Adult onset Still's disease-The evidence that anti-interleukin-1 treatment is effective and well-tolerated (a comprehensive literature review).

The literature contains many reports of the use of commercially available anti-IL-1 agents (anakinra/Kineret® , canakinumab/Ilaris® , or rilonacept/Arcalyst® ) in treatment-resistant adult-onset Still's disease (AOSD). These have been widely summarized in many review articles, but a full account of all reports with each of the agents used is not available. This literature review includes all reports of treatment outcomes in patients treated for AOSD with any commercially available anti-IL-1 agent (excluding cases of unconfirmed or atypical AOSD or treatments only for rare AOSD complications). The summary makes use of tabular formats, to identify the available reports and to provide data for compiling and comparison to classical therapies. For each anti-IL-1 agent used, a table shows the frequency of remission during treatment and the frequency of stopping or reducing steroid use, which were reported in almost all articles. A brief textual summary is used to describe other relevant but less often described efficacy aspects and any safety information. The compiled data show that treatment with all anti-IL-1 agents is effective in AOSD, indicating that IL-1 has a central role in the pathogenesis of AOSD. Rates of full or partial remission with each agent were similar to each other (91-100%) and superior to the outcomes published for classical therapies. Primary treatment failures were rare, but efficacy was lost over time in some cases. Of note, the newer anti-IL-1 agents with longer half-lives may show prolonged efficacy. An articular involvement seems to be less responsive than systemic features of disease. However, long-term follow-up shows that efficacy may persist for many years. There is substantial evidence that anti-IL-1 agents have a strong steroid-sparing effect and considerable evidence that the use of disease-modifying anti-rheumatic drugs can also be reduced or stopped. Thus, the use of anti-IL-1 agents may reduce the side-effects of co-treatment. The high response rate to anti-IL-1 agents, especially in refractory AOSD cases, suggests that their appropriate use in a timely manner can slow disease progression and reduce treatment side-effects.