Table 2 Comments of the expert committee concerning research questions that did not yield a formal recommendation because of lack of appropriate evidence
Research questionComment from the expert committee
ACE inhibitors and sartans
1Do angiotensin receptor antagonists (sartans) have beneficial effects in systemic sclerosis?One RCT indicates that losartan may reduce the frequency and severity of RP attacks
Losartan may be recommended for the treatment of RP secondary to SSc
1Are steroids beneficial in SSc?The expert opinion is that a low dose of steroids is commonly used for the treatment of inflammatory arthritis in patients with SSc but its efficacy is not substantiated by RCT
1Does HSCT have beneficial effects in SSc?The cumulative phase I–II experience supports the use of HSCT in a selected poor-prognosis subgroup of SSc. Currently, transplantation should be performed only in the context of a RCT
1Is there any evidence of the beneficial effects of ciclosporine A in SSc?Two RCT with cyclophosphamide have reported efficacy on skin, quality of life and function
2Is there any evidence of the beneficial effects of mycophenolate mofetil in SSc?Uncontrolled and retrospectively controlled studies with some immunosuppressive regimens (such as azathioprine, mycophenolate mofetil, ciclosporine A) have reported efficacy in selected manifestations of SSc. Their efficacy has to be evaluated further in RCT (see box 1 Research agenda)
3Is there any evidence of the beneficial effects of azathioprine in SSc?Of note, experts believe that great caution is necessary when using cyclosporine because it may decrease renal function and induce hypertension
Other treatments
1Which drugs are beneficial in subcutaneous calcinosis in SSc?Drugs that improve calcinosis are currently lacking
2Is there any evidence that NSAID are harmful in SSc?Experts believe that NSAID are in general not more harmful in SSc than in the general population, but formal evidence is lacking. NSAID toxicity in the general population is well recognised
  • HSCT, haematopoietic stem cell transplantation; NSAID, non-steroidal anti-inflammatory drug; RCT, randomised controlled trial; RP, Raynaud’s phenomenon; SSc, systemic sclerosis.