Interleukin-1 Targeting Drugs in Familial Mediterranean Fever: A Case Series and a Review of the Literature

Meinzer, Ulrich; Quartier, Pierre; Alexandra, Jean-François; Hentgen, Véronique; Rétornaz, F.; Koné‐Paut, Isabelle

doi:10.1016/j.semarthrit.2010.11.003

Cited by 181 publications

(104 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…For patients with other inherited autoinflammatory syndromes (28)(29)(30)(31)(32)(33)(34)(35), or more common inflammatory diseases such as heart disease and rheumatoid arthritis, IL-1 blockade has been a successful alternative to traditional therapy, even in patients previously receiving biologics (36)(37)(38). However, reports of patients with IL-1β-driven diseases that have failed IL-1-targeted therapy are emerging (39,40), suggesting that therapeutics aimed either at IL-18 or upstream at the level of the inflammasome may be valuable.…”

Section: Discussionmentioning

confidence: 99%

Divergence of IL-1, IL-18, and cell death in NLRP3 inflammasomopathies

Brydges¹,

Broderick²,

McGeough³

et al. 2013

J. Clin. Invest.

196

170

View full text Add to dashboard Cite

The inflammasome is a cytoplasmic multiprotein complex that promotes proinflammatory cytokine maturation in response to host-and pathogen-derived signals. Missense mutations in cryopyrin (NLRP3) result in a hyperactive inflammasome that drives overproduction of the proinflammatory cytokines IL-1β and IL-18, leading to the cryopyrin-associated periodic syndromes (CAPS) disease spectrum. Mouse lines harboring CAPS-associated mutations in Nlrp3 have elevated levels of IL-1β and IL-18 and closely mimic human disease. To examine the role of inflammasome-driven IL-18 in murine CAPS, we bred Nlrp3 mutations onto an Il18r-null background. Deletion of Il18r resulted in partial phenotypic rescue that abolished skin and visceral disease in young mice and normalized serum cytokines to a greater extent than breeding to Il1r-null mice. Significant systemic inflammation developed in aging Nlrp3 mutant Il18r-null mice, indicating that IL-1 and IL-18 drive pathology at different stages of the disease process. Ongoing inflammation in double-cytokine knockout CAPS mice implicated a role for caspase-1-mediated pyroptosis and confirmed that CAPS is inflammasome dependent. Our results have important implications for patients with CAPS and residual disease, emphasizing the need to explore other NLRP3-mediated pathways and the potential for inflammasome-targeted therapy.

show abstract

Section: Discussionmentioning

confidence: 99%

Divergence of IL-1, IL-18, and cell death in NLRP3 inflammasomopathies

Brydges¹,

Broderick²,

McGeough³

et al. 2013

J. Clin. Invest.

196

170

View full text Add to dashboard Cite

show abstract

“…The physiopathology of FMF involves the mutation of MFEV gene which induced a downregulation of innate immune system and an abnormal secretion of pro-inflammatory cytokines (IL1, IL6, IL18, TNFa). 4,6 In the physiopathology of DRESS, an elevation of cytokines to IL2, IFNc, TNFa is found. Actually, there is no known link between DRESS and FMF, but a virus could induce the two pathologies.…”

Section: Editormentioning

confidence: 99%

“…April is the month with highest precipitation in Luanda with a mean temperature of 26.7°C, both conditions required to increase the Paederus population. 6 Moreover, the beetle's natural predators, such as seabirds and marine mammals, have reduced their number in Angola. Climate change, global warming and waste accumulation could also increase the beetle population.…”

Section: Paederus Dermatitis Outbreak In Luanda City Angolamentioning

confidence: 99%

A familial mediterranean fever flare induced by a drug reaction with eosinophilia and systemic symptoms

Deschamps

Samain

Carvalho

et al. 2017

Acad Dermatol Venereol

View full text Add to dashboard Cite

“…In a boy with protracted febrile myalgia unresponsive to colchicine 1.5 mg/day was treated with anakinra 1 mg/kg/day with rapid resolution of clinical manifestations within 2 weeks [ 60 ]. Estubier C. et al described another patient with long-standing FMF and spondylarthritis who developed protracted myositis of quadriceps muscles unresponsive to colchicine and adalimumab.…”

Section: Anakinramentioning

confidence: 99%

“…Patients who are to start receiving anakinra are recommended to apply local treatment from the fi rst injection before they develop ISR. In the majority of the patients, this reaction disappears after a couple of weeks; however, the treatment is discontinued in some due to this side effect [ 60 ].…”

Section: Anakinramentioning

confidence: 99%

The Emerging Treatments in Familial Mediterranean Fever

Özdoğan

Uğurlu

2015

Rare Diseases of the Immune System

View full text Add to dashboard Cite

9As described in detail by Ben-Chetrit in the previous chapter, colchicine is the mainstay of the treatment of familial Mediterranean fever (FMF) [ 1 , 2 ]. If used daily for lifelong, it controls the febrile attacks in the majority of the patients and prevents the development of amyloidosis, the most serious complication of the disease.Colchicine is very effective as shown in controlled trials [ 3 ] and within the therapeutic range. Side effects are rare and/or mild, so that it can be used even during pregnancy and lactation, plus it is very cheap; however, even colchicine has some limitations in the treatment of FMF:1. Response to therapy: A small proportion of patients with FMF continue to experience attacks despite proper dose and good compliance to colchicine. Overall, it has been accepted that colchicine prevents febrile attacks in more than 60 % of patients and signifi cantly reduces the number of attacks in another 20-30 %. Five to ten percent of patients do not respond to therapy [ 4 , 5 ].Compliance is a main issue among patients who report attacks under colchicine treatment. Approximately 40 % of the patients are not compliant and receive lower than the dose needed to control the infl ammatory attacks and thus prone to high risk of amyloidosis [ 6 ]. Therefore, it is very important to check the compliance and the daily dose before diagnosing the patient as unresponsive to colchicine therapy. Recently the data that comes from the Eurofever Registry which collects information on patients with various autoinfl ammatory diseases, including FMF, showed that among the 121 FMF patients on colchicine, 62 % reported complete, 36 % partial, and 2 % no response [ 7 ]. In another recent report, only 5 % of the compliant patients on proper dose of colchicine continued to experience frequent attacks [ 8 ].

show abstract

Interleukin-1 Targeting Drugs in Familial Mediterranean Fever: A Case Series and a Review of the Literature

Cited by 181 publications

References 22 publications

Divergence of IL-1, IL-18, and cell death in NLRP3 inflammasomopathies

Divergence of IL-1, IL-18, and cell death in NLRP3 inflammasomopathies

A familial mediterranean fever flare induced by a drug reaction with eosinophilia and systemic symptoms

The Emerging Treatments in Familial Mediterranean Fever

Contact Info

Product

Resources

About