© 2019

Auto-Inflammatory Syndromes

Pathophysiology, Diagnosis, and Management

  • Petros Efthimiou
  • Provides thorough, up-to-date information and clinical applications for auto-inflammatory syndromes

  • Includes basic science and pathophysiology of these diseases to inform clinical decision making

  • Covers rare and rarely documented diseases in adults and pediatric patients, including systemic idiopathic arthritis, still’s disease, papa syndrome, and sweets syndrome


Table of contents

  1. Front Matter
    Pages i-xix
  2. Grant S. Schulert
    Pages 1-16
  3. Monogenic Autoinflammatory Diseases

    1. Front Matter
      Pages 17-17
    2. Rebecca Trachtman, Karen B. Onel
      Pages 61-67
    3. Marinka Twilt, Susanne M. Benseler
      Pages 95-109
    4. Selcan Demir, İlkin Elif Günel, Seza Özen
      Pages 111-123
    5. Christina Maria Flessa, Evangelia Argiriou, Clio P. Mavragani
      Pages 125-145
    6. Andreas Reiff
      Pages 147-157
    7. Qingping Yao
      Pages 159-166
  4. Polygenic Autoinflammatory Diseases

    1. Front Matter
      Pages 167-167
    2. Ricardo A. G. Russo, María Martha Katsicas
      Pages 169-191
    3. Bella Mehta, Petros Efthimiou
      Pages 193-201
    4. Paolo Sfriso, Paola Galozzi
      Pages 203-212
    5. Liza McCann, Michael W. Beresford, Gabriele Hahn, Christian M. Hedrich
      Pages 227-248

About this book


This book provides an overview of auto-inflammatory syndromes, covering the underlying immune mechanisms that lead to their development, specific disease presentations, and clinical treatment guidelines. The book is divided into two sections, adult and pediatric, with chapters focusing on individuals diseases such as systemic arthritis, hyper-IgD, pap syndrome, idiopathic recurrent pericarditis, and familial Mediterranean fever. Chapters incorporate the most recent advances in disease pathophysiology and examine the underlying inductive and effector mechanisms and therapies that relate to each auto-inflammatory disorder at the genetic, molecular, cellular, and epidemiologic levels. The book also discusses the research behind auto-inflammatory disorders to offer detailed clinical guidelines regarding diagnostic techniques, treatment plans, and advice on how to best transition pediatric patients into adult treatment. This is an invaluable reference on auto-inflammatory syndromes for clinicians and researchers in pediatric and adult rheumatology and immunology.


auto-inflammatory syndromes systemic idiopathic arthritis still's disease CAPS familial Mediterranean fever TRAPS hyper-IgD syndrome

Editors and affiliations

  • Petros Efthimiou
    • 1
  1. 1.New York University School of MedicineNew YorkUSA

About the editors

Petros Efthimiou, MD

Clinical Professor of Medicine

New York University School of Medicine

New York, NY

Bibliographic information

  • Book Title Auto-Inflammatory Syndromes
  • Book Subtitle Pathophysiology, Diagnosis, and Management
  • Editors Petros Efthimiou
  • DOI
  • Copyright Information Springer Nature Switzerland AG 2019
  • Publisher Name Springer, Cham
  • eBook Packages Medicine Medicine (R0)
  • Hardcover ISBN 978-3-319-96928-2
  • eBook ISBN 978-3-319-96929-9
  • Edition Number 1
  • Number of Pages XIX, 322
  • Number of Illustrations 8 b/w illustrations, 31 illustrations in colour
  • Topics Rheumatology
  • Buy this book on publisher's site
Industry Sectors
Health & Hospitals
Internal Medicine & Dermatology