Results

The search strategy yielded XXX potential citations. After the removal of duplicates, initial screening and review for eligibility, XXX articles remained. A further XXX articles were identified through hand searching and Google Scholar. In total, XXX articles were mapped. After the removal of non-English studies and conference abstracts, XXX articles were included in the results summary (Appendix 2).

Mapping of studies

The characteristics of the included studies are presented in Table 1. There has been a linear increase in the number of studies about treatment of CAPS over the past XX years (1996–2000, n = 8; 2011–2015, n = 50). Most studies were based in Europe (XX of XX), in particular the U.K. (XX of XX), Sweden (XX of XX), Germany (XX of XX) and Turkey (XX of XX) (Table 1 and Figure XX).

What is the evidence about the efficacy of the drugs that act on interleukin-1 in the treatment of CAPS?

What are the genetic and environmental factors associated with variability in response to the drugs that act on interleukin-1 in the treatment of CAPS?

Genetic factors
Environmental factors