Treatment of Familial Mediterranean Fever with Anakinra

Belkhir, Rakiba; Moulonguet-Doleris, L.; Hachulla, É.; Prinseau, J; Baglin, A; Hanslik, Thomas

doi:10.7326/0003-4819-146-11-200706050-00023

Cited by 105 publications

(47 citation statements)

References 3 publications

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“…IL-1β is associated with autoinflammatory and autoimmune diseases in humans and plays a central role during the tissue destructive phase of these diseases. Moreover, IL-1 neutralization is very efficient in treating several destructive diseases such as systemic onset juvenile idiopathic arthritis, hereditary periodic fever syndromes, and gout arthritis (67)(68)(69)(70)(71)(72)(73)(74). This illustrates the pleiotropic role of IL-1β and the potential of IL-1 neutralization in the resolution of severe diseases.…”

Section: Figurementioning

confidence: 93%

IL-1R1/MyD88 signaling and the inflammasome are essential in pulmonary inflammation and fibrosis in mice

Gasse¹,

Mary²,

Guénon³

et al. 2007

J. Clin. Invest.

338

409

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The molecular mechanisms of acute lung injury resulting in inflammation and fibrosis are not well established. Here we investigate the roles of the IL-1 receptor 1 (IL-1R1) and the common adaptor for Toll/IL-1R signal transduction, MyD88, in this process using a murine model of acute pulmonary injury. Bleomycin insult results in expression of neutrophil and lymphocyte chemotactic factors, chronic inflammation, remodeling, and fibrosis. We demonstrate that these end points were attenuated in the lungs of IL-1R1-and MyD88-deficient mice. Further, in bone marrow chimera experiments, bleomycin-induced inflammation required primarily MyD88 signaling from radioresistant resident cells. Exogenous rIL-1β recapitulated a high degree of bleomycin-induced lung pathology, and specific blockade of IL-1R1 by IL-1 receptor antagonist dramatically reduced bleomycin-induced inflammation. Finally, we found that lung IL-1β production and inflammation in response to bleomycin required ASC, an inflammasome adaptor molecule. In conclusion, bleomycin-induced lung pathology required the inflammasome and IL-1R1/MyD88 signaling, and IL-1 represented a critical effector of pathology and therapeutic target of chronic lung inflammation and fibrosis.

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Section: Figurementioning

confidence: 93%

IL-1R1/MyD88 signaling and the inflammasome are essential in pulmonary inflammation and fibrosis in mice

Gasse¹,

Mary²,

Guénon³

et al. 2007

J. Clin. Invest.

338

409

View full text Add to dashboard Cite

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“…106 IL-1 inhibition represents an alternative option for the treatment of FMF. [107][108][109][110][111] Etanercept and sulfasalazine, respectively, may prove to be helpful, whereas thalidomide administration has yielded conflicting results. 106,[112][113][114][115][116] Deficiency of IL-1-RA syndrome…”

Section: Hyper-igd Syndromementioning

confidence: 94%

Autoinflammation: From monogenic syndromes to common skin diseases

Nguyen

Cowen

Leslie

2013

Journal of the American Academy of Dermatology

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Autoinflammation is characterized by aberrant regulation of the innate immune system and often manifests as periodic fevers and systemic inflammation involving multiple organs, including the skin. Mutations leading to abnormal behavior or activity of the interleukin 1 beta (IL-1ß)-processing inflammasome complex have been found in several rare autoinflammatory syndromes, for which anticytokine therapy such as IL-1 or tumor necrosis factor-alfa inhibition may be effective. It is becoming clear that features of autoinflammation also affect common dermatoses, some of which were previously thought to be solely autoimmune in origin (eg, vitiligo, systemic lupus erythematosus). Recognizing the pathogenetic role of autoinflammation can open up new avenues for the targeted treatment of complex, inflammatory dermatoses.

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“…Several reports indicate that this offers a relatively safe and effective treatment (100 mg daily or every other day) for persons who do not respond to colchicine. [131][132][133][134][135][136][137][138] This drug is expensive and has mild side effects, such as painful local reactions at the site of injections and possibly bronchopulmonary infection complications, especially in persons with other risk factors for pulmonary infections. Further studies are needed to investigate the long-term effects and side effects of this drug if it is to be taken continuously as required in severely affected individuals with FMF.…”

Section: Newer Therapiesmentioning

confidence: 99%