Table 1 Demographic information for the patients with JDM in the CARRA Legacy Registry
Comparison of Global Assessment Scores | ||||
|---|---|---|---|---|
Variable | Overall Cohort (N = 563) | Concordant gVAS: (N = 344) | Discordant gVAS: PF gVAS > MD gVAS (N = 149) | Discordant gVAS: MD gVAS > PF gVAS (N = 70) |
Current Age, years Ϯ | 10.6 (6.9–14.7) | 10.8 (6.9–14.7) | 10.2 (6.7–14.2) | 10.4 (7.8–14.8) |
Age at Onset, years Ϯ | 5.5 (3.6–9.3) | 5.5 (3.6–9.2) | 5.3 (3.6–9.2) | 5.8 (3.6–10.0) |
Disease Duration, years Ϯ | 3.1 (1.3–6.4) | 3.4 (1.7–6.0) | 2.9 (1.3–6.8) | 2.8 (1.0–7.0) |
Gender, no. (%) | ||||
Male | 160 (28) | 99 (29) | 43 (29) | 18 (26) |
Female | 403 (72) | 245 (71) | 106 (71) | 52 (74) |
Race, no. (%) | ||||
Caucasian | 442 (79) | 280 (81) | 110 (74) | 52 (74) |
Black | 68 (12) | 38 (11) | 20 (13) | 10 (14) |
Asian | 20 (4) | 11 (3) | 7 (5) | 2 (3) |
Other | 33 (6) | 15 (5) | 12 (8) | 6 (9) |
Ethnicity, no. (%) | ||||
Non-Hispanic | 471 (84) | 287 (83) | 126 (85) | 58 (83) |
Hispanic | 92 (16) | 7 (17) | 23 (15) | 12 (17) |
Income Level, no. (%) | ||||
< $50,000 | 162 (36) | 90 (33) | 53 (44) | 19 (34) |
> $50,000 | 283 (64) | 180 (67) | 66 (56) | 37 (66) |
CMAS, no. (%) | ||||
< 48 | 122 (37) | 74 (35) | 29 (38) | 19 (44) |
> 48 | 211 (63) | 139 (65) | 48 (62) | 24 (56) |
Weakness, no. (%) a | ||||
None or Mild | 494 (89) | 311 (92) | 130 (90) | 53 (76) |
Moderate or Severe | 60 (11) | 28 (8) | 15 (10) | 17 (24) |
Muscle Enzyme, no. (%) a | ||||
Abnormal | 97 (18) | 48 (15) | 24 (17) | 25 (36) |
Normal | 446 (82) | 284 (85) | 117 (83) | 45 (64) |
JDM Rash, no. (%) a | ||||
Yes | 289 (52) | 160 (47) | 68 (47) | 61 (87) |
No | 265 (48) | 180 (53) | 76 (53) | 9 (13) |
Nailfold Changes, no. (%) a | ||||
Yes | 227 (42) | 126 (38) | 52 (37) | 49 (72) |
No | 313 (58) | 206 (62) | 88 (63) | 19 (28) |
Calcinosis, no. (%) a | ||||
Yes | 53 (10) | 18 (5) | 21 (15) | 14 (20) |
No | 487 (90) | 310 (95) | 121 (85) | 56 (80) |
GI-Cardiac, no. (%) | ||||
Yes | 17 (3) | 6 (2) | 7 (5) | 4 (6) |
No | 546 (97) | 338 (98) | 142 (95) | 66 (94) |
Joint Involvement, no. (%) a | ||||
Yes | 72 (13) | 34 (10) | 20 (14) | 18 (36) |
No | 481 (87) | 306 (90) | 124 (86) | 51 (64) |
Pulse Steroids Use, no. (%) a | ||||
Yes | 69 (14) | 32 (10) | 20 (15) | 17 (27) |
No | 441 (86) | 283 (90) | 113 (85) | 45 (73) |
Oral Steroids Use, no. (%) a | ||||
Yes | 261 (50) | 133 (42) | 84 (62) | 44 (69) |
No | 258 (50) | 187 (58) | 51 (38) | 20 (31) |
CHAQ Ϯ | 0 (0.0–0.63) | 0 (0.0–0.38) | 0.38 (0.0–1.09) | 0.13 (0.0–0.88) |
CMAS Strength | 50 (44–52) | 51 (45–52) | 49 (45–52) | 48 (37–52) |
Pain Score | 1 (0–3) | 0 (0–2) | 2 (0–5) | 0 (0–2) |
QoL Score a | ||||
Excellent | 129 (23) | 109 (32) | 5 (3) | 15 (21) |
Good or Very Good | 395 (71) | 217 (64) | 129 (88) | 49 (70) |
Poor | 31 (6) | 12 (4) | 13 (9) | 6 (9) |