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.
Petros Efthimiou, MD
Clinical Professor of Medicine
New York University School of Medicine
New York, NY
Introduction.- SECTION I: Pediatric Auto-Inflammatory Syndromes.- Systemic Arthritis.- Hyper-IgD.- TRAPS.- Schnitzler's Syndrome.- Papa Syndrome.- Blau Syndrome.- Dira Syndrome.- Cryopyrin Associated Periodic Syndromes.- Transition of Care: When the Pediatric Patient Becomes an Adult.- SECTION II: Adult Auto-Inflammatory Syndromes.- Adult Onset Still's Disease.- Idiopathic Recurrent Pericarditis.- Familial Mediterranean Fever.- Behcet's Disease.- Interferonopathies.- Sweet's Syndrome.- Anti-synthetase Syndrome.- Crystal Arthropathies.- Urticarial Vasculitis.- Relapsing Polychondritis.