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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.
TNF receptor associated periodic syndrome; Other names: Familial Hibernian fever, Tumor necrosis factor receptor associated periodic syndrome: Specialty: Immunology: Symptoms: Vertigo, pericarditis [1] Causes: Mutations in the TNFRSF1A gene [2] Diagnostic method: Blood test, Genetic test [3] [4] Treatment: Corticosteroids, NSAIDS [1]
Neonatal-onset multisystem inflammatory disease is a rare genetic periodic fever syndrome which causes uncontrolled inflammation in multiple parts of the body starting in the newborn period. Symptoms include skin rashes, severe arthritis, and chronic meningitis leading to neurologic damage. It is one of the cryopyrin-associated periodic syndromes.
The syndromes within CAPS overlap clinically, and patients may have features of more than one disorder. In a retrospective cohort of 136 CAPS patients with systemic involvement from 16 countries, [2] the most prevalent clinical features were fever (84% of cases, often with concurrent constitutional symptoms such as fatigue, malaise, mood disorders or failure to thrive), skin rash (either ...
Familial Mediterranean fever (FMF) is a hereditary inflammatory disorder. [ 1 ] : 149 FMF is an autoinflammatory disease caused by mutations in the Mediterranean fever (MEFV) gene, which encodes a 781–amino acid protein called pyrin . [ 2 ]
A hereditary disorder driven by pyrin mutation, called PAAND (Pyrin-associated autoinflammation with neutrophilic dermatosis), [7] is characterized by neutrophilic dermatosis, recurrent fever, increased acute-phase reactants, arthralgia, or myalgia. Patients with PAAND have a serine-to-arginine substitution at position 242 in pyrin.
MKD is a periodic fever syndrome originally described in 1984 by the internist Jos van der Meer, [4] then at Leiden University Medical Centre. No more than 300 cases have been described worldwide. MKD was originally described as hyperimmunoglobulin D syndrome (HIDS), but HIDS is now recognized as a mild manifestation of MKD. [1]
Autoinflammatory syndromes are a group of inherited disorders characterized by innate immune system dysregulation often manifesting with bouts of inflammatory skin lesions and periodic fevers The main article for this category is Periodic fever syndrome .
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