Table 1.
Normal = 538 N (%) | Indeterminate = 118 N (%) | DD = 150 N (%) | P-value | |
---|---|---|---|---|
Female sex | 452 (84) | 96 (81) | 129 (86) | 0.59 |
Age at echo, years, median (IQR) | 54.3 (45.0, 62.6) | 65.4 (54.7, 72.1) | 67.7 (60.4, 74.2) | 0.0001 |
Race | ||||
Caucasian | 382 (71) | 74 (63) | 109 (73) | 0.028 |
Black | 115 (21) | 38 (32) | 37 (25) | |
Other‡ | 41 (8) | 6 (5) | 4 (3) | |
SSc duration at time of echo, from first RP or non-RP symptom, years, median (IQR) | 9.3 (4.6, 16.6) N=536 |
11.5 (5.5, 18.8) N=116 |
13.8 (7.7, 22.8) N=149 |
0.0001 |
SSc Subtype | ||||
Limited/Sine | 326 (61) | 74 (63) | 110 (73) | 0.016 |
Diffuse | 212 (39) | 44 (37) | 40 (27) | |
Autoantibodies – ever positive | ||||
ANA | 515/529 (97) | 113/116 (97) | 140/146 (96) | 0.63 |
Anti-Centromere | 166/531 (31) | 34 (29) | 50/146 (34) | 0.63 |
Anti-Scl70/Topoisomerase I | 153/531 (29) | 22/116 (19) | 28/145 (19) | 0.014 |
Anti-RNA Polymerase III | 96/491 (20) | 19/103 (18) | 22/137 (16) | 0.65 |
PM/Scl | 31/403 (8) | 5/93 (5) | 6/110 (5) | 0.58 |
Th/To | 12/403 (3) | 4/93 (4) | 2/110 (2) | 0.58 |
Fibrillarin/U3RNP | 13/403 (3) | 3/93 (3) | 3/110 (3) | 0.96 |
Ro/SSA | 102/526 (19) | 31/114 (27) | 41/144 (28) | 0.026 |
Traditional Risk Factors for DD | ||||
HTN | 207/532 (39) | 77/117 (66) | 90/145 (62) | <0.001 |
CAD | 17/532 (3) | 13 (11) | 25/146 (17) | <0.001 |
DM | 25/527 (5) | 8/115 (7) | 14/147 (10) | 0.085 |
Obesity (BMI ≥ 30kg/m2) | 86 (16) | 27 (23) | 38 (25) | 0.016 |
COPD | 46/531 (9) | 15/117 (13) | 26/141 (18) | 0.004 |
CKD | 72/530 (14) | 24/115 (21) | 41/148 (28) | <0.001 |
Dyslipidemia | 190/505 (38) | 43/110 (39) | 71/137 (52) | 0.010 |
Former/Current Tobacco Use | 208 (37) | 57 (48) | 80 (53) | 0.002 |
Clinical Characteristics§ | ||||
FVC ≤ 70% | 256/528 (48) | 72 (61) | 82/145 (57) | 0.022 |
DLCO ≤ 60% | 213/517 (41) | 75/114 (66) | 84/138 (61) | <0.001 |
PH by RHC | ||||
None | 22/59 (37) | 9/44 (20) | 10/56 (18) | 0.24 |
Pre-capillary | 30/59 (51) | 28/44 (63) | 38/56 (68) | |
Post-capillary | 4/59 (7) | 2/44 (5) | 4/56 (7) | |
Combined pre- and post-capillary | 3/59 (5) | 5/44 (11) | 4/56 (7) | |
Severe RP | 305/537 (57) | 73 (62) | 86/148 (58) | 0.60 |
Myopathy (≤ 4/5 MMT) | 123/534 (23) | 24 (20) | 29/148 (20) | 0.60 |
Severe GI | 287/537 (53) | 69/117 (59) | 77/148 (52) | 0.48 |
TFR | 98/537 (18) | 16 (14) | 22/149 (15) | 0.35 |
Synovitis | 116/537 (22) | 25 (21) | 36/149 (24) | 0.78 |
Telangiectasias | 494/536 (92) | 110 (93) | 141/149 (95) | 0.58 |
Calcinosis | 182/536 (34) | 48 (41) | 58/149 (39) | 0.27 |
SRC | 21 (4) | 4/117 (3) | 13 (9) | 0.040 |
Max mRSS, median (IQR) | 6 (3, 16) | 6.5 (4, 16) | 6 (3, 13.5) | 0.67 |
Includes Asian, Indian, Middle Eastern, Native American, Native Alaskan, Native Hawaiian/Pacific Islander, Unknown/Not reported
Clinical characteristics are reported as the maximum expressed at any timepoint up until time of echo.
Severe RP: ever presence of digital pitting scars, digital tip ulceration, or digital gangrene; Severe GI: high-dose GERD meds, antibiotics for bacterial overgrowth, malabsorption syndrome, episodes of pseudo-obstructions, or TPN requirement; TFR: tendon friction rubs; Myopathy: ≤ 4/5 manual muscle testing (MMT); SRC: scleroderma renal crisis; mRSS: modified Rodnan skin score