mediterranean fever has opened the curtain on auto-inflammatory diseases. Auto-inflammatory diseases are systemic inflammatory diseases caused by mutations in genes that alter their encoded proteins, leading to dysregulation of intrinsic immunity. Due to their genetic nature, most of them have an early onset, from a few hours after birth to adolescence, with a few patients developing them in adulthood. Therefore, pediatricians have more access to these patients. However, since some patients can develop the disease in adulthood or continue to develop it in adulthood, non-pediatricians are also faced with the challenge of treating this group of patients. The majority of patients present with sudden onset of periodic fever, rash, pluritis, lymph node enlargement and arthritis, with elevated acute phase reactants such as blood sedimentation and C-reactive protein. In the asymptomatic interictal phase, the patient’s health and growth are as normal, and the acute phase reactants are completely normal. Because the symptoms are multisystemic and the manifestations are not specific, patients often travel to and from various departments, such as infection, dermatology, immunology, hematology, etc., because of unexplained fever, rash or arthritis. Many patients are chronically misdiagnosed or underdiagnosed due to the lack of diagnostic tools and the lack of awareness among clinicians. Autoinflammatory diseases comprise a large group of diseases, many of which are rare or uncommon, and the names of which are not familiar or even very awkward, so some acronyms are often used to simplify their designation. Auto-inflammatory diseases include both monogenic diseases (which simply mean that they have a family history of development and are hereditary) and polygenic diseases (which simply mean that their development is not associated with a specific gene, but is influenced by multiple genes and external factors). However, the vast majority of auto-inflammatory diseases are single-gene disorders. Monogenic genetic disorders in auto-inflammatory diseases include four major groups of disorders: (1) periodic fever: three main types: (1) familial Mediterranean fever; (2) mevalonate kinase deficiency, once known as high IgD syndrome (HIDS); (3) tumor necrosis factor receptor-associated periodic syndrome. 2, cryopyrin-related periodic syndrome: including three diseases: (1) familial cold auto-inflammatory syndrome; (2) Muckle-Wells syndrome; (3) chronic infantile neurocutaneous joint syndrome. 3, granulomatous disease: mainly refers to Blau syndrome. 4, septic disease: including three diseases: (1) septic aseptic arthritis – gangrenous sepsis – acne; (2) Majeed syndrome; (3) interleukin 1 receptor antagonist deficiency. Currently, most scholars believe that auto-inflammatory diseases also include some polygenic diseases, such as adult Still disease (AOSD), systemic juvenile idiopathic arthritis (sJIA), leukoarthrosis, and Crohn’s disease. More new auto-inflammatory diseases are being recognized and named, such as: nucleotide oligomerization domain 2 (NOD2)-associated auto-inflammatory diseases, NOD-like receptor protein 12 (NLRP12)-associated auto-inflammatory diseases, etc.