Anakinra treatment in patients with adult-onset Still's disease is fast, effective, safe and steroid sparing: experience from an uncontrolled trial

Kalliolias, George D.; Georgiou, Panagiotis; Antonopoulos, Ioannis A; Andonopoulos, Andrew P.; Liossis, Stamatis–Nick C.

doi:10.1136/ard.2006.066381

Cited by 89 publications

(31 citation statements)

References 5 publications

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“…This is in line with some other reports showing a dramatic response to Anakinra within days of onset of therapy [33]. Other reports show that Anakinra can be effective in treatment-refractory Still's disease [34][35][36]. It would have been informative to observe the evolution of the PAH after a few weeks of treatment with this IL-1 antagonist.…”

Section: Discussionsupporting

confidence: 70%

STILL out of Breath?

Robillard

2013

J Med Cases

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Adult onset Still's disease (AOSD) is a rare inflammatory syndrome mostly seen in young adults. Known for its wide range of clinical manifestations, AOSD often presents with non-remitting systemic signs and symptoms. Many rare case associations have been described with AOSD, but only few with pulmonary hypertension (PAH). We are presenting a sixth known case of a young female adult with AOSD and PAH in the literature.

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Section: Discussionsupporting

confidence: 70%

STILL out of Breath?

Robillard

2013

J Med Cases

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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: 95%

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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“…Anakinra and IL-1 inhibitor have been studied in many case series [66][67][68][69][70] and appear to be effective in achieving remission in up to 80% of the cases vs 25% noted with TNF inhibitors.…”

Section: Treatmentmentioning

confidence: 95%