Periodic and Non-Periodic Fevers, 1st ed. 2020 Rare Diseases of the Immune System Series

The book is part of Springer?s series Rare Diseases of the Immune System, which presents recently acquired knowledge on pathogenesis, diagnosis, and therapy with the aim of promoting a more holistic approach to these conditions. Autoinflammatory diseases are hereditary disorders that are caused by single-gene defects in innate immune regulatory pathways and are characterized by a clinical and biological inflammatory syndrome in which there is limited, if any, evidence of autoimmunity. Periodic and Non-Periodic Fevers will be an invaluable source of up-to-date information for all practitioners involved in the care of patients with these disease.

Preface.- I From Pathogenesis to Classification.- 1 Historical notes on Autoinflammatory disorders.- 2 Pathogenesis:Genetic basis, Innate Immunity, Inflammosomes, Cytokines.- 3 Classification and nomenclature of Periodic and Non Periodic fevers.- II Specific diseases.- 4 Mevalonate Kinase Deficiency (MKD).- 5 Tumor necrosis factor receptors associated syndrome (TRAPS).- 6 Cryopyrin-Associated Periodic Syndromes (CAPS).- 7 Pyogenic Arthritis, Pyoderma gangrenosum and Acne Syndrome (PAPA).- 8 Adenosine deaminase syndrome 2 (ADA2).- 9 Interferonopathies.- 10 Inflammatory bone disease.- 11 Amyloidosis.- 12 Dermatologic autoinflammatory diseases.- 13 Autoinflammatory diseases with immunodeficiency.- 14 Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA).- 15 Proteasome. related disorders.- 16 Chronic Non-bacterial osteomyelitis.- 17 Systemic JIA and adult onset Still disease.- 18 Behcet disease.- 19 Recurrent idiopathic pericarditis.- 20 Schnitzler syndrome .- Subject Index.

Written by internationally known experts in the field of Periodic and Non-Periodic Fevers

State of the art contents in disease pathogenesis

Wide readership, the book is addressed to specialists in rare disorders