This protocol is of a systematic review for risk factors of pulmonary arterial hypertension in systemic sclerosis.

A systematic review was conducted to determine the risk/associated factors for pulmonary arterial hypertension in systemic sclerosis, including clinical/disease characteristics, antibody status, test results and biomarkers. The frequency of publications featuring a risk/association were reported. The following databases were searched: Medline, EMBASE, the Cochrane Library, Web of Science, and SCOPUS.

The following search terms were used:

( essential pulmonary hypertension or familial primary pulmonary hypertension or hypertension, pulmonary or hypertension,lung or hypertensive pulmonary vascular disease or idiopathic pulmonary arterial hypertension or lung arterial hypertension or lung artery hypertension or lung hypertension or primary pulmonary hypertension or pulmonary arterial hypertension or pulmonary artery hypertension or pulmonary fixed hypertension or pulmonary hypertensive disease or pulmonary hypertensive diseases or pulmonary hypertensive disorder or pulmonary hypertensive disorders )

AND

( generalised scleroderma or generalized scleroderma or progressive scleroderma or progressive sclerodermia or progressive sclerosis,systemic or progressive systemic sclerosis or scleroderma, generalised or scleroderma, generalized or scleroderma, progressive or scleroderma, systemic or sclerosis,progressive systemic or sclerosis,systemic or sclerosis,systemic progressive or systemic progressive sclerosis or systemic scleroderma or systemic sclerosis,progressive or

scleroderma, limited or limited scleroderma or limited cutaneous scleroderma or

diffuse scleroderma or progressive diffuse scleroderma or scleroderma, diffuse )

AND

( prevalence or prevalence study or relative risk or risk factors )

Inclusion Criteria:

• Studies were included they were (1) in patients with SSc; and if they (2) determined PAH with right heart catheterization; (3) involved a comparison of SSc patients with and without PAH; and (4) had sample size larger than 20.

Exclusion Criteria:

• Studies were excluded if they were review articles, and if the study was written in languages other than English. When the same patients were used in more than one analysis, the most recent or largest sample-size study was included.

The study reflects a search strategy of available literature on pulmonary arterial hypertension in scleroderma.