Expert Agreement on EULAR/EUSTAR Recommendations for the Management of Systemic Sclerosis

MATERIALS AND METHODS

A survey was generated using Survey Monkey, which included the 14 EULAR/EUSTAR recommendations. Participants were asked to indicate how little or strongly they agreed with each recommendation on a 10-point scale, from 0 (not at all) to 9 (completely agree). The survey was sent to 118 participants; 3 opportunities were given to reply, and there were 66 replies (56% response rate). After excluding respondents not currently practicing in North America or Europe, 59 participants remained.

Mean agreement ranged from 5.3 to 8.9 out of 9, with a range of 40.3% to 98.3% agreement with various guidelines (as measured by the percentage of responses of 7, 8, or 9 on the 10-point scale) when North American and European rheumatologists were grouped together. Mean agreement of North American rheumatologists ranged from 5.2 to 8.9, with a range of 41.4% to 100% agreement. Mean agreement of European rheumatologists ranged from 5.5 to 8.8, with a range of 38.9% to 100% agreement.

RESULTS

Many but not all recommendations had strong support (Table 1). Experts from North America and Europe differed in the strength of agreement with digital vasculopathy recommendations, but iloprost is not available in North America and bosentan is not approved for digital ulcer prevention in North America. Pulmonary arterial hypertension (PAH) agreement was also different. We also studied agreement among the respondents who were authors of the EULAR/EUSTAR recommendations compared to the respondents who were not authors and found that the recommendation authors had significantly higher agreement (p = 0.006) with the use of proton pump inhibitors for prevention of SSc-related gastroesophageal reflux, esophageal ulcers, and strictures.

DISCUSSION

Despite high agreement for many recommendations, it is not known whether rheumatologists actually follow these recommendations. We surveyed only SSc experts, all of whom were aware of the SSc treatment recommendations. However, awareness of these recommendations by practicing rheumatologists and their actual adherence has not been studied. Sources of variability and limitation leading to decreased adherence may include practice size and experience, individual patient characteristics, geographic location and drug availability, health insurance, and the lack of a clear algorithm for the treatment and management of SSc.

Agreement was lowest in areas with randomized controlled trials (methotrexate for skin involvement and bosentan in the prevention of digital ulcers), which may be due to the modest efficacy or in the latter case, lack of availability of the drug for this indication^3,4,5,6. Further, the lack of agreement with the indicated uses of methotrexate and bosentan is independent of accessibility, as agreement was low for both North American and European rheumatologists (Table 1). As expected, access to treatment influenced agreement with recommendations regarding the use of iloprost. European rheumatologists favored the use of iloprost for active digital ulcers more than North American rheumatologists, while North American rheumatologists favored the use of epoprostenol more than their European counterparts, as indicated by a larger degree of agreement with its use in SSc-PAH (Table 1).

Lacking from these recommendations are guidelines for diagnostic and investigational procedures. Because of the low incidence and heterogeneity of SSc, such information would be extremely valuable.

There were 10 rheumatologists involved in the guidelines who also answered the survey, and if we remove them, the results do not change.

There are limitations of the recommendations in terms of the areas of SSc that are not addressed. For instance, there are no guidelines on treatment of pain, treatment of gastric antral vascular ectasia, maintenance therapy after interstitial lung disease is treated with cyclophosphamide, treatment of inflammatory arthritis, and therapy for erectile dysfunction; and nonpharmacologic treatment was not addressed.

The EULAR/EUSTAR recommendations for the treatment of SSc are relatively well accepted among the world’s SSc experts. How strongly rheumatologists follow these recommendations requires further investigation. The agreement was for the most part similar among experts who did and did not write the recommendations. There were some differences in agreement between European and North American rheumatologists, mainly because of variability in access to drugs or indications of some medications.