Auto-Inflammatory Syndromes: Pathophysiology, Diagnosis, and Management

Description

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. Front Matter ....Pages i-xix Immunology of Auto-inflammatory Syndromes (Grant S. Schulert)....Pages 1-16 Front Matter ....Pages 17-17 Mevalonate Kinase Deficiency (MKD)/Hyperimmunoglobulin D Syndrome (HIDS) (Olga Petryna, Neha Purat)....Pages 19-27 The TNF Receptor-Associated Autoinflammatory Syndrome (TRAPS) (Marco Gattorno)....Pages 29-38 PAPA Syndrome and the Spectrum of PSTPIP1-Associated Inflammatory Diseases (Dirk Holzinger, Johannes Roth)....Pages 39-59 Blau Syndrome (Rebecca Trachtman, Karen B. Onel)....Pages 61-67 Deficiency of the Interleukin-1 Receptor Antagonist (DIRA) (Arturo Diaz)....Pages 69-83 Deficiency of the Interleukin-36 Receptor Antagonist (DITRA) and Generalized Pustular Psoriasis (Arturo Diaz)....Pages 85-94 Cryopyrin-Associated Periodic Syndromes (CAPS) (Marinka Twilt, Susanne M. Benseler)....Pages 95-109 Familial Mediterranean Fever (Selcan Demir, İlkin Elif Günel, Seza Özen)....Pages 111-123 Type I Interferonopathies: From Pathophysiology to Clinical Expression (Christina Maria Flessa, Evangelia Argiriou, Clio P. Mavragani)....Pages 125-145 Deficiency of Adenosine Deaminase 2 (DADA2) (Andreas Reiff)....Pages 147-157 Yao Syndrome (Qingping Yao)....Pages 159-166 Front Matter ....Pages 167-167 Systemic Juvenile Idiopathic Arthritis (Ricardo A. G. Russo, María Martha Katsicas)....Pages 169-191 Macrophage Activation Syndrome (MAS) (Bella Mehta, Petros Efthimiou)....Pages 193-201 Schnitzler Syndrome (Paolo Sfriso, Paola Galozzi)....Pages 203-212 Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome (Ezgi Deniz Batu, Fatma Dedeoglu)....Pages 213-226 Chronic Nonbacterial Osteomyelitis (Liza McCann, Michael W. Beresford, Gabriele Hahn, Christian M. Hedrich)....Pages 227-248 Improving the Transition from Pediatric to Adult Care for Adolescents and Young Adults with Autoinflammatory Diseases (Jonathan S. Hausmann, Kitty O’Hare)....Pages 249-259 Adult-Onset Still’s Disease (Petros Efthimiou, Sujani Yadlapati)....Pages 261-276 Idiopathic Recurrent Acute Pericarditis (Apostolos Kontzias)....Pages 277-286 Adamantiades-Behçet’s Disease (Petros P. Sfikakis)....Pages 287-296 Amyloidosis (Michael Voulgarelis, Ioannis Mitroulis, Athanasios G. Tzioufas)....Pages 297-311 Back Matter ....Pages 313-322