Cryopyrin-associated periodic syndrome natural history, complications and prognosis: Difference between revisions
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===Natural History=== | ===Natural History=== | ||
*The symptoms of ( | *The symptoms of neonatal-onset multisystem inflammatory disease (NOMID) usually develops during infancy, and start with symptoms such as continuous often low-grade fever, skin rash, neurologic involvement, and arthropathy.<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> | ||
*The symptoms of (disease name) typically develop ___ years after exposure to ___. | *The symptoms of (disease name) typically develop ___ years after exposure to ___. | ||
*If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. | *If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. | ||
===Complications=== | ===Complications=== | ||
*Common complications of | *Common complications of neonatal-onset multisystem inflammatory disease (NOMID) include:<ref name="SibleyPlass2012">{{cite journal|last1=Sibley|first1=Cailin H.|last2=Plass|first2=Nikki|last3=Snow|first3=Joseph|last4=Wiggs|first4=Edythe A.|last5=Brewer|first5=Carmen C.|last6=King|first6=Kelly A.|last7=Zalewski|first7=Christopher|last8=Kim|first8=H. Jeffrey|last9=Bishop|first9=Rachel|last10=Hill|first10=Suvimol|last11=Paul|first11=Scott M.|last12=Kicker|first12=Patrick|last13=Phillips|first13=Zachary|last14=Dolan|first14=Joseph G.|last15=Widemann|first15=Brigitte|last16=Jayaprakash|first16=Nalini|last17=Pucino|first17=Frank|last18=Stone|first18=Deborah L.|last19=Chapelle|first19=Dawn|last20=Snyder|first20=Christopher|last21=Butman|first21=John A.|last22=Wesley|first22=Robert|last23=Goldbach-Mansky|first23=Raphaela|title=Sustained response and prevention of damage progression in patients with neonatal-onset multisystem inflammatory disease treated with anakinra: A cohort study to determine three- and five-year outcomes|journal=Arthritis & Rheumatism|volume=64|issue=7|year=2012|pages=2375–2386|issn=00043591|doi=10.1002/art.34409}}</ref><ref name="HillNamde2006">{{cite journal|last1=Hill|first1=Suvimol Chirathivat|last2=Namde|first2=Madjimbaye|last3=Dwyer|first3=Andrew|last4=Poznanski|first4=Andrew|last5=Canna|first5=Scott|last6=Goldbach-Mansky|first6=Raphaela|title=Arthropathy of neonatal onset multisystem inflammatory disease (NOMID/CINCA)|journal=Pediatric Radiology|volume=37|issue=2|year=2006|pages=145–152|issn=0301-0449|doi=10.1007/s00247-006-0358-0}}</ref> | ||
**Sensorineural hearing loss | **Sensorineural hearing loss | ||
**Hydrocephalus and increased intracranial pressure | **Hydrocephalus and increased intracranial pressure | ||
Revision as of 14:53, 3 July 2019
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Cryopyrin-associated periodic syndrome Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]
Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of neonatal-onset multisystem inflammatory disease (NOMID) usually develops during infancy, and start with symptoms such as continuous often low-grade fever, skin rash, neurologic involvement, and arthropathy.[1]
- The symptoms of (disease name) typically develop ___ years after exposure to ___.
- If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Complications
- Common complications of neonatal-onset multisystem inflammatory disease (NOMID) include:[2][3]
- Sensorineural hearing loss
- Hydrocephalus and increased intracranial pressure
- Optic nerve atrophy and progressive vision loss
- Cognitive delay
- Deforming arthropathy (30-40% 0f the cases)
- Renal amyloidosis
Prognosis
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
- Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
References
- ↑ Goldbach-Mansky, Raphaela; Dailey, Natalie J.; Canna, Scott W.; Gelabert, Ana; Jones, Janet; Rubin, Benjamin I.; Kim, H. Jeffrey; Brewer, Carmen; Zalewski, Christopher; Wiggs, Edythe; Hill, Suvimol; Turner, Maria L.; Karp, Barbara I.; Aksentijevich, Ivona; Pucino, Frank; Penzak, Scott R.; Haverkamp, Margje H.; Stein, Leonard; Adams, Barbara S.; Moore, Terry L.; Fuhlbrigge, Robert C.; Shaham, Bracha; Jarvis, James N.; O'Neil, Kathleen; Vehe, Richard K.; Beitz, Laurie O.; Gardner, Gregory; Hannan, William P.; Warren, Robert W.; Horn, William; Cole, Joe L.; Paul, Scott M.; Hawkins, Philip N.; Pham, Tuyet Hang; Snyder, Christopher; Wesley, Robert A.; Hoffmann, Steven C.; Holland, Steven M.; Butman, John A.; Kastner, Daniel L. (2006). "Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1β Inhibition". New England Journal of Medicine. 355 (6): 581–592. doi:10.1056/NEJMoa055137. ISSN 0028-4793.
- ↑ Sibley, Cailin H.; Plass, Nikki; Snow, Joseph; Wiggs, Edythe A.; Brewer, Carmen C.; King, Kelly A.; Zalewski, Christopher; Kim, H. Jeffrey; Bishop, Rachel; Hill, Suvimol; Paul, Scott M.; Kicker, Patrick; Phillips, Zachary; Dolan, Joseph G.; Widemann, Brigitte; Jayaprakash, Nalini; Pucino, Frank; Stone, Deborah L.; Chapelle, Dawn; Snyder, Christopher; Butman, John A.; Wesley, Robert; Goldbach-Mansky, Raphaela (2012). "Sustained response and prevention of damage progression in patients with neonatal-onset multisystem inflammatory disease treated with anakinra: A cohort study to determine three- and five-year outcomes". Arthritis & Rheumatism. 64 (7): 2375–2386. doi:10.1002/art.34409. ISSN 0004-3591.
- ↑ Hill, Suvimol Chirathivat; Namde, Madjimbaye; Dwyer, Andrew; Poznanski, Andrew; Canna, Scott; Goldbach-Mansky, Raphaela (2006). "Arthropathy of neonatal onset multisystem inflammatory disease (NOMID/CINCA)". Pediatric Radiology. 37 (2): 145–152. doi:10.1007/s00247-006-0358-0. ISSN 0301-0449.