Patients and physicians differently consider severity and outcomes of systemic sclerosis-associated interstitial lung disease: Results of an international survey

Objectives: We aimed to identify which measures are considered important by patients and physicians when assessing disease severity and outcomes in systemic sclerosis-associated interstitial lung disease. Methods: We designed an international survey based on a systematic literature review data and distributed it to patients with systemic sclerosis-associated interstitial lung disease and physicians with expertise in treating it. Survey participants rated on a scale of 1–10 the importance of each item defining severe systemic sclerosis-associated interstitial lung disease and outcomes considering the aim of the treatment, with higher values representing increasing importance. Items scored on average ⩾ 8 and voted ⩾ 8 by ⩾ 80% of participants were arbitrarily considered as highly ‘important’. Results: In general, 388 physicians and 350 patients completed the survey. Referral to lung transplantation, need for oxygen supplementation, interstitial lung disease extent and dyspnoea were important severity domains to both physicians and patients. Similarly, death, decline of pulmonary functional tests, increase of interstitial lung disease extent on high-resolution computed tomography and onset/worsening of dyspnoea were important treatment outcomes for both groups. We observed a discrepancy for both severity tools and outcomes, with patients deeming patients-reported outcomes domains and lung infections as more important than physicians did. Conclusion: The perspectives of patients and physicians partially overlap, when considering targets of treatment of systemic sclerosis-associated interstitial lung disease and items contributing to the perceived disease severity. Based on these insights, the opinions of both stakeholders should be included in defining the long-term targets of systemic sclerosis-associated interstitial lung disease treatment.