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Auto-Inflammatory Syndromes

Pathophysiology, Diagnosis, and Management

  • Book
  • © 2019

Overview

Editors:
  1. Petros Efthimiou

    1. New York University School of Medicine, New York, USA

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  • 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

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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.

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Table of contents (22 chapters)

  1. Front Matter

    Pages i-xix
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  2. Immunology of Auto-inflammatory Syndromes

    • Grant S. Schulert
    Pages 1-16

  3. Monogenic Autoinflammatory Diseases

    1. Front Matter

      Pages 17-17
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    2. Mevalonate Kinase Deficiency (MKD)/Hyperimmunoglobulin D Syndrome (HIDS)

      • Olga Petryna, Neha Purat
      Pages 19-27

    3. The TNF Receptor-Associated Autoinflammatory Syndrome (TRAPS)

      • Marco Gattorno
      Pages 29-38

    4. PAPA Syndrome and the Spectrum of PSTPIP1-Associated Inflammatory Diseases

      • Dirk Holzinger, Johannes Roth
      Pages 39-59

    5. Blau Syndrome

      • Rebecca Trachtman, Karen B. Onel
      Pages 61-67

    6. Deficiency of the Interleukin-1 Receptor Antagonist (DIRA)

      • Arturo Diaz
      Pages 69-83

    7. Deficiency of the Interleukin-36 Receptor Antagonist (DITRA) and Generalized Pustular Psoriasis

      • Arturo Diaz
      Pages 85-94

    8. Cryopyrin-Associated Periodic Syndromes (CAPS)

      • Marinka Twilt, Susanne M. Benseler
      Pages 95-109

    9. Familial Mediterranean Fever

      • Selcan Demir, İlkin Elif Günel, Seza Özen
      Pages 111-123

    10. Type I Interferonopathies: From Pathophysiology to Clinical Expression

      • Christina Maria Flessa, Evangelia Argiriou, Clio P. Mavragani
      Pages 125-145

    11. Deficiency of Adenosine Deaminase 2 (DADA2)

      • Andreas Reiff
      Pages 147-157

    12. Yao Syndrome

      • Qingping Yao
      Pages 159-166

  4. Polygenic Autoinflammatory Diseases

    1. Front Matter

      Pages 167-167
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    2. Systemic Juvenile Idiopathic Arthritis

      • Ricardo A. G. Russo, María Martha Katsicas
      Pages 169-191

    3. Macrophage Activation Syndrome (MAS)

      • Bella Mehta, Petros Efthimiou
      Pages 193-201

    4. Schnitzler Syndrome

      • Paolo Sfriso, Paola Galozzi
      Pages 203-212

    5. Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome

      • Ezgi Deniz Batu, Fatma Dedeoglu
      Pages 213-226

    6. Chronic Nonbacterial Osteomyelitis

      • Liza McCann, Michael W. Beresford, Gabriele Hahn, Christian M. Hedrich
      Pages 227-248
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Editors and Affiliations

  • New York University School of Medicine, New York, USA

    Petros Efthimiou

About the editor

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: https://doi.org/10.1007/978-3-319-96929-9

  • Publisher: Springer Cham

  • eBook Packages: Medicine, Medicine (R0)

  • Copyright Information: Springer Nature Switzerland AG 2019

  • Hardcover ISBN: 978-3-319-96928-2Published: 17 January 2019

  • eBook ISBN: 978-3-319-96929-9Published: 04 January 2019

  • Edition Number: 1

  • Number of Pages: XIX, 322

  • Number of Illustrations: 8 b/w illustrations, 31 illustrations in colour

  • Topics: Rheumatology, Immunology

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