Efficacy of tocilizumab in refractory adult-onset still's disease: Multicenter retrospective open-label study of 34 patients

This study (n=34) reported that, after one year of tocilizumab therapy, joint manifestations had decreased from 97.1% to 32.4%, cutaneous from 58.8% to 5.9%, fever from 58.8% to 5.9%, and lymphadenopathy from 29.4% to 0%.

Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology, characterised by spiking fevers with an evanescent rash, arthritis, and multi-organ involvement.  It is rare and not readily diagnosed, and there is currently no consensus on its incidence and prevalence in different populations.

 

Treatment of AOSD has been empirical, with data on the efficacy of different treatments extrapolated from case reports and small retrospective studies.  The regimens used have been inconsistent with frequent qualitative descriptions of outcomes, which further confounds their interpretation.

 

Tocilizumab is not licensed to treat AOSD. Tocilizumab is an IL-6 inhibitor, licensed in the UK for the treatment of rheumatoid arthritis, active systemic juvenile idiopathic arthritis and juvenile idiopathic polyarthritis.

 

There are no national guidelines on the management of Still’s disease.