| Clinical feature during flare . | (a) Patient-reported flare (n = 123), N (%) . | (b) Flare by need for IS dose increase (n = 163), N (%) . | (c) Clinical flare (n = 112), N (%) . | (d) Flare by PROMIS PF10a 7.9-point worsening (n = 16), N (%) . |
|---|---|---|---|---|
| Flare prevalence (%) | 9.6 | 12.7 | 8.7 | 19.6 |
| Rashes | 41 (33.3) | 28 (17.2) | 41 (36.6) | 0 (0) |
| Muscle weakness | 85 (69.1) | 47 (28.8) | 85 (75.9) | 1 (6.3) |
| Muscle pain | 51 (41.5) | 42 (25.8) | 68 (60.7) | 1 (6.3) |
| Arthritis of hands | 54 (43.9) | 37 (22.7) | 54 (48.2) | 1 (6.3) |
| Pain in shoulders and hips | 44 (35.8) | 30 (18.4) | 42 (37.5) | 1 (6.3) |
| Arthritis of other joints | 36 (29.3) | 20 (12.3) | 36 (32.1) | 0 (0) |
| Raynaud’s | 23 (18.7) | 14 (8.6) | 22 (19.6) | 0 (0) |
| Skin tightening of hands | 12 (9.8) | 7 (4.3) | 12 (10.7) | 0 (0) |
| Skin tightening in new areas | 7 (5.7) | 5 (3.1) | 7 (6.3) | 0 (0) |
| Finger-tip ulcers | 7 (5.7) | 3 (1.8) | 7 (6.3) | 0 (0) |
| Shortness of breath | 33 (26.8) | 20 (12.3) | 27 (24.1) | 0 (0) |
| Chest pain | 15 (12.2) | 7 (4.3) | 14 (12.5) | 0 (0) |
| Difficulty in swallowing | 20 (16.3) | 13 (8.0) | 20 (17.9) | 0 (0) |
| Fever | 11 (8.9) | 6 (3.7) | 10 (8.9) | 0 (0) |
| Fatigue | 70 (56.9) | 39 (23.9) | 67 (59.8) | 0 (0) |
| Dry eyes | 18 (14.6) | 15 (9.2) | 18 (16.1) | 0 (0) |
| Dry mouth | 13 (10.6) | 7 (4.3) | 13 (11.6) | 0 (0) |
| Oral/nasal ulcers | 8 (6.5) | 6 (3.7) | 7 (6.3) | 0 (0) |
| Severe hair loss | 10 (8.1) | 7 (4.3) | 10 (14.3) | 0 (0) |
| Headache | 17 (13.8) | 6 (3.7) | 16 (14.3) | 0 (0) |
| Active kidney disease | 2 (1.6) | 1 (0.6) | 1 (0.9) | 0 (0) |
| Elevated muscle enzymes | 23 (18.7) | 16 (9.8) | 22 (19.6) | 0 (0) |
| Elevated inflammatory markers | 20 (16.3) | 14 (8.6) | 19 (17.0) | 0 (0) |
| Types of rashes | ||||
| Heliotrope | 14 (11.4) | 11 (6.7) | 14 (12.5) | 0 (0) |
| Gottron’s over knuckles | 22 (17.9) | 16 (9.8) | 22 (19.6) | 0 (0) |
| Gottron’s over knees | 10 (8.1) | 6 (3.7) | 10 (8.9) | 0 (0) |
| Holster sign | 6 (4.9) | 4 (2.5) | 6 (5.4) | 0 (0) |
| Malar rash | 11 (8.9) | 9 (5.5) | 11 (9.8) | 0 (0) |
| V sign | 17 (13.8) | 14 (8.6) | 17 (15.2) | 0 (0) |
| Forearm rashes | 17 (13.8) | 11 (6.7) | 17 (15.2) | 0 (0) |
| Mechanic’s hand | 9 (7.3) | 7 (4.3) | 9 (8.0) | 0 (0) |
| Clinical feature during flare . | (a) Patient-reported flare (n = 123), N (%) . | (b) Flare by need for IS dose increase (n = 163), N (%) . | (c) Clinical flare (n = 112), N (%) . | (d) Flare by PROMIS PF10a 7.9-point worsening (n = 16), N (%) . |
|---|---|---|---|---|
| Flare prevalence (%) | 9.6 | 12.7 | 8.7 | 19.6 |
| Rashes | 41 (33.3) | 28 (17.2) | 41 (36.6) | 0 (0) |
| Muscle weakness | 85 (69.1) | 47 (28.8) | 85 (75.9) | 1 (6.3) |
| Muscle pain | 51 (41.5) | 42 (25.8) | 68 (60.7) | 1 (6.3) |
| Arthritis of hands | 54 (43.9) | 37 (22.7) | 54 (48.2) | 1 (6.3) |
| Pain in shoulders and hips | 44 (35.8) | 30 (18.4) | 42 (37.5) | 1 (6.3) |
| Arthritis of other joints | 36 (29.3) | 20 (12.3) | 36 (32.1) | 0 (0) |
| Raynaud’s | 23 (18.7) | 14 (8.6) | 22 (19.6) | 0 (0) |
| Skin tightening of hands | 12 (9.8) | 7 (4.3) | 12 (10.7) | 0 (0) |
| Skin tightening in new areas | 7 (5.7) | 5 (3.1) | 7 (6.3) | 0 (0) |
| Finger-tip ulcers | 7 (5.7) | 3 (1.8) | 7 (6.3) | 0 (0) |
| Shortness of breath | 33 (26.8) | 20 (12.3) | 27 (24.1) | 0 (0) |
| Chest pain | 15 (12.2) | 7 (4.3) | 14 (12.5) | 0 (0) |
| Difficulty in swallowing | 20 (16.3) | 13 (8.0) | 20 (17.9) | 0 (0) |
| Fever | 11 (8.9) | 6 (3.7) | 10 (8.9) | 0 (0) |
| Fatigue | 70 (56.9) | 39 (23.9) | 67 (59.8) | 0 (0) |
| Dry eyes | 18 (14.6) | 15 (9.2) | 18 (16.1) | 0 (0) |
| Dry mouth | 13 (10.6) | 7 (4.3) | 13 (11.6) | 0 (0) |
| Oral/nasal ulcers | 8 (6.5) | 6 (3.7) | 7 (6.3) | 0 (0) |
| Severe hair loss | 10 (8.1) | 7 (4.3) | 10 (14.3) | 0 (0) |
| Headache | 17 (13.8) | 6 (3.7) | 16 (14.3) | 0 (0) |
| Active kidney disease | 2 (1.6) | 1 (0.6) | 1 (0.9) | 0 (0) |
| Elevated muscle enzymes | 23 (18.7) | 16 (9.8) | 22 (19.6) | 0 (0) |
| Elevated inflammatory markers | 20 (16.3) | 14 (8.6) | 19 (17.0) | 0 (0) |
| Types of rashes | ||||
| Heliotrope | 14 (11.4) | 11 (6.7) | 14 (12.5) | 0 (0) |
| Gottron’s over knuckles | 22 (17.9) | 16 (9.8) | 22 (19.6) | 0 (0) |
| Gottron’s over knees | 10 (8.1) | 6 (3.7) | 10 (8.9) | 0 (0) |
| Holster sign | 6 (4.9) | 4 (2.5) | 6 (5.4) | 0 (0) |
| Malar rash | 11 (8.9) | 9 (5.5) | 11 (9.8) | 0 (0) |
| V sign | 17 (13.8) | 14 (8.6) | 17 (15.2) | 0 (0) |
| Forearm rashes | 17 (13.8) | 11 (6.7) | 17 (15.2) | 0 (0) |
| Mechanic’s hand | 9 (7.3) | 7 (4.3) | 9 (8.0) | 0 (0) |
IIM: idiopathic inflammatory myopathies; IS: immunosuppression; PROMIS PF10a: Patient-Reported Outcomes Measurement Information System 10 Item Patient-Reported Measure of Physical Function.
| Clinical feature during flare . | (a) Patient-reported flare (n = 123), N (%) . | (b) Flare by need for IS dose increase (n = 163), N (%) . | (c) Clinical flare (n = 112), N (%) . | (d) Flare by PROMIS PF10a 7.9-point worsening (n = 16), N (%) . |
|---|---|---|---|---|
| Flare prevalence (%) | 9.6 | 12.7 | 8.7 | 19.6 |
| Rashes | 41 (33.3) | 28 (17.2) | 41 (36.6) | 0 (0) |
| Muscle weakness | 85 (69.1) | 47 (28.8) | 85 (75.9) | 1 (6.3) |
| Muscle pain | 51 (41.5) | 42 (25.8) | 68 (60.7) | 1 (6.3) |
| Arthritis of hands | 54 (43.9) | 37 (22.7) | 54 (48.2) | 1 (6.3) |
| Pain in shoulders and hips | 44 (35.8) | 30 (18.4) | 42 (37.5) | 1 (6.3) |
| Arthritis of other joints | 36 (29.3) | 20 (12.3) | 36 (32.1) | 0 (0) |
| Raynaud’s | 23 (18.7) | 14 (8.6) | 22 (19.6) | 0 (0) |
| Skin tightening of hands | 12 (9.8) | 7 (4.3) | 12 (10.7) | 0 (0) |
| Skin tightening in new areas | 7 (5.7) | 5 (3.1) | 7 (6.3) | 0 (0) |
| Finger-tip ulcers | 7 (5.7) | 3 (1.8) | 7 (6.3) | 0 (0) |
| Shortness of breath | 33 (26.8) | 20 (12.3) | 27 (24.1) | 0 (0) |
| Chest pain | 15 (12.2) | 7 (4.3) | 14 (12.5) | 0 (0) |
| Difficulty in swallowing | 20 (16.3) | 13 (8.0) | 20 (17.9) | 0 (0) |
| Fever | 11 (8.9) | 6 (3.7) | 10 (8.9) | 0 (0) |
| Fatigue | 70 (56.9) | 39 (23.9) | 67 (59.8) | 0 (0) |
| Dry eyes | 18 (14.6) | 15 (9.2) | 18 (16.1) | 0 (0) |
| Dry mouth | 13 (10.6) | 7 (4.3) | 13 (11.6) | 0 (0) |
| Oral/nasal ulcers | 8 (6.5) | 6 (3.7) | 7 (6.3) | 0 (0) |
| Severe hair loss | 10 (8.1) | 7 (4.3) | 10 (14.3) | 0 (0) |
| Headache | 17 (13.8) | 6 (3.7) | 16 (14.3) | 0 (0) |
| Active kidney disease | 2 (1.6) | 1 (0.6) | 1 (0.9) | 0 (0) |
| Elevated muscle enzymes | 23 (18.7) | 16 (9.8) | 22 (19.6) | 0 (0) |
| Elevated inflammatory markers | 20 (16.3) | 14 (8.6) | 19 (17.0) | 0 (0) |
| Types of rashes | ||||
| Heliotrope | 14 (11.4) | 11 (6.7) | 14 (12.5) | 0 (0) |
| Gottron’s over knuckles | 22 (17.9) | 16 (9.8) | 22 (19.6) | 0 (0) |
| Gottron’s over knees | 10 (8.1) | 6 (3.7) | 10 (8.9) | 0 (0) |
| Holster sign | 6 (4.9) | 4 (2.5) | 6 (5.4) | 0 (0) |
| Malar rash | 11 (8.9) | 9 (5.5) | 11 (9.8) | 0 (0) |
| V sign | 17 (13.8) | 14 (8.6) | 17 (15.2) | 0 (0) |
| Forearm rashes | 17 (13.8) | 11 (6.7) | 17 (15.2) | 0 (0) |
| Mechanic’s hand | 9 (7.3) | 7 (4.3) | 9 (8.0) | 0 (0) |
| Clinical feature during flare . | (a) Patient-reported flare (n = 123), N (%) . | (b) Flare by need for IS dose increase (n = 163), N (%) . | (c) Clinical flare (n = 112), N (%) . | (d) Flare by PROMIS PF10a 7.9-point worsening (n = 16), N (%) . |
|---|---|---|---|---|
| Flare prevalence (%) | 9.6 | 12.7 | 8.7 | 19.6 |
| Rashes | 41 (33.3) | 28 (17.2) | 41 (36.6) | 0 (0) |
| Muscle weakness | 85 (69.1) | 47 (28.8) | 85 (75.9) | 1 (6.3) |
| Muscle pain | 51 (41.5) | 42 (25.8) | 68 (60.7) | 1 (6.3) |
| Arthritis of hands | 54 (43.9) | 37 (22.7) | 54 (48.2) | 1 (6.3) |
| Pain in shoulders and hips | 44 (35.8) | 30 (18.4) | 42 (37.5) | 1 (6.3) |
| Arthritis of other joints | 36 (29.3) | 20 (12.3) | 36 (32.1) | 0 (0) |
| Raynaud’s | 23 (18.7) | 14 (8.6) | 22 (19.6) | 0 (0) |
| Skin tightening of hands | 12 (9.8) | 7 (4.3) | 12 (10.7) | 0 (0) |
| Skin tightening in new areas | 7 (5.7) | 5 (3.1) | 7 (6.3) | 0 (0) |
| Finger-tip ulcers | 7 (5.7) | 3 (1.8) | 7 (6.3) | 0 (0) |
| Shortness of breath | 33 (26.8) | 20 (12.3) | 27 (24.1) | 0 (0) |
| Chest pain | 15 (12.2) | 7 (4.3) | 14 (12.5) | 0 (0) |
| Difficulty in swallowing | 20 (16.3) | 13 (8.0) | 20 (17.9) | 0 (0) |
| Fever | 11 (8.9) | 6 (3.7) | 10 (8.9) | 0 (0) |
| Fatigue | 70 (56.9) | 39 (23.9) | 67 (59.8) | 0 (0) |
| Dry eyes | 18 (14.6) | 15 (9.2) | 18 (16.1) | 0 (0) |
| Dry mouth | 13 (10.6) | 7 (4.3) | 13 (11.6) | 0 (0) |
| Oral/nasal ulcers | 8 (6.5) | 6 (3.7) | 7 (6.3) | 0 (0) |
| Severe hair loss | 10 (8.1) | 7 (4.3) | 10 (14.3) | 0 (0) |
| Headache | 17 (13.8) | 6 (3.7) | 16 (14.3) | 0 (0) |
| Active kidney disease | 2 (1.6) | 1 (0.6) | 1 (0.9) | 0 (0) |
| Elevated muscle enzymes | 23 (18.7) | 16 (9.8) | 22 (19.6) | 0 (0) |
| Elevated inflammatory markers | 20 (16.3) | 14 (8.6) | 19 (17.0) | 0 (0) |
| Types of rashes | ||||
| Heliotrope | 14 (11.4) | 11 (6.7) | 14 (12.5) | 0 (0) |
| Gottron’s over knuckles | 22 (17.9) | 16 (9.8) | 22 (19.6) | 0 (0) |
| Gottron’s over knees | 10 (8.1) | 6 (3.7) | 10 (8.9) | 0 (0) |
| Holster sign | 6 (4.9) | 4 (2.5) | 6 (5.4) | 0 (0) |
| Malar rash | 11 (8.9) | 9 (5.5) | 11 (9.8) | 0 (0) |
| V sign | 17 (13.8) | 14 (8.6) | 17 (15.2) | 0 (0) |
| Forearm rashes | 17 (13.8) | 11 (6.7) | 17 (15.2) | 0 (0) |
| Mechanic’s hand | 9 (7.3) | 7 (4.3) | 9 (8.0) | 0 (0) |
IIM: idiopathic inflammatory myopathies; IS: immunosuppression; PROMIS PF10a: Patient-Reported Outcomes Measurement Information System 10 Item Patient-Reported Measure of Physical Function.
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