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- Periodic_fever_syndrome abstract "The periodic fever syndromes' (also known as autoinflammatory diseases or autoinflammatory syndromes) are a set of disorders characterized by recurrent episodes of systemic and organ-spefic inflammation. Unlike autoimmune disorders such as systemic lupus erythematosus, in which the disease is caused by abnormalities of the adaptive immune system, patients with autoinflammatory diseases do not produce autoantibodies or antigen-specific T or B cells. Instead, the autoinflammatory diseases are characterized by errors in the innate immune system.The syndromes are diverse, but tend to cause episodes of fever, joint pains, skin rashes, abdominal pains and may lead to chronic complications such as amyloidosis.Most autoinflammatory diseases are genetic and present during childhood. The most common genetic autoinflammatory syndrome is familial Mediterranean fever, which causes short episodes of fever, abdominal pain, serositis, lasting less than 72 hours. It is caused by mutations in the MEFV gene, which codes for the protein pyrin.Pyrin is a protein normally present in the inflammasome. The mutated pyrin protein is thought to cause inappropriate activation of the inflammasome, leading to release of the pro-inflammatory cytokine IL-1β. Most other autoinflammatory diseases also cause disease by inappropriate release of IL-1β. Thus, IL-1β has become a common therapeutic target, and medications such as anakinra, rilonacept, and canakinumab have revolutionized the treatment of autoinflammatory diseases.However, there are some autoinflammatory diseases that are not known to have a clear genetic cause. This includes PFAPA, which is the most common autoinflammatory disease seen in children, characterized by episodes of fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Other autoinflammatory diseases that do not have clear genetic causes include Adult-onset Still's disease, Systemic-onset juvenile idiopathic arthritis, Schnitzler syndrome, and Chronic recurrent multifocal osteomyelitis. It is likely that these diseases are multifactorial, with genes that make people susceptible to these diseases, but they require an additional environmental factor to trigger the disease.".
- Periodic_fever_syndrome diseasesdb "9836".
- Periodic_fever_syndrome emedicineSubject "article".
- Periodic_fever_syndrome emedicineTopic "952254".
- Periodic_fever_syndrome icd10 "E85.0".
- Periodic_fever_syndrome icd9 "277.31".
- Periodic_fever_syndrome meshId "D056660".
- Periodic_fever_syndrome wikiPageExternalLink autoinflammatory-vs-autoimmune-whats-the-difference.
- Periodic_fever_syndrome wikiPageID "6253974".
- Periodic_fever_syndrome wikiPageRevisionID "605211276".
- Periodic_fever_syndrome diseasesdb "9836".
- Periodic_fever_syndrome emedicinesubj "article".
- Periodic_fever_syndrome emedicinetopic "952254".
- Periodic_fever_syndrome hasPhotoCollection Periodic_fever_syndrome.
- Periodic_fever_syndrome icd "277.31".
- Periodic_fever_syndrome icd "E85.0".
- Periodic_fever_syndrome meshid "D056660".
- Periodic_fever_syndrome name "Periodic fever syndrome".
- Periodic_fever_syndrome subject Category:Inflammations.
- Periodic_fever_syndrome subject Category:Syndromes.
- Periodic_fever_syndrome type Abstraction100002137.
- Periodic_fever_syndrome type Attribute100024264.
- Periodic_fever_syndrome type Cognition100023271.
- Periodic_fever_syndrome type Complex105870365.
- Periodic_fever_syndrome type Concept105835747.
- Periodic_fever_syndrome type Condition113920835.
- Periodic_fever_syndrome type Content105809192.
- Periodic_fever_syndrome type Disease114070360.
- Periodic_fever_syndrome type Evidence105823932.
- Periodic_fever_syndrome type Idea105833840.
- Periodic_fever_syndrome type IllHealth114052046.
- Periodic_fever_syndrome type Illness114061805.
- Periodic_fever_syndrome type Inflammation114336539.
- Periodic_fever_syndrome type Inflammations.
- Periodic_fever_syndrome type Information105816287.
- Periodic_fever_syndrome type PathologicalState114051917.
- Periodic_fever_syndrome type PhysicalCondition114034177.
- Periodic_fever_syndrome type PsychologicalFeature100023100.
- Periodic_fever_syndrome type State100024720.
- Periodic_fever_syndrome type Symptom114299637.
- Periodic_fever_syndrome type Syndrome105870790.
- Periodic_fever_syndrome type Syndromes.
- Periodic_fever_syndrome type Whole105869584.
- Periodic_fever_syndrome type Disease.
- Periodic_fever_syndrome type AilmentCondition.
- Periodic_fever_syndrome type Situation.
- Periodic_fever_syndrome comment "The periodic fever syndromes' (also known as autoinflammatory diseases or autoinflammatory syndromes) are a set of disorders characterized by recurrent episodes of systemic and organ-spefic inflammation. Unlike autoimmune disorders such as systemic lupus erythematosus, in which the disease is caused by abnormalities of the adaptive immune system, patients with autoinflammatory diseases do not produce autoantibodies or antigen-specific T or B cells.".
- Periodic_fever_syndrome label "Periodic fever syndrome".
- Periodic_fever_syndrome label "Syndrome de fièvre périodique".
- Periodic_fever_syndrome label "自己炎症症候群".
- Periodic_fever_syndrome sameAs Syndrome_de_fièvre_périodique.
- Periodic_fever_syndrome sameAs 自己炎症症候群.
- Periodic_fever_syndrome sameAs m.0fz207.
- Periodic_fever_syndrome sameAs Q3278145.
- Periodic_fever_syndrome sameAs Q3278145.
- Periodic_fever_syndrome sameAs Periodic_fever_syndrome.
- Periodic_fever_syndrome wasDerivedFrom Periodic_fever_syndrome?oldid=605211276.
- Periodic_fever_syndrome isPrimaryTopicOf Periodic_fever_syndrome.
- Periodic_fever_syndrome name "Periodic fever syndrome".