Diffusing capacity of the lungs for carbon monoxide (DLCO) percent predicted ()
72 (50-86)
64 (50-79)
Pulmonary hypertension estimated by echocardiogram ()
18 (17)
6 (16)
Pulmonary hypertension confirmed by right heart catheterization ()
8 (6%)
2 (5%)
Baseline dyspnea index ()
3 (1-4)
4 (2-8)
History of digital ischemic ulcers
11 (9%)
3 (8%)
Modified Rodnan skin score ()
4 (2-10)
6 (2-17)
Gastrointestinal manifestations‡
113 (88%)
36 (95%)
UCLA SCTC GIT 2.0 total score ()
0.5 (0.1-0.8)
0.8 (0.4-1.1)
History of scleroderma renal crisis ()
1 (1%)
0
Immunomodulatory medication use at enrollment
Mycophenolate or mycophenolic acid
48 (37%)
19 (50%)
Prednisone
19 (15%)
4 (10%)
Methotrexate
13 (10%)
4 (10%)
Hydroxychloroquine
11 (9%)
3 (8%)
Other
3 (2%)
2 (2%)
Patient global assessment
3 (2-6)
6 (4-7)
Health assessment questionnaire disability index ()
0.6 (0.1-1.4)
1.4 (0.8-1.9)
PROMIS-29 Profile v2.0 ()
Physical function
43 (37-57)
39 (32-42)
Anxiety
51 (40-60)
61 (56-65)
Depression
49 (41-56)
57 (54-62)
Fatigue
55 (46-63)
63 (59-67)
Sleep
51 (46-56)
58 (52-64)
Activities
51 (44-64)
44 (37-44)
Pain
54 (42-61)
61 (57-65)
By physician assessment at study intake, not all patients had high-resolution chest CT available in PRISM. Transthoracic echocardiogram with estimated pulmonary artery systolic . ‡Patients with any of the following gastrointestinal symptoms on baseline physician assessment: gastroesophageal reflux disease, esophageal dysmotility, esophageal stricture, small bowel hypomotility, or small intestine bacterial overgrowth. Percentages calculated with 129 as denominator, irrespective of missing data.
