Table 2.
Type of Study Author Year (REF) | Total Cohort | PAH | ||||||
---|---|---|---|---|---|---|---|---|
N | Age-mean (SD) | % Female | % lcSSc | N | Age-Mean (SD) | % Female | % lcSSc | |
Cohort SSc | ||||||||
Biomarker | ||||||||
Avouac 2015 [15] | 161 | 58 (22–88)# | 84 | 60 | 13 | NA | NA | NA |
Chung 2016 [16] | 157 | 57.6 (10.4) | 86 | 67 | 16 | NA | NA | NA |
Iudici 2013 [13]* | 867 | 59 (12–89)# | 89 | 78 | 29 | NA | 90 | 93 |
Composite | ||||||||
Gladue 2013 [17]** | 347 | 57.2 (11.6) PHAROS 53.7 (11.8) Cochin | 84 | 60 | 69 | 62.4 (10.3) PHAROS 63.1 (12.9) Cochin | 84 | 75 |
Guillen de Castillo 2017 [18] | 83 | 62.4 (11.6) | 94 | 84 | 35 | 64.4 (10.8) | 97 | 89 |
Hao 2015 [19] | 73 | NA | 67 | 82 | 27 | 66.8 (8.3) | 93 | 85 |
Morrisroe 2016 [20] | 1579 | NA | NA | NA | 132 | 62.7 (10.3) | 85 | 69 |
Morrisroe 2017 [21] | 1636 | 57.2 (12.8) | 86 | 69 | 209 | 63.1 (10.4) | 87 | 74 |
Soukup 2016 [22] | 34 | 58.4 (11.7) | 88 | 41 | 7 | 71.3 (5.4) | 71 | 43 |
Thakkar 2013 [23] | 49 | NA | NR | NA | 17 | 56.4 (13.4) | 100 | 82 |
Vandecasteele 2017 [24] | 195 | 54 (42–62)# | 80 | 71 | 3 | NA | NA | 100 |
TTE | ||||||||
Meune 2016 [25] | 212 | 55.3 (13.2) | 81 | 51 | 27 | 63.3 (14.4) | 74 | NA |
Kusunose, 2015 [26] | 78 | 58 (12) | 91 | 70† | 16 | 60 (15) | 77 | NA |
Yoo 2016 [27] | 37 | 54 (9.9) | 76 | 68 | 4 | 58.5 (13.6) | 100 | 25 |
PFT | ||||||||
Sivova 2013 [28] | 63 | 55 (NA) | 81 | 79 | 6 | 68 (NA) | 100 | 83 |
CPET | ||||||||
Dumitrescu 2016 [29] | 173 | NA | 85 | NA | 48 | 67 (9) | 90 | NA |
Type of Study Author Year (REF) | Total Cohort | PAH | ||||||
N | Age-mean (SD) | % Female | % lcSSc | N | Age-Mean (SD) | % Female | % lcSSc | |
Case-control SSc | ||||||||
Biomarker | ||||||||
Kozij 2017 [30] | 16 | 51 (NA)# | 100 | 94 | 7 | 51 (NA)# | 71 | 71 |
Morrisroe 2014 [31] | 816 | NA | NA | NA | 124 | NA | NA | NA |
Nailfold capillaroscopy | ||||||||
Corrado 2017 [11] | 20 | 55.8 (11.4) | NA | NA | 19 | 48.1 (6.8) | NA | NA |
Case-control CTD | ||||||||
Biomarker | ||||||||
Hachulla 2017 (SLE) [32] | 101 | 46.9 (16.2) | 92 | NA | 51 | 47.6 (12.2) | 92 | NA |
Huang 2016 (SLE) [33] | 444 | 34.6 (8.5) | 97 | NA | 111 | 34.6 (8.6) | 97 | NA |
Tamura 2012 (CTD) [34] | 34 | 56.3 (2.7) | 91 | 59‡ | 17 | 56.3 (4.6) | 88 | NA |
Iudici 2013 also evaluated TTE and PFT parameters in a multivariate analysis [13].
Gladue 2013 also evaluated TTE and PFT parameters separately in addition to their combination [17].
Huang 2016 also evaluated various clinical manifestations [33].
Median (IQR).
70% had SSc.
59% had SSc
CTD- connective tissue disease, PAH – pulmonary arterial hypertension, SSc- systemic sclerosis, lcSSc – limited cutaneous systemic sclerosis, SLE – systemic lupus erythematosus, REF-reference, TTE – transthoracic echocardiography, PFT- pulmonary function test, CPET – cardiopulmonary exercise test, NA – not available