Cryopyrin-associated periodic syndrome medical therapy
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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
- Pharmacologic medical therapies for cryopyrin-associated periodic syndrome include (either) anakinra, canakinumab, and/or rilonacept.[1]
- Patients with NOMID are treated with anakinra, whereas patients with FCAS and MWS are treated with canakinumab.
- Symptomatic treatment options include:[2]
FCAS
- Anakinra has been observed to be effective in preventing the cold-induced attacks of FCAS.[3][4]
- Anakinra, has also been effective in reduction of proteinuria and stabilization of serum creatinin in patients with secondary amyloidosis.[5]
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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.