Autoinflammatory Disorders Publisher



Jamee M^{1, 2} ; Rezaei N^{3, 4, 5} Show Affiliations
Source: Translational Autoimmunity: Autoimmune Diseases in Different Organs Published:2022

Abstract

Autoinflammatory disorders are a broad spectrum of systemic disorders characterized by aberrant activation of the innate immunity with various combinations of mainly mucocutaneous, musculoskeletal, ocular, cardiovascular, neurological, and gastrointestinal manifestations. In the recent decade, the number of patients with systemic autoinflammation or disorders with autoinflammatory components has remarkably increased and several new disorders have been categorized in the autoinflammatory disorder entity. In addition, the development of advanced genetic techniques has played a pivotal role in expanding our knowledge about underlying genetic defects and immunopathogenesis. However, as patients with such abnormalities often present with nonspecific manifestations, for an accurate diagnosis, the primary care physician should have a high index of suspicion for the referral, and a multidisciplinary approach is required for both diagnosis and management of autoinflammatory disorders. In this chapter, autoinflammatory disorders are introduced based on the latest available updates in the literature and a diagnosis-to-treatment approach is perused to increase awareness among clinicians. © 2022 Elsevier Inc. All rights reserved.