Periodic fever syndrome: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
Line 7: Line 7:
The '''periodic fever syndromes''' (also known as '''autoinflammatory syndromes''') are a set of genetic disorders in which the mechanisms which initiate and control [[inflammation]] are disturbed leading to uncontrolled inflammation throughout the body. The syndromes are diverse but tend to cause fever, [[arthralgia|joint pains]], [[abdominal pain]]s and may lead to chronic complications such as [[amyloidosis]].  
The '''periodic fever syndromes''' (also known as '''autoinflammatory syndromes''') are a set of genetic disorders in which the mechanisms which initiate and control [[inflammation]] are disturbed leading to uncontrolled inflammation throughout the body. The syndromes are diverse but tend to cause fever, [[arthralgia|joint pains]], [[abdominal pain]]s and may lead to chronic complications such as [[amyloidosis]].  
==Causes==
==Causes==
* Peridic fever syndrome are [[cause|caused]] by a mutation in the relative genes.<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="KrausCulican2009">{{cite journal|last1=Kraus|first1=Courtney L|last2=Culican|first2=Susan M|title=Nummular keratopathy in a patient with Hyper-IgD Syndrome|journal=Pediatric Rheumatology|volume=7|issue=1|year=2009|issn=1546-0096|doi=10.1186/1546-0096-7-14}}</ref>
* Periodic fever syndrome are caused by a [[mutation]] in the relative [[Gene|genes]].
* For more information on [[familial Mediterranean fever]] [[causes]] [[Familial mediterranean fever causes|click here]].
* For more information on [[familial Mediterranean fever]] [[causes]] [[Familial mediterranean fever causes|click here]].
* For more information on [[Hyperimmunoglobulinemia D with recurrent fever]] [[causes]] [[Hyperimmunoglobulinemia D with recurrent fever|click here]].
* For more information on [[Hyperimmunoglobulinemia D with recurrent fever]] [[causes]] [[Hyperimmunoglobulinemia D with recurrent fever|click here]].
Line 16: Line 16:
* For more information on [[pyogenic sterile arthritis, pyoderma gangrenosum, acne]] [[causes]] [[Pyogenic sterile arthritis, pyoderma gangrenosum, acne|click here]].
* For more information on [[pyogenic sterile arthritis, pyoderma gangrenosum, acne]] [[causes]] [[Pyogenic sterile arthritis, pyoderma gangrenosum, acne|click here]].
==Classification==
==Classification==
* Periodic fever syndromes are classified to:<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="KrausCulican2009">{{cite journal|last1=Kraus|first1=Courtney L|last2=Culican|first2=Susan M|title=Nummular keratopathy in a patient with Hyper-IgD Syndrome|journal=Pediatric Rheumatology|volume=7|issue=1|year=2009|issn=1546-0096|doi=10.1186/1546-0096-7-14}}</ref>
* Periodic fever syndromes are [[Classification|classified]] to:<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="KrausCulican2009">{{cite journal|last1=Kraus|first1=Courtney L|last2=Culican|first2=Susan M|title=Nummular keratopathy in a patient with Hyper-IgD Syndrome|journal=Pediatric Rheumatology|volume=7|issue=1|year=2009|issn=1546-0096|doi=10.1186/1546-0096-7-14}}</ref>
** [[Familial Mediterranean fever]]
**[[Familial Mediterranean fever]]
** [[Hyperimmunoglobulinemia D with recurrent fever]]
** [[Hyperimmunoglobulinemia D with recurrent fever]]
** [[TNF receptor associated periodic syndrome]] ([[TNF receptor associated periodic syndrome|TRAPS]])
** [[TNF receptor associated periodic syndrome]] ([[TNF receptor associated periodic syndrome|TRAPS]])
Line 58: Line 58:
| rowspan="11" |
| rowspan="11" |
===Autoinflammatory diseases===
===Autoinflammatory diseases===
| '''[[Familial mediterranean fever]]'''<ref>{{Cite journal
| '''[[Familial mediterranean fever]]'''<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref>
| author = [[M. Medlej-Hashim]], [[I. Petit]], [[S. Adib]], [[E. Chouery]], [[N. Salem]], [[V. Delague]], [[M. Rawashdeh]], [[I. Mansour]], [[G. Lefranc]], [[R. Naman]], [[J. Loiselet]], [[J. C. Lecron]], [[J. L. Serre]] & [[A. Megarbane]]
| title = Familial Mediterranean Fever: association of elevated IgD plasma levels with specific MEFV mutations
| journal = [[European journal of human genetics : EJHG]]
| volume = 9
| issue = 11
| pages = 849–854
| year = 2001
| month = November
| doi = 10.1038/sj.ejhg.5200725
| pmid = 11781702
}}</ref><ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref>
|
|
* [[Autosomal recessive]]/[[Autosomal Dominant|dominant]]
* [[Autosomal recessive]]/[[Autosomal Dominant|dominant]]
Line 98: Line 87:
* [[Mutation]] in [[MEFV]] [[gene]]
* [[Mutation]] in [[MEFV]] [[gene]]
|-
|-
| '''[[Hyperimmunoglobulinemia D with recurrent fever|Hyper IgD with recurrent fever]]'''<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="KrausCulican2009">{{cite journal|last1=Kraus|first1=Courtney L|last2=Culican|first2=Susan M|title=Nummular keratopathy in a patient with Hyper-IgD Syndrome|journal=Pediatric Rheumatology|volume=7|issue=1|year=2009|issn=1546-0096|doi=10.1186/1546-0096-7-14}}</ref><ref name="Mulders-MandersSimon2015">{{cite journal|last1=Mulders-Manders|first1=C. M.|last2=Simon|first2=A.|title=Hyper-IgD syndrome/mevalonate kinase deficiency: what is new?|journal=Seminars in Immunopathology|volume=37|issue=4|year=2015|pages=371–376|issn=1863-2297|doi=10.1007/s00281-015-0492-6}}</ref>
| '''[[Hyperimmunoglobulinemia D with recurrent fever|Hyper IgD with recurrent fever]]'''<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="KrausCulican2009">{{cite journal|last1=Kraus|first1=Courtney L|last2=Culican|first2=Susan M|title=Nummular keratopathy in a patient with Hyper-IgD Syndrome|journal=Pediatric Rheumatology|volume=7|issue=1|year=2009|issn=1546-0096|doi=10.1186/1546-0096-7-14}}</ref>
|
|
* [[Autosomal recessive]]
* [[Autosomal recessive]]
Line 129: Line 118:
* [[Mutation]] in [[Mevalonate kinase|MVK]] [[gene]]
* [[Mutation]] in [[Mevalonate kinase|MVK]] [[gene]]
|-
|-
| '''[[TNF receptor associated periodic syndrome|TNF receptor-associated periodic syndrome]]'''<ref name="ToroAksentijevich2000">{{cite journal|last1=Toro|first1=Jorge R.|last2=Aksentijevich|first2=Ivona|last3=Hull|first3=Keith|last4=Dean|first4=Jane|last5=Kastner|first5=Daniel L.|title=Tumor Necrosis Factor Receptor–Associated Periodic Syndrome|journal=Archives of Dermatology|volume=136|issue=12|year=2000|issn=0003-987X|doi=10.1001/archderm.136.12.1487}}</ref><ref name="LachmannPapa2014">{{cite journal|last1=Lachmann|first1=H J|last2=Papa|first2=R|last3=Gerhold|first3=K|last4=Obici|first4=L|last5=Touitou|first5=I|last6=Cantarini|first6=L|last7=Frenkel|first7=J|last8=Anton|first8=J|last9=Kone-Paut|first9=I|last10=Cattalini|first10=M|last11=Bader-Meunier|first11=B|last12=Insalaco|first12=A|last13=Hentgen|first13=V|last14=Merino|first14=R|last15=Modesto|first15=C|last16=Toplak|first16=N|last17=Berendes|first17=R|last18=Ozen|first18=S|last19=Cimaz|first19=R|last20=Jansson|first20=A|last21=Brogan|first21=P A|last22=Hawkins|first22=P N|last23=Ruperto|first23=N|last24=Martini|first24=A|last25=Woo|first25=P|last26=Gattorno|first26=M|title=The phenotype of TNF receptor-associated autoinflammatory syndrome (TRAPS) at presentation: a series of 158 cases from the Eurofever/EUROTRAPS international registry|journal=Annals of the Rheumatic Diseases|volume=73|issue=12|year=2014|pages=2160–2167|issn=0003-4967|doi=10.1136/annrheumdis-2013-204184}}</ref>
| '''[[TNF receptor associated periodic syndrome|TNF receptor-associated periodic syndrome]]'''
|
|
* [[Autosomal dominant]]
* [[Autosomal dominant]]
Line 159: Line 148:
* [[Mutation]] in TNFRSF1A [[gene]]
* [[Mutation]] in TNFRSF1A [[gene]]
|-
|-
|'''[[Muckle-Wells syndrome|Muckle-Wells Syndrome]]'''<ref name="HawkinsLachmann2004">{{cite journal|last1=Hawkins|first1=Philip N.|last2=Lachmann|first2=Helen J.|last3=Aganna|first3=Ebun|last4=McDermott|first4=Michael F.|title=Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra|journal=Arthritis & Rheumatism|volume=50|issue=2|year=2004|pages=607–612|issn=0004-3591|doi=10.1002/art.20033}}</ref><ref name="AhmadiBrewer2011">{{cite journal|last1=Ahmadi|first1=Neda|last2=Brewer|first2=Carmen C.|last3=Zalewski|first3=Christopher|last4=King|first4=Kelly A.|last5=Butman|first5=John A.|last6=Plass|first6=Nicole|last7=Henderson|first7=Cailin|last8=Goldbach-Mansky|first8=Raphaela|last9=Kim|first9=H. Jeffrey|title=Cryopyrin-Associated Periodic Syndromes|journal=Otolaryngology–Head and Neck Surgery|volume=145|issue=2|year=2011|pages=295–302|issn=0194-5998|doi=10.1177/0194599811402296}}</ref>
|'''[[Muckle-Wells syndrome|Muckle-Wells Syndrome]]'''
|
|
* [[Autosomal dominant]]
* [[Autosomal dominant]]
Line 188: Line 177:
* [[Mutation]] in NLRP3 [[gene]]
* [[Mutation]] in NLRP3 [[gene]]
|-
|-
|[[Familial cold urticaria|'''Familial cold urticaria''']]<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="StychDobrovolny2008">{{cite journal|last1=Stych|first1=Beate|last2=Dobrovolny|first2=Diana|title=Familial cold auto-inflammatory syndrome (FCAS): characterization of symptomatology and impact on patients' lives|journal=Current Medical Research and Opinion|volume=24|issue=6|year=2008|pages=1577–1582|issn=0300-7995|doi=10.1185/03007990802081543}}</ref>
|[[Familial cold urticaria|'''Familial cold urticaria''']]<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref>
|
|
* [[Autosomal dominant]]
* [[Autosomal dominant]]
Line 218: Line 207:
* [[Mutation]] in NLRP3 [[gene]]
* [[Mutation]] in NLRP3 [[gene]]
|-
|-
|[[Neonatal onset multisystem inflammatory disease|'''Neonatal onset multisystem inflammatory disease''']]<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref><ref name="Goldbach-ManskyDailey2006">{{cite journal|last1=Goldbach-Mansky|first1=Raphaela|last2=Dailey|first2=Natalie J.|last3=Canna|first3=Scott W.|last4=Gelabert|first4=Ana|last5=Jones|first5=Janet|last6=Rubin|first6=Benjamin I.|last7=Kim|first7=H. Jeffrey|last8=Brewer|first8=Carmen|last9=Zalewski|first9=Christopher|last10=Wiggs|first10=Edythe|last11=Hill|first11=Suvimol|last12=Turner|first12=Maria L.|last13=Karp|first13=Barbara I.|last14=Aksentijevich|first14=Ivona|last15=Pucino|first15=Frank|last16=Penzak|first16=Scott R.|last17=Haverkamp|first17=Margje H.|last18=Stein|first18=Leonard|last19=Adams|first19=Barbara S.|last20=Moore|first20=Terry L.|last21=Fuhlbrigge|first21=Robert C.|last22=Shaham|first22=Bracha|last23=Jarvis|first23=James N.|last24=O'Neil|first24=Kathleen|last25=Vehe|first25=Richard K.|last26=Beitz|first26=Laurie O.|last27=Gardner|first27=Gregory|last28=Hannan|first28=William P.|last29=Warren|first29=Robert W.|last30=Horn|first30=William|last31=Cole|first31=Joe L.|last32=Paul|first32=Scott M.|last33=Hawkins|first33=Philip N.|last34=Pham|first34=Tuyet Hang|last35=Snyder|first35=Christopher|last36=Wesley|first36=Robert A.|last37=Hoffmann|first37=Steven C.|last38=Holland|first38=Steven M.|last39=Butman|first39=John A.|last40=Kastner|first40=Daniel L.|title=Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1β Inhibition|journal=New England Journal of Medicine|volume=355|issue=6|year=2006|pages=581–592|issn=0028-4793|doi=10.1056/NEJMoa055137}}</ref><ref name="KimMontealegre Sanchez2014">{{cite journal|last1=Kim|first1=Hanna|last2=Montealegre Sanchez|first2=Gina A.|last3=Chapelle|first3=Dawn C.|last4=Plass|first4=Nicole|last5=Dwyer|first5=Andrew|last6=Goldbach-Mansky|first6=Raphaela|last7=Hill|first7=Suvimol|title=A80: Skeletal Features of Neonatal-Onset Multisystem Inflammatory Disease (NOMID) on Anakinra Treatment: Long-Term Follow-up|journal=Arthritis & Rheumatology|volume=66|year=2014|pages=S113–S113|issn=23265191|doi=10.1002/art.38496}}</ref>
|[[Neonatal onset multisystem inflammatory disease|'''Neonatal onset multisystem inflammatory disease''']]<ref name="Kastner2005">{{cite journal|last1=Kastner|first1=D. L.|title=Hereditary Periodic Fever Syndromes|journal=Hematology|volume=2005|issue=1|year=2005|pages=74–81|issn=1520-4391|doi=10.1182/asheducation-2005.1.74}}</ref>
|
|
* [[Autosomal dominant]]
* [[Autosomal dominant]]
Line 254: Line 243:
* [[Mutation]] in NLRP3 [[gene]]
* [[Mutation]] in NLRP3 [[gene]]
|-
|-
|'''[[Pyogenic sterile arthritis, pyoderma gangrenosum, acne]] ([[Papa syndrome]])'''<ref name="YeonLindor2000">{{cite journal|last1=Yeon|first1=Howard B.|last2=Lindor|first2=Noralane M.|last3=Seidman|first3=J.G.|last4=Seidman|first4=Christine E.|title=Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne Syndrome Maps to Chromosome 15q|journal=The American Journal of Human Genetics|volume=66|issue=4|year=2000|pages=1443–1448|issn=00029297|doi=10.1086/302866}}</ref><ref name="SchellevisStoffels2011">{{cite journal|last1=Schellevis|first1=M. A.|last2=Stoffels|first2=M.|last3=Hoppenreijs|first3=E. P. A. H.|last4=Bodar|first4=E.|last5=Simon|first5=A.|last6=van der Meer|first6=J. W. M.|title=Variable expression and treatment of PAPA syndrome|journal=Annals of the Rheumatic Diseases|volume=70|issue=6|year=2011|pages=1168–1170|issn=0003-4967|doi=10.1136/ard.2009.126185}}</ref>
|'''[[Pyogenic sterile arthritis, pyoderma gangrenosum, acne]] ([[Papa syndrome]])'''
|
|
* [[Autosomal dominant]]
* [[Autosomal dominant]]
Line 285: Line 274:
* [[Mutation]] in PSTPIP1 [[gene]]
* [[Mutation]] in PSTPIP1 [[gene]]
|-
|-
| '''[[Periodic fever, aphthous stomatitis, pharyngitis and adenitis|Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA)]]'''<ref name="VanoniFederici2018">{{cite journal|last1=Vanoni|first1=Federica|last2=Federici|first2=Silvia|last3=Antón|first3=Jordi|last4=Barron|first4=Karyl S.|last5=Brogan|first5=Paul|last6=De Benedetti|first6=Fabrizio|last7=Dedeoglu|first7=Fatma|last8=Demirkaya|first8=Erkan|last9=Hentgen|first9=Veronique|last10=Kallinich|first10=Tilmann|last11=Laxer|first11=Ronald|last12=Russo|first12=Ricardo|last13=Toplak|first13=Natasa|last14=Uziel|first14=Yosef|last15=Martini|first15=Alberto|last16=Ruperto|first16=Nicolino|last17=Gattorno|first17=Marco|last18=Hofer|first18=Michael|title=An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA)|journal=Pediatric Rheumatology|volume=16|issue=1|year=2018|issn=1546-0096|doi=10.1186/s12969-018-0246-9}}</ref><ref name="CattaliniSoliani2015">{{cite journal|last1=Cattalini|first1=Marco|last2=Soliani|first2=Martina|last3=Rigante|first3=Donato|last4=Lopalco|first4=Giuseppe|last5=Iannone|first5=Florenzo|last6=Galeazzi|first6=Mauro|last7=Cantarini|first7=Luca|title=Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease|journal=Mediators of Inflammation|volume=2015|year=2015|pages=1–11|issn=0962-9351|doi=10.1155/2015/570418}}</ref><ref name="GattornoCaorsi2009">{{cite journal|last1=Gattorno|first1=M.|last2=Caorsi|first2=R.|last3=Meini|first3=A.|last4=Cattalini|first4=M.|last5=Federici|first5=S.|last6=Zulian|first6=F.|last7=Cortis|first7=E.|last8=Calcagno|first8=G.|last9=Tommasini|first9=A.|last10=Consolini|first10=R.|last11=Simonini|first11=G.|last12=Pelagatti|first12=M. A.|last13=Baldi|first13=M.|last14=Ceccherini|first14=I.|last15=Plebani|first15=A.|last16=Frenkel|first16=J.|last17=Sormani|first17=M. P.|last18=Martini|first18=A.|title=Differentiating PFAPA Syndrome From Monogenic Periodic Fevers|journal=PEDIATRICS|volume=124|issue=4|year=2009|pages=e721–e728|issn=0031-4005|doi=10.1542/peds.2009-0088}}</ref>
| '''[[Periodic fever, aphthous stomatitis, pharyngitis and adenitis|Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA)]]'''
|
|
* <nowiki>Unkown</nowiki>
* <nowiki>Unkown</nowiki>
Line 313: Line 302:
* Unknown  
* Unknown  
|-
|-
|'''[[Blau syndrome]]'''<ref name="RoséAróstegui2009">{{cite journal|last1=Rosé|first1=Carlos D.|last2=Aróstegui|first2=Juan I.|last3=Martin|first3=Tammy M.|last4=Espada|first4=Graciela|last5=Scalzi|first5=Lisabeth|last6=Yagüe|first6=Jordi|last7=Rosenbaum|first7=James T.|last8=Modesto|first8=Consuelo|last9=Cristina Arnal|first9=Maria|last10=Merino|first10=Rosa|last11=García-Consuegra|first11=Julia|last12=Carballo Silva|first12=María Antonia|last13=Wouters|first13=Carine H.|title=NOD2-Associated pediatric granulomatous arthritis, an expanding phenotype: Study of an international registry and a national cohort in spain|journal=Arthritis & Rheumatism|volume=60|issue=6|year=2009|pages=1797–1803|issn=00043591|doi=10.1002/art.24533}}</ref><ref name="KimPark2016">{{cite journal|last1=Kim|first1=Woojoong|last2=Park|first2=Eujin|last3=Ahn|first3=Yo Han|last4=Lee|first4=Jiwon M.|last5=Kang|first5=Hee Gyung|last6=Kim|first6=Byung Joo|last7=Ha|first7=Il-Soo|last8=Cheong|first8=Hae Il|title=A familial case of Blau syndrome caused by a novelNOD2genetic mutation|journal=Korean Journal of Pediatrics|volume=59|issue=Suppl 1|year=2016|pages=S5|issn=1738-1061|doi=10.3345/kjp.2016.59.11.S5}}</ref>
|'''[[Blau syndrome]]'''
|
|
* [[Autosomal dominant]]
* [[Autosomal dominant]]
Line 354: Line 343:
{{WH}}
{{WH}}
{{WS}}
{{WS}}
<references />

Revision as of 14:45, 26 September 2019

Periodic Fever Syndrome Microchapters

Patient Information

Overview

Classification

Familial Mediterranean fever
Hyperimmunoglobulinemia D with recurrent fever
TNF receptor associated periodic syndrome
Cryopyrin-associated periodic syndrome
Periodic fever, aphthous stomatitis, pharyngitis and adenitis
Blau syndrome
Pyogenic sterile arthritis, pyoderma gangrenosum, acne

Causes

Differential Diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Synonyms and keywords: Autoinflammatory syndrome

Overview

The periodic fever syndromes (also known as autoinflammatory syndromes) are a set of genetic disorders in which the mechanisms which initiate and control inflammation are disturbed leading to uncontrolled inflammation throughout the body. The syndromes are diverse but tend to cause fever, joint pains, abdominal pains and may lead to chronic complications such as amyloidosis.

Causes

Classification

Differential Diagnosis

  • Periodic fever syndromes should be differentiated from each other.
Category of Disease Diseases Signs and symptoms Laboratory findings
Inheritance pattern Fever duration Frequency of attacks Abdominal pain Arthralgia/Arthritis Chest pain Skin rash Myalgia/Body pain Diarrhea/Vomiting Neurologic manifestations Conjunctivitis Aphthous stomatitis Lymphadenopathy Splenomegaly Complete blood count (CBC) C- reactive protein (CRP)
Erythrocyte sedimentation rate (ESR) Other findings Genetic analysis

Autoinflammatory diseases

  Familial mediterranean fever[1]
  • 12-72 h
  • Weekly or 3-4 times/year
 + + + + + -/+ -/+ -/+ + ↑ ↑
 Hyper IgD with recurrent fever[1][2]
  • 3-7 days
  • Every 2-12 weeks
 + + + + + - +/- +/- +/- ↑ ↑
 TNF receptor-associated periodic syndrome
  • 3-4 weeks
  • Variable
 + + -
  • Migrating rash with deep pain under the areas with the rash
  • Severe pain follows the rash path in a centrifugal pattern
 - - + - +/- + ↑ ↑
Muckle-Wells Syndrome
  • 2-3 days
  • More common during cold seasons
 + + - + + + + - - ↑ ↑
  • Cold-triggered attacks
Familial cold urticaria[1]
  • 12-24 hours, or longer
  • Common in cold seasons
 - + - - - + +/- - - ↑ ↑
  • Cold-triggered attacks
Neonatal onset multisystem inflammatory disease[1]
  • Continuous
  • Common in cold seasons
 + + + + + +/- + ↑ ↑
Pyogenic sterile arthritis, pyoderma gangrenosum, acne (Papa syndrome)
  • Variable
  • Variable
 +/- +/- +/- +/- - - - - - ↑ ↑
 Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA)
  • Unkown
  • 3-6 days
  • Every 21-28 days
 + + - - + + - - + - ↑ ↑
  • Unknown
Blau syndrome
  • Intermittent or persistent daily fever
  • Variable
 +/- + +/- + +/- - + +/- + ↑ ↑


References

Template:WH Template:WS

  1. ↑ 1.0 1.1 1.2 1.3 1.4 Kastner, D. L. (2005). "Hereditary Periodic Fever Syndromes". Hematology. 2005 (1): 74–81. doi:10.1182/asheducation-2005.1.74. ISSN 1520-4391.
  2. ↑ 2.0 2.1 Kraus, Courtney L; Culican, Susan M (2009). "Nummular keratopathy in a patient with Hyper-IgD Syndrome". Pediatric Rheumatology. 7 (1). doi:10.1186/1546-0096-7-14. ISSN 1546-0096.
Retrieved from "https://www.wikidoc.org/index.php?title=Periodic_fever_syndrome&oldid=1582629"