Table 3.
Demographic and clinical features by prevalence of antibodies against various subunits of the Th/To complex. Patients with antibodies against any subunit tend to have limited cutaneous disease, a lower MRSS, and fewer TFR.
| hPOP1 N (%)/median (IQR) Negative (N=739) | Positive (N=65) | p-value | RPP25 N (%)/median (IQR) Negative (N=762) | Positive (N=42) | p-value | RPP30 N (%)/median (IQR) Negative (N=756) | Positive (N=48) | p-value | RPP40 N (%)/median (IQR) Negative (N=742) | Positive (N=62) | p-value | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Female Sex | 54 (83) | 621 (84) | 0.8600 | 640 (84) | 35 (83) | 0.8310 | 635 (84) | 40 (83) | 0.8410 | 624 (84) | 51 (82) | 0.7190 |
| Ethnicity | 1 (3) | 21 (5) | 1.0000 | 21 (5) | 1 (5) | 0.6130 | 21 (5) | 1 (5) | 1.0000 | 21 (5) | 1 (3) | 1.0000 |
| Caucasian | 52 (80) | 609 (82) | 0.8860 | 628 (82) | 33 (79) | 0.7460 | 621 (82) | 40(83) | 0.8880 | 609 (82) | 52 (84) | 0.9440 |
| African American | 11(17) | 94 (13) | 97 (13) | 8 (19) | 98 (13) | 7 (15) | 96 (13) | 9 (15) | ||||
| History of smoking (ever) | 347(48) | 39 (61) | 0.0500 | 358 (48) | 28 (68) | 0.0100 | 356 (48) | 30 (64) | 0.0350 | 348 (47) | 38 (62) | 0.0320 |
| Age at SSc-symptom onset (RP or non-RP) | 44 (33–55) | 42 (32–48) | 0.1200 | 44 (33–55) | 43 (37–51) | 0.6360 | 44 (33–55) | 44 (32–54) | 0.8740 | 44 (33–55) | 43 (32–53) | 0.5120 |
| Disease Duration Upon Cohort Entry | 5.6 (1.9–15) | 9.8 (4.2–16) | 0.1150 | 5.7 (1.9–15) | 9.8 (4.4–15) | 0.0760 | 6 (1.9–15) | 10 (3.6–16) | 0.0960 | 5.5 (1.9–15) | 10 (4.4–16) | 0.0230 |
| Cutaneous Subtype | ||||||||||||
| Sine | 26 (4) | 1 (2) | <0.0001 | 26 (3) | 1 (2) | <0.0001 | 26 (3) | 1 (2) | <0.0001 | 26 (4) | 1 (2) | <0.0001 |
| Limited | 356 (48) | 53 (82) | 376 (49) | 33 (79) | 369 (49) | 40 (83) | 358 (48) | 51 (82) | ||||
| Type II Skin | 74 (10) | 5 (8) | 74 (10) | 5 (12) | 74 (10) | 5 (10) | 74 (10) | 5 (8) | ||||
| Diffuse | 283 (38) | 6 (9) | 286 (38) | 3 (7) | 287 (38) | 2 (4) | 284 (38) | 5 (8) | ||||
| Cutaneous Subtype (Limited vs Diffuse) | 283 (38) | 6 (9) | <0.0001 | 286 (38) | 3 (7) | <0.0001 | 287 (38) | 2 (4) | <0.0001 | 284 (38) | 5 (8) | <0.0001 |
| Maximum MRSS | 8(4–19) | 4 (3–6) | <0.0001 | 7 (4–19) | 4 (2–6) | <0.0001 | 7 (4–19) | 4 (3–6) | <0.0001 | 8 (4–19) | 4 (3–6) | <0.0001 |
| History of scleroderma renal crisis | 39 (5) | 1 (2) | 0.2430 | 39 (5) | 1 (2) | 0.7160 | 39 (5) | 1 (2) | 0.5050 | 39 (5) | 1 (2) | 0.3560 |
| Minimum FVC % predicted over follow-up | 72 (56–86) | 69 (47–81) | 0.7910 | 72 (56–85) | 70 (52–81) | 0.7450 | 72 (56–86) | 66 (42–81) | 0.4420 | 72 (56–86) | 70 (46–82) | 0.6840 |
| Maximum Right Ventricular Systolic Pressure (mmHg) | 38 (32–50) | 40 (34–63) | 0.2020 | 38 (32–50) | 41 (34–65) | 0.3500 | 38 (32–50) | 42 (34–66) | 0.2250 | 38 (32–50) | 40 (34–63) | 0.1140 |
| Pulmonary Hypertension (mean PAP>20) | 100 (14) | 15 (23) | 0.0420 | 107 (14) | 8 (19) | 0.3650 | 104 (14) | 11 (23) | 0.0890 | 101 (14) | 14 (23) | 0.0590 |
| Pulmonary Hypertension (mean PAP>24) | 87 (12) | 14 (22) | 0.0310 | 93 (12) | 8 (20) | 0.2270 | 90 (12) | 11 (23) | 0.0400 | 88 (12) | 13 (21) | 0.0460 |
| Baseline TFR | 81 (11) | 0 (0) | 0.0010 | 81 (11) | 0 (0) | 0.0160 | 81 (11) | 0 (0) | 0.0110 | 81 (11) | 0 (0) | 0.0020 |
| Presence of synovitis on any exam | 153 (21) | 14 (22) | 0.8740 | 158 (21) | 9 (21) | 0.8480 | 156 (21) | 11 (23) | 0.7140 | 155 (21) | 12 (19) | 0.8710 |
| Deceased | 246 (34) | 24 (38) | 0.5830 | 256 (34) | 14 (34) | 1.0000 | 248 (33) | 22 (46) | 0.0850 | 246 (34) | 24 (39) | 0.4000 |
MRSS=Modified Rodnan Skin Score, RVSP=right ventricular systolic pressure, FVC = forced vital capacity, TFR= tendon friction rub, P-value: Fisher’s exact for binary and categorical variable comparisons; rank sum for non-parametric continuous variable comparisons.