Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

Table 3 Patient-reported outcomes in patients with SSc-PAH compared to patients with SSc without PAH

Outcomes	PAH	No PAH	P value
SHAQ domain^a
Patient number	132	1447
Total score	3.2 ± 1.7	3.3 ± 2.1	0.74
Breathing	4.9 ± 2.5	2.3 ± 2.4	<0.001
Digital ulcers	1.9 ± 2.5	1.4 ± 2.4	0.02
Intestinal	2.8 ± 2.5	2.2 ± 2.4	0.002
Patient global assessment	5.1 ± 2.2	3.6 ± 2.4	<0.001
Pain	3.9 ± 2.6	3.4 ± 2.6	0.03
Vascular (RP)	3.3 ± 2.5	2.7 ± 2.5	0.01
SF-36 domain^b
Physical functioning	35.7 ± 23.8	57.5 ± 28.9	<0.001
Role limitation, physical	27.2 ± 39.1	49.2 ± 43.4	<0.001
Role limitation, emotional	55.9 ± 44.9	67.3 ± 40.4	0.05
Social functioning	64.2 ± 27.7	70.5 ± 26.9	0.07
Mental health	66.1 ± 21.1	68.9 ± 20.2	0.28
Energy/vitality	38.6 ± 22.2	47.1 ± 24.1	0.01
Bodily pain	55.3 ± 28.8	60.7 ± 27.9	0.10
General health perception	36.6 ± 20.5	46.2 ± 22.7	0.01
Physical component score	31.7 ± 8.7	38.9 ± 11.6	<0.001
Mental component score	46.3 ± 10.7	46.3 ± 10.4	0.48

Systemic sclerosis (SSc)-specific health assessment questionnaire (SHAQ) and Medical Outcomes Study Short Form-36 (SF-36) values are based on the average of all instrument scores collected annually in the database. For those with pulmonary hypertension (PAH), this includes only those scores that have been collected since PAH diagnosis. For those without PAH this includes all scores within the database for these patients. Raynaud's phenomenon (RP)
^aThe SHAQ is a generic instrument measuring functional outcome validated for use in SSc. The score ranges from 0 to 10, with 0 being no functional limitation and 10 being severe functional limitation
^bThe SF-36 form is a 36-item scale that measures eight domains of health status. The final score is standardised to the general population normative score of 50. The final score for each domain lies between 0 and 100, with 0 being the worst possible health and 100 the best possible health

ISSN: 1478-6362