Periodic and Non-Periodic Fevers

1. Front Matter

   Pages i-x

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2. Periodic and Non-Periodic Fevers

   • Marco Gattorno

   Pages 1-20

3. Fever of Unknown Origin

   • Estíbaliz Iglesias, Antoni Noguera-Julian, Laia Alsina, Jordi Antón

   Pages 21-26

4. PFAPA: Periodic Fever, Aphthous Ulceration, Pharyngitis, Adenitis

   • Jeffrey Chaitow

   Pages 27-44

5. Kawasaki Disease

   • Rakesh Kumar Pilania, Surjit Singh

   Pages 45-63

6. Systemic-Onset Juvenile Idiopathic Arthritis

   • María Martha Katsicas, Ricardo A. G. Russo

   Pages 65-91

7. Adult-Onset Still’s Disease

   • Stéphane Mitrovic, Eugen Feist, Bruno Fautrel

   Pages 93-132

8. Recurrent Pericarditis

   • Antonio Brucato, Anna Valenti, Silvia Maestroni

   Pages 133-146

9. Chronic Nonbacterial Osteomyelitis

   • Andrea Taddio, Serena Pastore

   Pages 147-160

10. Behçet Disease

    • Erdal Sag, Yelda Bilginer, Seza Ozen

    Pages 161-175

11. Extraintestinal Manifestations of Inflammatory Bowel Disease

    • Skirmante Rusoniene, Vaidotas Urbonas, Tadej Avcin

    Pages 177-213

12. Castleman Disease

    • Dale M. Kobrin, David C. Fajgenbaum

    Pages 215-228

13. Mevalonate Kinase Deficiency

    • Brigitte Bader-Meunier

    Pages 229-234

14. Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)

    • Charalampia Papadopoulou, Helen J. Lachmann

    Pages 235-245

15. Type I Interferonopathies

    • Cécile Frachette, Rolando Cimaz, Alexandre Belot

    Pages 247-256

16. Rare Monogenic Causes of Periodic Fevers

    • Serife Gulmus, Roberta Berard, Erkan Demirkaya

    Pages 257-272

17. Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne (PAPA) Syndrome

    • Antonella Insalaco

    Pages 273-279

18. Back Matter

    Pages 281-283

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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.

• Autoinflammatory sindrome
• Recurrent fevers
• Monogenic disorders
• Innate immunity
• Genetic disorders
• Amyloidosis
• Proteasome related disorders
• Interferonopathies

• Department of Clinical Sciences and Community Health, University of Milano, Milano, Italy

  Rolando Cimaz

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".

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