Short summary

An international SSc expert meeting for the update of EULAR/EUSTAR recommendations for the treatment of SSc under the umbrella of EUSTAR was organized at the study site of the DeSScipher Project Coordinator in November 2014. Compared to the 14 EULAR/EUSTAR recommendations on the treatment of SSc published in 2009, a new set of 16 recommendations was developed which address treatment options for several SSc-related organ complications: Raynaud’s phenomenon, digital ulcers, pulmonary arterial hypertension, skin and lung disease, scleroderma renal crisis, and gastrointestinal involvement. Compared with the previous recommendations, the 2015 recommendations include phosphodiestase-5 inhibitors in the treatment of SSc-related RP and DUs, as well as riociguat and new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and the option of hematopoietic stem cell transplantation for selected patients with rapidly progressing SSc were added. In addition, a previous statement regarding sitaxentan for PAH was removed, because this drug has been withdrawn from the market. While the full-text paper of the update of the EULAR/EUSTAR recommendations has not been published yet, the updated recommendation have already been presented by the Project Coordinator Ulf Müller-Ladner to the public at the International Scleroderma Symposium in Berlin, September 2015, on the occasion of a special lecture and interactive discussion of symposium attendees with SSc experts of the EUSTAR recommendation task force.

In addition, preliminary recommendations based on the interim analysis of four of the five prospective OTs of the DeSScipher project have been developed and voted on. However, the wide variety in the rating of the recommendations by the expert panel reflected the limited amount of data that could be collected as the scientific basis for the preliminary set of recommendations until the end of the DeSScipher funding period. Therefore, updated final recommendations based on the outcome of the five OTs of DeSScipher will be facilitated once the follow-up of the OTs will be completed in November 2017, and are planned to be integrated into the next update of the EULAR-endorsed EULAR/EUSTAR recommendations on treatment of SSc.