Autoinflammatory Diseases

  • Min Shen
  • Di Wu
  • Qingping YaoEmail author


Autoinflammatory diseases (AIDs) are a relatively new group of rheumatic and inflammatory diseases. They are distinct from systemic autoimmune diseases and generally have no detectable autoantibodies. Among this group of disorders are hereditary monogenic periodic fever syndromes and polygenic/complex autoinflammatory diseases. In this chapter, we have discussed the pathophysiology, clinical phenotypes, diagnosis, and treatment of each of the classic monogenic autoinflammatory diseases and some complex autoinflammatory diseases.


Autoinflammatory disease Periodic fever syndrome Familial Mediterranean fever Cryopyrin-associated periodic syndrome TNF receptor-associated periodic fever syndrome Hyper-IgD syndrome Nucleotide-binding oligomerization domain 2 Blau syndrome Yao syndrome 


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Rheumatology and ImmunologyPeking Union Medical College HospitalBeijingChina
  2. 2.Division of Rheumatology, Allergy and Immunology, Department of MedicineStony Brook UniversityStony BrookUSA

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