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Autoinflammatory diseases (AIDs) are a group of rare disorders caused by dysfunction of the innate immune system. These responses are characterized by periodic or chronic systemic inflammation , usually without the involvement of adaptive immunity.
Yao syndrome (YAOS), formerly called NOD2-associated autoinflammatory disease, [1] is an autoinflammatory syndrome involving episodes of fever and abnormal inflammation affecting many parts of the body, particularly the skin, joints, and gastrointestinal system.
Periodic fever syndromes are a set of disorders characterized by recurrent episodes of systemic and organ-specific inflammation.Unlike autoimmune disorders such as systemic lupus erythematosus, in which the disease is caused by abnormalities of the adaptive immune system, people with autoinflammatory diseases do not produce autoantibodies or antigen-specific T or B cells.
Autoimmune/inflammatory syndrome induced by adjuvants (ASIA), or Shoenfeld's syndrome, is a hypothesised autoimmune disorder proposed by Israeli immunologist Yehuda Shoenfeld in 2011. [1]
It encompasses a spectrum of three clinically overlapping autoinflammatory syndromes including familial cold autoinflammatory syndrome (FCAS, formerly termed familial cold-induced urticaria), the Muckle–Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID, also called chronic infantile neurologic cutaneous and ...
As of 2017 the cause of the disease was not understood. [1] A 2024 review by Braud and Lipsker aimed to "describe what is currently known about the pathogenesis of this peculiar disease, as well as to address its diagnosis and management" and concluded that "physiopathology of Schnitzler syndrome remains elusive" and "the main question regarding the relationship between the autoinflammatory ...
Treatment Colchicine [ 5 ] Deficiency of the interleukin-1–receptor antagonist ( DIRA ) is an autosomal recessive , genetic autoinflammatory syndrome resulting from mutations in IL1RN , the gene encoding the interleukin 1 receptor antagonist .
Many doctors and articles described CRMO as an autoimmune disease that has symptoms similar to osteomyelitis, but without the infection. Some doctors thought CRMO was related to SAPHO syndrome. Research now classifies CRMO as an inherited autoinflammatory disease but have yet to isolate the exact gene or other causes responsible for it.