Table 1

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

Domain

Measurement tools

Specific points to consider in application to PAH-SSc^a

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

^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.

Denton et al. Arthritis Research & Therapy 2011 13:114   doi:10.1186/ar3346