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==Overview==
==Overview==
There is no definitive treatment for cryopyrin-associated periodic syndrome; the mainstay of therapy is supportive care. Supportive therapy for cryopyrin-associated periodic syndrome includes anakinra, rilonacept, and canakinumab. Patients with NOMID are treated with anakinra, whereas patients with FCAS and MWS are treated with canakinumab.
There is no definitive treatment for cryopyrin-associated periodic syndrome; the mainstay of therapy is supportive care. Supportive therapy for cryopyrin-associated periodic syndrome includes anakinra, rilonacept, and canakinumab. [[Patients]] with NOMID are treated with anakinra, whereas patients with FCAS and MWS are treated with canakinumab. [[Symptomatic]] treatment options include [[nonsteroidal anti-inflammatory drugs]], [[disease-modifying antirheumatic drugs]], and [[glucocorticoids]].
==Medical Therapy==  
==Medical Therapy==  
*Pharmacologic medical therapies for cryopyrin-associated periodic syndrome include (either) anakinra, canakinumab, and/or rilonacept.
*Pharmacologic medical therapies for cryopyrin-associated periodic syndrome include (either) anakinra, canakinumab, and/or rilonacept.<ref name="pmid27143789">{{cite journal |vauthors=Kullenberg T, Löfqvist M, Leinonen M, Goldbach-Mansky R, Olivecrona H |title=Long-term safety profile of anakinra in patients with severe cryopyrin-associated periodic syndromes |journal=Rheumatology (Oxford) |volume=55 |issue=8 |pages=1499–506 |date=August 2016 |pmid=27143789 |pmc=4957676 |doi=10.1093/rheumatology/kew208 |url=}}</ref>
*Patients with NOMID are treated with anakinra, whereas patients with FCAS and MWS are treated with canakinumab.
*Patients with NOMID are treated with anakinra, whereas patients with FCAS and MWS are treated with canakinumab.
===Disease Name===
*Symptomatic treatment options include:<ref name="HoffmanSimon2009">{{cite journal|last1=Hoffman|first1=Hal M.|last2=Simon|first2=Anna|title=Recurrent febrile syndromes—what a rheumatologist needs to know|journal=Nature Reviews Rheumatology|volume=5|issue=5|year=2009|pages=249–256|issn=1759-4790|doi=10.1038/nrrheum.2009.40}}</ref>
 
**[[Nonsteroidal anti-inflammatory drugs]]
* '''1 Stage 1 - Name of stage'''
**[[Disease-modifying antirheumatic drugs]]
** 1.1 '''Specific Organ system involved 1'''
**[[Glucocorticoids]]
*** 1.1.1 '''Adult'''
===FCAS===
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
*Anakinra has been observed to be effective in preventing the cold-induced attacks of FCAS.<ref name="pmid18174231">{{cite journal |vauthors=Maksimovic L, Stirnemann J, Caux F, Ravet N, Rouaghe S, Cuisset L, Letellier E, Grateau G, Morin AS, Fain O |title=New CIAS1 mutation and anakinra efficacy in overlapping of Muckle-Wells and familial cold autoinflammatory syndromes |journal=Rheumatology (Oxford) |volume=47 |issue=3 |pages=309–10 |date=March 2008 |pmid=18174231 |doi=10.1093/rheumatology/kem318 |url=}}</ref><ref name="pmid15541451">{{cite journal |vauthors=Hoffman HM, Rosengren S, Boyle DL, Cho JY, Nayar J, Mueller JL, Anderson JP, Wanderer AA, Firestein GS |title=Prevention of cold-associated acute inflammation in familial cold autoinflammatory syndrome by interleukin-1 receptor antagonist |journal=Lancet |volume=364 |issue=9447 |pages=1779–85 |date=2004 |pmid=15541451 |pmc=4321997 |doi=10.1016/S0140-6736(04)17401-1 |url=}}</ref>
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
*Anakinra, has also been effective in reduction of [[proteinuria]] and stabilization of serum [[creatinin]] in [[patients]] with secondary [[amyloidosis]].<ref name="pmid17336710">{{cite journal |vauthors=Thornton BD, Hoffman HM, Bhat A, Don BR |title=Successful treatment of renal amyloidosis due to familial cold autoinflammatory syndrome using an interleukin 1 receptor antagonist |journal=Am. J. Kidney Dis. |volume=49 |issue=3 |pages=477–81 |date=March 2007 |pmid=17336710 |doi=10.1053/j.ajkd.2006.10.026 |url=}}</ref>
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
===MWS===
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
*Anakinra has also been reported to be effective in reduction of systemic [[symptoms]] of MWS, and [[amyloidosis]] risk.<ref name="pmid14872505">{{cite journal |vauthors=Hawkins PN, Lachmann HJ, Aganna E, McDermott MF |title=Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra |journal=Arthritis Rheum. |volume=50 |issue=2 |pages=607–12 |date=February 2004 |pmid=14872505 |doi=10.1002/art.20033 |url=}}</ref>
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
*However, its efficacy in the prevention of hearing loss sounds partial.<ref name="pmid18311804">{{cite journal |vauthors=Yamazaki T, Masumoto J, Agematsu K, Sawai N, Kobayashi S, Shigemura T, Yasui K, Koike K |title=Anakinra improves sensory deafness in a Japanese patient with Muckle-Wells syndrome, possibly by inhibiting the cryopyrin inflammasome |journal=Arthritis Rheum. |volume=58 |issue=3 |pages=864–8 |date=March 2008 |pmid=18311804 |doi=10.1002/art.23261 |url=}}</ref>
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
===NOMID===
*** 1.1.2 '''Pediatric'''
*Anakinra been observed to be effective in the treatment of NOMID, but it sounds to be less effective for [[bone]] and [[joint]] abnormalities.
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
===Rilonacept===
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
*Rilonacept, an interleukin (IL) 1 trap, is able to reduce the symptoms, signs, and inflammatory markers associated with CAPS.<ref name="pmid18668535">{{cite journal |vauthors=Hoffman HM, Throne ML, Amar NJ, Sebai M, Kivitz AJ, Kavanaugh A, Weinstein SP, Belomestnov P, Yancopoulos GD, Stahl N, Mellis SJ |title=Efficacy and safety of rilonacept (interleukin-1 Trap) in patients with cryopyrin-associated periodic syndromes: results from two sequential placebo-controlled studies |journal=Arthritis Rheum. |volume=58 |issue=8 |pages=2443–52 |date=August 2008 |pmid=18668535 |doi=10.1002/art.23687 |url=}}</ref>
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
===Canakinumab===
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
*Canakinumab, a human anti-IL-1 beta monoclonal antibody, has successfully improved NOMID symptoms, however, its efficacy was partial in case of aseptic meningitis.<ref name="pmid24906637">{{cite journal |vauthors=Sibley CH, Chioato A, Felix S, Colin L, Chakraborty A, Plass N, Rodriguez-Smith J, Brewer C, King K, Zalewski C, Kim HJ, Bishop R, Abrams K, Stone D, Chapelle D, Kost B, Snyder C, Butman JA, Wesley R, Goldbach-Mansky R |title=A 24-month open-label study of canakinumab in neonatal-onset multisystem inflammatory disease |journal=Ann. Rheum. Dis. |volume=74 |issue=9 |pages=1714–9 |date=September 2015 |pmid=24906637 |pmc=4258169 |doi=10.1136/annrheumdis-2013-204877 |url=}}</ref>
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose) 
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
** 1.2 '''Specific Organ system involved 2'''
*** 1.2.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
*** 1.2.2  '''Pediatric'''
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
 
* 2 '''Stage 2 - Name of stage'''
** 2.1 '''Specific Organ system involved 1 '''
**: '''Note (1):'''
**: '''Note (2)''':
**: '''Note (3):'''
*** 2.1.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.1.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
**: '''Note (1):'''
**: '''Note (2)''':
**: '''Note (3):'''
*** 2.2.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.2.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
 
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 21:44, 16 July 2019

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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

There is no definitive treatment for cryopyrin-associated periodic syndrome; the mainstay of therapy is supportive care. Supportive therapy for cryopyrin-associated periodic syndrome includes anakinra, rilonacept, and canakinumab. Patients with NOMID are treated with anakinra, whereas patients with FCAS and MWS are treated with canakinumab. Symptomatic treatment options include nonsteroidal anti-inflammatory drugs, disease-modifying antirheumatic drugs, and glucocorticoids.

Medical Therapy

FCAS

MWS

  • Anakinra has also been reported to be effective in reduction of systemic symptoms of MWS, and amyloidosis risk.[6]
  • However, its efficacy in the prevention of hearing loss sounds partial.[7]

NOMID

  • Anakinra been observed to be effective in the treatment of NOMID, but it sounds to be less effective for bone and joint abnormalities.

Rilonacept

  • Rilonacept, an interleukin (IL) 1 trap, is able to reduce the symptoms, signs, and inflammatory markers associated with CAPS.[8]

Canakinumab

  • Canakinumab, a human anti-IL-1 beta monoclonal antibody, has successfully improved NOMID symptoms, however, its efficacy was partial in case of aseptic meningitis.[9]

References

  1. ↑ Kullenberg T, Löfqvist M, Leinonen M, Goldbach-Mansky R, Olivecrona H (August 2016). "Long-term safety profile of anakinra in patients with severe cryopyrin-associated periodic syndromes". Rheumatology (Oxford). 55 (8): 1499–506. doi:10.1093/rheumatology/kew208. PMC 4957676. PMID 27143789.
  2. ↑ Hoffman, Hal M.; Simon, Anna (2009). "Recurrent febrile syndromes—what a rheumatologist needs to know". Nature Reviews Rheumatology. 5 (5): 249–256. doi:10.1038/nrrheum.2009.40. ISSN 1759-4790.
  3. ↑ Maksimovic L, Stirnemann J, Caux F, Ravet N, Rouaghe S, Cuisset L, Letellier E, Grateau G, Morin AS, Fain O (March 2008). "New CIAS1 mutation and anakinra efficacy in overlapping of Muckle-Wells and familial cold autoinflammatory syndromes". Rheumatology (Oxford). 47 (3): 309–10. doi:10.1093/rheumatology/kem318. PMID 18174231.
  4. ↑ Hoffman HM, Rosengren S, Boyle DL, Cho JY, Nayar J, Mueller JL, Anderson JP, Wanderer AA, Firestein GS (2004). "Prevention of cold-associated acute inflammation in familial cold autoinflammatory syndrome by interleukin-1 receptor antagonist". Lancet. 364 (9447): 1779–85. doi:10.1016/S0140-6736(04)17401-1. PMC 4321997. PMID 15541451.
  5. ↑ Thornton BD, Hoffman HM, Bhat A, Don BR (March 2007). "Successful treatment of renal amyloidosis due to familial cold autoinflammatory syndrome using an interleukin 1 receptor antagonist". Am. J. Kidney Dis. 49 (3): 477–81. doi:10.1053/j.ajkd.2006.10.026. PMID 17336710.
  6. ↑ Hawkins PN, Lachmann HJ, Aganna E, McDermott MF (February 2004). "Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra". Arthritis Rheum. 50 (2): 607–12. doi:10.1002/art.20033. PMID 14872505.
  7. ↑ Yamazaki T, Masumoto J, Agematsu K, Sawai N, Kobayashi S, Shigemura T, Yasui K, Koike K (March 2008). "Anakinra improves sensory deafness in a Japanese patient with Muckle-Wells syndrome, possibly by inhibiting the cryopyrin inflammasome". Arthritis Rheum. 58 (3): 864–8. doi:10.1002/art.23261. PMID 18311804.
  8. ↑ Hoffman HM, Throne ML, Amar NJ, Sebai M, Kivitz AJ, Kavanaugh A, Weinstein SP, Belomestnov P, Yancopoulos GD, Stahl N, Mellis SJ (August 2008). "Efficacy and safety of rilonacept (interleukin-1 Trap) in patients with cryopyrin-associated periodic syndromes: results from two sequential placebo-controlled studies". Arthritis Rheum. 58 (8): 2443–52. doi:10.1002/art.23687. PMID 18668535.
  9. ↑ Sibley CH, Chioato A, Felix S, Colin L, Chakraborty A, Plass N, Rodriguez-Smith J, Brewer C, King K, Zalewski C, Kim HJ, Bishop R, Abrams K, Stone D, Chapelle D, Kost B, Snyder C, Butman JA, Wesley R, Goldbach-Mansky R (September 2015). "A 24-month open-label study of canakinumab in neonatal-onset multisystem inflammatory disease". Ann. Rheum. Dis. 74 (9): 1714–9. doi:10.1136/annrheumdis-2013-204877. PMC 4258169. PMID 24906637.

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