Anakinra in Adult‐Onset Still's Disease: Long‐Term Treatment in Patients Resistant to Conventional Therapy

Abstract

Objective. Anakinra is effective in adult-onset Still's disease (AOSD) in the short term, but little is known regarding its efficacy over the long term. Our objective was to assess the long-term efficacy and safety of anakinra in AOSD. Methods. A nationwide survey was conducted between 2009 and 2010 to identify AOSD patients treated with anakinra. Collected data consisted of disease characteristics at diagnosis and at medication onset; anakinra efficacy, safety, and dose adaptation; and reasons for discontinuation, if applicable. Results. The study included 28 AOSD patients, with a mean age of 40.3 years and a mean disease duration at the start of anakinra of 9.3 years. All patients had previously failed to respond to steroids and disease-modifying antirheumatic drugs. All patients responded to anakinra, with a rapid and sustained decrease in steroid doses. At the last followup (mean 23 months), 16 patients were still being treated with anakinra: 4 had a partial response and 12 were in complete remission. Twelve patients had discontinued anakinra: 2 due to an insufficient response, 4 due to an AOSD flare after a period of complete remission, 2 due to side effects, and 1 due to a desire for pregnancy. In 3 patients, the drug discontinuation was possible because they achieved complete remission. Six additional patients experienced anakinra dose tapering, with sustained remission in 2 and relapse in the others. Anakinra was well tolerated and adverse events were rated as mild. Conclusion. Anakinra was consistently efficacious in AOSD and displayed good therapeutic maintenance. Anakinra dose tapering or discontinuation was associated with relapse in half of the patients.

Myocarditis in a young man with adult onset Still's disease successfully treated with Il-1 blocker

“…Unlike etanercept that requires prolonged treatment with the risk of developing opportunistic infections, anakinra shows quick answers sometimes within days or even hours, without increasing rates of infections [6]. However, this treatment must be continued for several months to avoid a risk of relapse [2].…”

Steroid-sparing Effects of Etanercept in a Patient with Steroid-dependent Adult-onset Still's Disease

“…Many reports have demonstrated the marked efficacy of anakinra, a recombinant human interleukin-1 receptor antagonist, in AOSD patients, with the efficacy being maintained over the long term (35). The drug, however, is not currently available in Japan.…”

Role of Interleukin 1 in the Development of Human Diseases

“…При обоих заболеваниях отмечено существенное уве-личение концентрации ИЛ1β в сыворотках пациентов [97][98][99]. Имеются данные о высокой эффективности инги-биторов ИЛ1 при болезни Стилла у детей [100,101] и у взрослых [96,97,[102][103][104][105][106][107][108][109][110][111][112][113][114][115][116][117]. Хотя при болезни Стилла у взрослых до сих пор чаще применялась анакинра [117], продемонстрирована высокая эффективность канакину-маба [106,111].…”

“…Как у детей, так и у взрослых ингибиторы ИЛ1 более эффективны у пациентов с так называемым «системным» вариантом болезни Стилла, по сравнению с «ревматическим» ее вариантом [95,96]. Действительно, ингибиторы ИЛ1 быстро купируют «системные» проявле-ния, но менее эффективны в отношении активности арт-рита и не замедляют прогрессирование деструкции суста-вов [96,103,108]. У многих пациентов, в том числе рефра-ктерных к стандартной терапии, может развиваться стой-кая ремиссия [96], хотя у некоторых из них после заверше-ния терапии наблюдалось обострение заболевания [103,108].…”

“…Действительно, ингибиторы ИЛ1 быстро купируют «системные» проявле-ния, но менее эффективны в отношении активности арт-рита и не замедляют прогрессирование деструкции суста-вов [96,103,108]. У многих пациентов, в том числе рефра-ктерных к стандартной терапии, может развиваться стой-кая ремиссия [96], хотя у некоторых из них после заверше-ния терапии наблюдалось обострение заболевания [103,108]. Активно изучается место ингибиторов ИЛ1 в лечении тяжелого осложнения болезни Стилла -синдрома актива-ции макрофагов.…”