Untangling the Web of Systemic Autoinflammatory Diseases
نویسندگان
چکیده
The innate immune system is involved in the pathophysiology of systemic autoinflammatory diseases (SAIDs), an enlarging group of disorders caused by dysregulated production of proinflammatory cytokines, such as interleukin-1β and tumor necrosis factor-α, in which autoreactive T-lymphocytes and autoantibodies are indeed absent. A widely deranged innate immunity leads to overactivity of proinflammatory cytokines and subsequent multisite inflammatory symptoms depicting various conditions, such as hereditary periodic fevers, granulomatous disorders, and pyogenic diseases, collectively described in this review. Further research should enhance our understanding of the genetics behind SAIDs, unearth triggers of inflammatory attacks, and result in improvement for their diagnosis and treatment.
منابع مشابه
AAO: Autoimmune and Autoinflammatory (Disease) in Otology: What is New in Immune‐Mediated Hearing Loss
OBJECTIVES Autoinflammatory diseases are a family of immune-mediated, rare diseases, some of which, exhibit sensorineural hearing loss (SNHL), suggesting potentially similar mechanisms of molecular pathogenesis between autoinflammatory-mediated hearing loss and autoimmune inner ear disease (AIED) may exist. The purpose of this review is to compare the clinical features of autoimmune and autoinf...
متن کاملPW02-017 - New tools for the ISSAID society website
Objectives The purpose of the website is to: • gather resources related to the systemic autoinflammatory diseases in order to facilitate contacts between interested physicians and researchers. • provide support to share and rapidly disseminate information, thoughts, feelings and experiences to improve the quality of life of patients and families affected by systemic auto-inflammatory diseases, ...
متن کاملAutoinflammatory diseases in childhood
Autoinflammatory diseases are defined as recurrent attacks of systemic inflammation that are often unprovoked (or triggered by a minor event) related to a lack of adequate regulation of the innate immune system. Within the past decade, the list of autoinflammatory diseases has included cryopyrin-associated periodic syndromes, familial Mediterranean fever, mevalonate kinase deficiency, tumor nec...
متن کاملFifth International Congress on Familial Mediterranean Fever and Systemic Autoinflammatory Diseases.
The Fifth International Congress on Familial Mediterranean Fever and Systemic Autoinflammatory Diseases (Rome, Italy, 4-8 April, 2008) reviewed developments in the field of innate immunity and discussed their relevance to the pathogenesis of associated diseases. The meeting gathered over 300 participants from 32 countries. New modes of inheritance of autoinflammatory diseases, animal models, no...
متن کامل[Hereditary systemic autoinflammatory diseases].
Systemic autoinflammatory diseases encompass different rare clinical entities characterized by recurrent acute inflammatory episodes secondary to a dysregulated inflammatory process. Since their first clinical descriptions, the Mendelian hereditary nature of some of them became evident, with their genetic and molecular basis being recently elucidated. There are disease-causing mutations in gene...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 2014 شماره
صفحات -
تاریخ انتشار 2014