This book, written by very well-known opinion leaders in the field, covers all aspects of periodic and non -periodic fevers, and related disorders. The expression refers to several different auto-inflammatory diseases, showing similar symptoms-the primary symptom being a recurrent fever for an infectious cause cannot be found. The opening chapters give some historical hints, explain the genetic basis of the disease and provide insights into the pathogenesis derived from recent experimental studies and guides the reader through classification and nomenclature. A large part of the book is then devoted to a detailed description of the specific related diseases and their clinical presentations, the disease course, and potential complications in both pediatric and adult patients. The advice regarding treatment is based on the best currently available evidence in this constantly evolving area. 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.

Autorentext
Rolando Cimaz is working as Head of the Pediatric Rheumatology Unit at Meyer Children's Hopital in Florence, and is an Associate professor of Pediartics at the University of Florence, Italy. He graduated from Milan University in Italy in 1987, is a specialist in Pediatrics (1991) and in Rheumatology (2003), and did his Fellowship in Pediatric Rheumatology in Dallas, Texas, in 1993-1994. He has been a Maitre de Conférences des Universités at Université Claude Bernard-Lyon I in Lyon, France from 2005 to 2007. His main interest is pediatric rheumatology, and has special expertise and scientific interest in osteoporosis, antiphospholipid antibodies, Kawasaki disease, synovial T cells, and neonatal lupus. Currently he is involved in many research projects, nationally and internationally. He has been in the Council of the Pediatric Rheumatology European Society from 1999 to 2005 (treasurer), and from 2014-present (Research Chair). He is a member of the American College of Rheumatology, the Italian Society of Pediatrics, and the French Society of Pediatric Inflammatory Diseases. He is co-author of more than 280 peer reviewed articles on PubMed, as well as more than 20 book chapters, all in the field of pediatric rheumatology. Editorial board member of international journals (Pediatric Rheumatology Online Journal, Lupus, Arthritis and Rheumatism) and co-editor of the book "Systemic Autoimmune Diseases in Pediatrics".

Inhalt

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.