Skip to main content

Table 1 Domains and measurement tools for the assessment of pulmonary arterial hypertension in systemic sclerosis

From: Systemic sclerosis-associated pulmonary hypertension: why disease-specific composite endpoints are needed

Domain Measurement tools Specific points to consider in application to PAH-SSca
Lung vascular Right heart catheter, echocardiography Other forms of PAH are prevalent in SSc, including type II (cardiac disease) and type III (lung fibrosis) and confound assessment, especially by echocardiography
Exercise testing 6MWD, oxygen saturation at exercise Musculoskeletal involvement and deconditioning have a major impact on exercise capacity in SSc in addition to PAH
Cardiac function Right heart catheter, echocardiography Cardiac abnormalities (diagnosed and undiagnosed) are prevalent in SSc, including high frequency of diastolic dysfunction
Dyspnea Dyspnea VAS There are multiple non-PAH causes for dyspnoea in SSc (lung fibrosis, deconditioning, anaemia, cardiac disease)
Discontinuation of treatment Adverse events, serious adverse events Concomitant medication for SSc or co-morbidity may impact on adverse events
Quality of life SF-36, HAQ DI Extra-pulmonary involvement has a major impact on health status and function in SSc
Global state by physician Survival SSc-PAH currently has a substantially worse mortality than iPAH
  1. aDomains identified through the EPOSS initiative may be integrated into the TTCW composite end-point and whether these may be different for idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc). 6MWD, 6-minute walking distance; HAQ DI = Health Assessment Questionnaire Disability Index; iPAH, idiopathic pulmonary arterial hypertension; PAH, pulmonary arterial hypertension; SF-36, Short Form 36 score; SSc, systemic sclerosis; VAS, visual analog scale.