Efthimiou Petros (ed.) Auto-Inflammatory Syndromes: Pathophysiology, Diagnosis, and Management

Springer, 2019. — 324 p. — ISBN: 978-3-319-96928-2This 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.Immunology of Auto-inflammatory Syndromes
Monogenic Autoinflammatory Diseases
Mevalonate Kinase Deficiency (MKD)/Hyperimmunoglobulin D Syndrome (HIDS)
The TNF Receptor-Associated Autoinflammatory Syndrome (TRAPS)
PAPA Syndrome and the Spectrum of PSTPIP1-Associated Inflammatory Diseases
Blau Syndrome
Deficiency of the Interleukin-1 Receptor Antagonist (DIRA)
Deficiency of the Interleukin-36 Receptor Antagonist (DITRA) and Generalized Pustular Psoriasis
Cryopyrin-Associated Periodic Syndromes (CAPS)
Familial Mediterranean Fever
Type I Interferonopathies: From Pathophysiology to Clinical Expression
Deficiency of Adenosine Deaminase 2 (DADA2)
Yao Syndrome
Polygenic Autoinflammatory Diseases
Systemic Juvenile Idiopathic Arthritis
Macrophage Activation Syndrome (MAS)
Schnitzler Syndrome
Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome
Chronic Nonbacterial Osteomyelitis
Improving the Transition from Pediatric to Adult Care for Adolescents and Young Adults with Autoinflammatory Diseases
Adult-Onset Still’s Disease
Idiopathic Recurrent Acute Pericarditis
Adamantiades-Behçet’s Disease
Amyloidosis