Chapter 10 Episodic Autoinflammatory Disorders in Children

摘要: Publisher Summary This chapter presents a clinical presentation and outlines the recent progress in elucidating underlying pathophysiology for episodic autoinflammatory disorders children. The diseases are newly recognized expanding class of inflammatory that share many features with autoimmune diseases. They differ significantly, syndromes characterized by an absence, not only pathogens, but also high titre autoantibodies pathogenic auto reactive T cells. intermittent disorders, known as periodic fever syndromes, lead to recurrent episodes alternating more or less prolonged periods disease remission. usually accompanied additional systemic localized symptoms involving joints, skin, eyes, abdomen. To diagnose patient presenting fever, combination characteristics such age onset, length attacks, precipitating factors, associated family history documented along genetic screening appropriate genes.