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F r o m W i k i p e d i a , t h e f r e e e n c y c l o p e d i a
( R e d i r e c t e d f r o m P e r i o d i c f e v e r )
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. Instead, the autoinflammatory diseases are characterized by errors in the innate immune system .[1]
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 .[2]
Most autoinflammatory diseases are genetic and present during childhood.[3] 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 .[citation needed ]
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.[4] Thus, IL-1β has become a common therapeutic target, and medications such as anakinra , rilonacept , and canakinumab have revolutionized the treatment of autoinflammatory diseases.[citation needed ]
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.[citation needed ]
Individual periodic fever syndromes [ edit ]
See also [ edit ]
Further reading [ edit ]
Hobart A. Reimann , Periodic Disease: a probable syndrome including periodic fever, benign paroxysmal peritonitis, cyclic neutropenia and intermittent arthralgia. JAMA, 1948.[8]
Hobart A Reimann, Periodic Disease: periodic fever, periodic abdominalgia, cyclic neutropenia, intermittent arthralgia, angioneurotic edema, anaphylactoid purpura and periodic paralysis. JAMA, 1949.[9]
Hobart A Reimann, Moadié, J; Semerdjian, S; Sahyoun, PF, Periodic Peritonitis—Heredity & Pathology: report of seventy-two cases. JAMA, 1954.[10]
Hobart A Reimann, Periodic fever, an entity: A collection of 52 cases. AmJMedSci, 1962.[11]
References [ edit ]
^ Hausmann, JS; Dedeoglu, F (July 2013). "Autoinflammatory diseases in pediatrics". Dermatologic Clinics . 31 (3 ): 481–94. doi :10.1016/j.det.2013.04.003 . PMID 23827250 .
^ Jamilloux, Y; Bourdonnay, E; Gerfaud-Valentin, M; Py, BF; Lefeuvre, L; Barba, T; Broussolle, C; Henry, T; Sève, P (14 September 2016). "[Interleukin-1, inflammasome and autoinflammatory diseases]". La Revue de Médecine Interne . 39 (4 ): 233–239. doi :10.1016/j.revmed.2016.07.007 . PMID 27639913 .
^ Houten SM, Frenkel J, Waterham HR (2003). "Isoprenoid biosynthesis in hereditary periodic fever syndromes and inflammation" . Cell. Mol. Life Sci . 60 (6 ): 1118–34. doi :10.1007/s00018-003-2296-4 . PMC 11146049 . PMID 12861380 . S2CID 23745920 .
^ Kozycki CT, Kodati S, Huryn L, et al. (2022). "Gain-of-function mutations in ALPK1 cause an NF-κB-mediated autoinflammatory disease: functional assessment, clinical phenotyping and disease course of patients with ROSAH syndrome" . Annals of the Rheumatic Diseases . 81 (10 ): 1453–1464. doi :10.1136/annrheumdis-2022-222629 . ISSN 0003-4967 . PMC 9484401 . PMID 35868845 .
^ Marrani E, Burns JC, Cimaz R (2018). "How Should We Classify Kawasaki Disease?" . Frontiers in Immunology . 9 : 2974. doi :10.3389/fimmu.2018.02974 . PMC 6302019 . PMID 30619331 .
^ Reimann, Hobart A (1948). "Periodic Disease: a probable syndrome including periodic fever, benign paroxysmal peritonitis, cyclic neutropenia and intermittent arthralgia". JAMA . 136 (4 ): 239–244. doi :10.1001/jama.1948.02890210023004 . PMID 18920089 .
^ Reimann, Hobart A (1949). "Periodic Disease: periodic fever, periodic abdominalgia, cyclic neutropenia, intermittent arthralgia, angioneurotic edema, anaphylactoid purpura and periodic paralysis". JAMA . 141 (3 ): 175–183. doi :10.1001/jama.1949.02910030005002 . PMID 18139542 .
^ Reimann, Hobart A; Moadié, J; Semerdjian, S; Sahyoun, PF (1954). "Periodic Peritonitis—Heredity and Pathology". JAMA . 154 (15 ): 1254–1259. doi :10.1001/jama.1954.02940490018005 . PMID 13151833 .
^ Reimann, Hobart A (1962). "Periodic fever, an entity: A collection of 52 cases". The American Journal of the Medical Sciences . 243 (Feb): 162–74. doi :10.1097/00000441-196202000-00006 . PMID 14491227 . S2CID 27897376 .
External links [ edit ]
R e t r i e v e d f r o m " https://en.wikipedia.org/w/index.php?title=Periodic_fever_syndrome&oldid=1228242176 "
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