Table 1.
Demographic, clinical and pathologic characteristics of studied JDM patients
| Patient number | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 |
|---|---|---|---|---|---|---|---|---|---|---|
| Sex | F | F | F | F | F | F | F | F | F | M |
| Age, years | 4 | 7 | 16 | 16 | 3 | 4 | 14 | 15 | 14 | 14 |
| TN or flare | TN | TN | TN | TN | TN | TN | Flare | TN | Flare | Flare |
| Disease duration, months | 5 | 4 | 6 | 4 | 3 | 4 | 0.5 a | 3 | 0.75 a | 0.75 a |
| Definite or probable JDM | Probable | Definite | Definite | Definite | Probable | Definite | Definite | Definite | Probable | Probable (patient amyopathic) |
| Medications during disease courseb | S/I/P/M/H | S/I/P/M/H | S/I/P/M/R | S/I/P/M/R | S/I/P/M | S/I/P/M | S/I/P/M | P/M/H | P/M | P/M/H |
| Medications initiated with flare | — | — | — | — | — | — | S/I/P/M | — | P/M | P/M/H |
| BMI/weight (kg) prior to treatment | 14.8/16.1 | 14.8/23 | 29/75.1 | 18.5/45.3 | 15.8/15.8 | 21/26.7 | 21.4/50.5 | 18.6/49 | 15/36.3 | 16.9/46.2 |
| BMI/weight (kg) at longitudinal sample | 15.3/19.2 | 15.8/25.4 | 39.5/104.1 | 17.4/43.1 | 18.8/20.1 | 28.8/30.7 | 20.1/52.8 | NA | NA | NA |
| Muscle biopsy | ND | D | D | D | ND | D | D | D | ND | ND |
| Biopsy findingsc | ND | M, P, T | M, P | M, P | ND | M | M, P, T | P, T, MI | ND | ND |
| MRI | D, ab | ND | D, ab | D, ab | D, ab | D, ab | D, ab | D, ab | D, ab | D, nl |
| EMG | ND | ND | D, ab | ND | ND | D, ab | D, ab | ND | ND | ND |
| Rash present | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes |
| CK, U/L | 80 | 132 | 49 | 5759 | 191 | 6668 | 25 | 1277 | 96 | 182 |
| Myositis-specific antibody | UD | UD | UD | MI-2 | PM/SCL | HMG-CoA | UD | NXP2 | TIF-1ɣ | UD |
D: done; ND: not done; nl: normal; ab: abnormal; CK: creatinine kinase; UD: undetected.
Baseline samples were obtained from all patients; longitudinal samples were available from those patients indicated by bold number.
a
Onset of flare to treatment in months.
b
Medications during disease course: S: solumedrol; I: IVIG; P: prednisone; M: methotrexate; R: rituximab; H: hydroxychloroquine.
c
Skeletal muscle biopsy findings: M: major histocompatibility complex I upregulation; P: perifascicular atrophy; T: tubuloreticular inclusions; MI: microinfarction.