Table 1.
Demographic and Disease Characteristics of Entire anti-MDA5 Cohort, as well as those who experienced clinical remission. Ro52 antibody status was determined by Euroimmun. Physical examination findings are reported as ever/never recorded throughout longitudinal follow-up.
| Total N=51 |
Chronic Course (n=42) Median (IQR) |
Remission (n=9) Median (IQR) |
p-value | |
|---|---|---|---|---|
| Patient age at IIM symptom onset | 47.3 (39.7-54.3) | 47.6 (39.7-53.8) | 47.1 (42.1-59.9) | 0.770 |
| Patient age at JH Cohort Entry | 48.8 (41.1-55.3) | 49.2 (41.1-55.0) | 48.0 (43.3-61.3) | 0.880 |
| Male Sex | 27% | 26% | 33% | 0.690 |
| Race | 0.700 | |||
| Caucasian | 47% | 45% | 56% | |
| African American | 31% | 33% | 22% | |
| Asian | 6% | 7% | 0% | |
| Other | 10% | 7% | 22% | |
| Declined | 2% | 2% | 0% | |
| Unknown | 4% | 5% | 0% | |
| Ethnicity | 0.610 | |||
| Hispanic | 2% | 2% | 0% | |
| Not Hispanic | 86% | 83% | 100% | |
| Unknown | 12% | 14% | 0% | |
| Duration of follow-up | 3.7 (1.4-5.9) | 3.8 (1.7-6.3) | 1.8 (1.1-3.8) | 0.130 |
| Ro52 Positive | 42% | 38% | 56% | 0.460 |
| Gottron's sign or papules | 98% | 98% | 100% | 1.000 |
| Heliotrope Sign | 75% | 76% | 67% | 0.680 |
| Synovitis on physical examination | 57% | 57% | 56% | 1.000 |
| Calcinosis on physical examination | 35% | 36% | 33% | 1.000 |
| Elevated CPK | 15% | 19% | 0% | 0.320 |
| Maximum CPK | 101.0 (52.0-160.0) | 106.5 (52.0-177.0) | 56.0 (48.0-92.0) | 0.130 |
| Maximum aldolase | 8.7 (6.9-10.8) | 8.4 (6.9-10.8) | 9.1 (6.4-12.6) | 0.550 |
| History of malignancy (ever) | 12% | 12% | 13% | 1.000 |
| ILD on high-resolution CT | 80% | 81% | 78% | 1.000 |
| Nadir FVC over longitudinal follow-up | 70.5 (57.5-85.5) | 75.0 (59.0-86.0) | 59.0 (51.0-69.0) | 0.096 |
| Nadir TLC over longitudinal follow-up | 68.5 (59.0-79.0) | 70.0 (59.0-80.0) | 61.0 (58.0-72.5) | 0.320 |
| Nadir DLCO over longitudinal follow-up | 62.5 (45.5-78.5) | 62.0 (45.5-79.5) | 68.0 (51.0-78.0) | 0.600 |
| Ulcerations (composite) (digital ulcers, cutaneous ulcers, and mucosal ulcers) | 67% | 71% | 44% | 0.140 |
| Ischemic digital ulcers on examination or pits | 31% | 31% | 33% | 1.000 |
| Mucosal ulcerations (tongue, larynx, Vocal cords, mouth, NOT attributable to MTX or HS | 35% | 34% | 43% | 0.690 |
| Cutaneous ulcerations on skin (hands or rest of body) | 63% | 67% | 44% | 0.270 |
| Required intubation for rapidly progressive ILD (excludes elective/BAL/bx) | 12% | 14% | 0% | 0.570 |
| Spontaneous pneumothorax or pneumomediastinum | 8% | 10% | 0% | 1.000 |
| Proximal muscle weakness on examination | 73% | 79% | 44% | 0.093 |
| Received pulse dose solumedrol | 35% | 40% | 11% | 0.130 |
| Death | 10% | 12% | 0% | 0.570 |