| . | Eosinophilic fasciitis . | Localized scleroderma . | (Dermato) myositis . | Spinal muscular atrophy . | Healthy controls . | . |
|---|---|---|---|---|---|---|
| . | EF . | LoS . | (J)DM+MCTD . | SMA . | HC . | . |
| . | Netherlands . | Netherlands . | Netherlands, France & Singapore . | Netherlands . | Netherlands . | . |
| . | n=8 . | n=30 . | n=33+1 . | n=43 . | n=22 . | P-value . |
| Age at diagnosis (years), median (IQR) | 64.9 (17.1) | 35.5 (40.4) | 8.4 (9.5) | 1.5 (1.7) | — | <0.0001 |
| Age at sampling (years), median (IQR) | 65.1 (12.8) | 41.9 (35.1) | 8.4 (9.5) | 35.7 (28.4) | 31 (21) | <0.0001 |
| Sex, % female | 62.5 | 66.7 | 55.9 | 60.5 | 68.2 | 0.8750 |
| Pediatric patients,% | 0.0 | 16.7 | 91.2 | 23.3 | 0.0 | |
| Clinical disease activity scores | ||||||
| Muscle weakness (% of patients) | — | — | 91.2 | — | — | n/a |
| (J)DM skin symptoms (% of patients) | — | — | 94.1 | — | — | n/a |
| CMAS (0–52), median (IQR) | — | — | 28.0 (22.3) | — | — | n/a |
| NR=12 | ||||||
| PGA (0–10), median (IQR) | — | — | 6.0 (1.8) | — | — | n/a |
| NR=9 | ||||||
| EF/LoS VAS activity (0–100), median (IQR) | 46.5 (11.8) | 20.5 (21.3) | — | — | — | 0.0054 |
| NR=2 | NR=2 | |||||
| EF/LoS mLoSSI (0–162), median (IQR) | 42.5 (18.3) | 16 (12.5) | — | — | — | 0.0035 |
| NR=2 | NR=2 | |||||
| EF/LoS VAS damage (0–100), median (IQR) | 17.5 (20) | 12 (17) | — | — | — | 0.5839 |
| NR=2 | NR=1 | |||||
| EF/LoS LoSDI (0–162), median (IQR) | 8.5 (6.8) | 6 (6.5) | — | — | — | 0.8560 |
| NR=2 | NR=2 | |||||
| SMA motor score (HMFSE, 0–66), median (IQR) | — | — | — | 4 (36) | — | n/a |
| NR=4 | ||||||
| Laboratory parameters | ||||||
| % ANA positive | 42.9 | 60.0 | 59.4 | — | — | 0.7006 |
| NR=1 | NR=10 | NR=2 | ||||
| CRP (mg/l), median (IQR) | 10 (1) | 5 (1.5) | 1 (2) | — | — | 0.0172 |
| NR=6 | NR=26 | NR=3 | ||||
| ESR (mm/hour), median (IQR) | 16 (7) | 5 (7.8) | 16 (10) | — | — | 0.0126 |
| NR=5 | NR=18 | NR=5 | ||||
| ALT (IU/l), median (IQR) | 20.5 (9.5) | 20.5 (7.8) | 48.5 (83.3) | — | — | 0.0031 |
| NR=4 | N=18 | |||||
| AST (IU/l), median (IQR) | 24 (20.5) | — | 102 (480) | — | — | 0.0060 |
| NR=5 | NR=1 | |||||
| CK (IU/l), median (IQR) | 37 (2) | — | 659 (2865.3) | — | — | 0.0032 |
| NR=6 | ||||||
| LDH (IU/l), median (IQR) | 214.5 (20.5) | — | 642 (480) | — | — | 0.0214 |
| NR=6 | NR=3 |
| . | Eosinophilic fasciitis . | Localized scleroderma . | (Dermato) myositis . | Spinal muscular atrophy . | Healthy controls . | . |
|---|---|---|---|---|---|---|
| . | EF . | LoS . | (J)DM+MCTD . | SMA . | HC . | . |
| . | Netherlands . | Netherlands . | Netherlands, France & Singapore . | Netherlands . | Netherlands . | . |
| . | n=8 . | n=30 . | n=33+1 . | n=43 . | n=22 . | P-value . |
| Age at diagnosis (years), median (IQR) | 64.9 (17.1) | 35.5 (40.4) | 8.4 (9.5) | 1.5 (1.7) | — | <0.0001 |
| Age at sampling (years), median (IQR) | 65.1 (12.8) | 41.9 (35.1) | 8.4 (9.5) | 35.7 (28.4) | 31 (21) | <0.0001 |
| Sex, % female | 62.5 | 66.7 | 55.9 | 60.5 | 68.2 | 0.8750 |
| Pediatric patients,% | 0.0 | 16.7 | 91.2 | 23.3 | 0.0 | |
| Clinical disease activity scores | ||||||
| Muscle weakness (% of patients) | — | — | 91.2 | — | — | n/a |
| (J)DM skin symptoms (% of patients) | — | — | 94.1 | — | — | n/a |
| CMAS (0–52), median (IQR) | — | — | 28.0 (22.3) | — | — | n/a |
| NR=12 | ||||||
| PGA (0–10), median (IQR) | — | — | 6.0 (1.8) | — | — | n/a |
| NR=9 | ||||||
| EF/LoS VAS activity (0–100), median (IQR) | 46.5 (11.8) | 20.5 (21.3) | — | — | — | 0.0054 |
| NR=2 | NR=2 | |||||
| EF/LoS mLoSSI (0–162), median (IQR) | 42.5 (18.3) | 16 (12.5) | — | — | — | 0.0035 |
| NR=2 | NR=2 | |||||
| EF/LoS VAS damage (0–100), median (IQR) | 17.5 (20) | 12 (17) | — | — | — | 0.5839 |
| NR=2 | NR=1 | |||||
| EF/LoS LoSDI (0–162), median (IQR) | 8.5 (6.8) | 6 (6.5) | — | — | — | 0.8560 |
| NR=2 | NR=2 | |||||
| SMA motor score (HMFSE, 0–66), median (IQR) | — | — | — | 4 (36) | — | n/a |
| NR=4 | ||||||
| Laboratory parameters | ||||||
| % ANA positive | 42.9 | 60.0 | 59.4 | — | — | 0.7006 |
| NR=1 | NR=10 | NR=2 | ||||
| CRP (mg/l), median (IQR) | 10 (1) | 5 (1.5) | 1 (2) | — | — | 0.0172 |
| NR=6 | NR=26 | NR=3 | ||||
| ESR (mm/hour), median (IQR) | 16 (7) | 5 (7.8) | 16 (10) | — | — | 0.0126 |
| NR=5 | NR=18 | NR=5 | ||||
| ALT (IU/l), median (IQR) | 20.5 (9.5) | 20.5 (7.8) | 48.5 (83.3) | — | — | 0.0031 |
| NR=4 | N=18 | |||||
| AST (IU/l), median (IQR) | 24 (20.5) | — | 102 (480) | — | — | 0.0060 |
| NR=5 | NR=1 | |||||
| CK (IU/l), median (IQR) | 37 (2) | — | 659 (2865.3) | — | — | 0.0032 |
| NR=6 | ||||||
| LDH (IU/l), median (IQR) | 214.5 (20.5) | — | 642 (480) | — | — | 0.0214 |
| NR=6 | NR=3 |
Three patients (two JDM, one LoS) in whom treatment was started max 1 week before sampling, were also considered treatment-naive. For continuous variables, medians and interquartile ranges (IQR) are shown. For categorical variables, frequencies are shown. For comparison between two groups, the Mann–Whitney U test was used for continuous variables and the Fisher’s exact test for categorical variables. For comparison between more than two groups, the Kruskal–Wallis test was used for continuous variables and the chi-squared test for categorical variables. (J)DM: (juvenile) dermatomyositis (n = 33); EF: eosinophilic fasciitis (n = 8); HC: healthy controls (n = 22); LoS: localized scleroderma (n = 30); MCTD: mixed connective tissue disease (n = 1); NR: not reported; SMA: spinal muscular atrophy (n = 43); ALT: alanine aminotransferase; AST: aspartate aminotransferase; CK: creatine kinase; LDH: lactate dehydrogenase; VAS: visual analogue scale; HMFSE: Hammersmith Functional Motor Scale Expanded.
| . | Eosinophilic fasciitis . | Localized scleroderma . | (Dermato) myositis . | Spinal muscular atrophy . | Healthy controls . | . |
|---|---|---|---|---|---|---|
| . | EF . | LoS . | (J)DM+MCTD . | SMA . | HC . | . |
| . | Netherlands . | Netherlands . | Netherlands, France & Singapore . | Netherlands . | Netherlands . | . |
| . | n=8 . | n=30 . | n=33+1 . | n=43 . | n=22 . | P-value . |
| Age at diagnosis (years), median (IQR) | 64.9 (17.1) | 35.5 (40.4) | 8.4 (9.5) | 1.5 (1.7) | — | <0.0001 |
| Age at sampling (years), median (IQR) | 65.1 (12.8) | 41.9 (35.1) | 8.4 (9.5) | 35.7 (28.4) | 31 (21) | <0.0001 |
| Sex, % female | 62.5 | 66.7 | 55.9 | 60.5 | 68.2 | 0.8750 |
| Pediatric patients,% | 0.0 | 16.7 | 91.2 | 23.3 | 0.0 | |
| Clinical disease activity scores | ||||||
| Muscle weakness (% of patients) | — | — | 91.2 | — | — | n/a |
| (J)DM skin symptoms (% of patients) | — | — | 94.1 | — | — | n/a |
| CMAS (0–52), median (IQR) | — | — | 28.0 (22.3) | — | — | n/a |
| NR=12 | ||||||
| PGA (0–10), median (IQR) | — | — | 6.0 (1.8) | — | — | n/a |
| NR=9 | ||||||
| EF/LoS VAS activity (0–100), median (IQR) | 46.5 (11.8) | 20.5 (21.3) | — | — | — | 0.0054 |
| NR=2 | NR=2 | |||||
| EF/LoS mLoSSI (0–162), median (IQR) | 42.5 (18.3) | 16 (12.5) | — | — | — | 0.0035 |
| NR=2 | NR=2 | |||||
| EF/LoS VAS damage (0–100), median (IQR) | 17.5 (20) | 12 (17) | — | — | — | 0.5839 |
| NR=2 | NR=1 | |||||
| EF/LoS LoSDI (0–162), median (IQR) | 8.5 (6.8) | 6 (6.5) | — | — | — | 0.8560 |
| NR=2 | NR=2 | |||||
| SMA motor score (HMFSE, 0–66), median (IQR) | — | — | — | 4 (36) | — | n/a |
| NR=4 | ||||||
| Laboratory parameters | ||||||
| % ANA positive | 42.9 | 60.0 | 59.4 | — | — | 0.7006 |
| NR=1 | NR=10 | NR=2 | ||||
| CRP (mg/l), median (IQR) | 10 (1) | 5 (1.5) | 1 (2) | — | — | 0.0172 |
| NR=6 | NR=26 | NR=3 | ||||
| ESR (mm/hour), median (IQR) | 16 (7) | 5 (7.8) | 16 (10) | — | — | 0.0126 |
| NR=5 | NR=18 | NR=5 | ||||
| ALT (IU/l), median (IQR) | 20.5 (9.5) | 20.5 (7.8) | 48.5 (83.3) | — | — | 0.0031 |
| NR=4 | N=18 | |||||
| AST (IU/l), median (IQR) | 24 (20.5) | — | 102 (480) | — | — | 0.0060 |
| NR=5 | NR=1 | |||||
| CK (IU/l), median (IQR) | 37 (2) | — | 659 (2865.3) | — | — | 0.0032 |
| NR=6 | ||||||
| LDH (IU/l), median (IQR) | 214.5 (20.5) | — | 642 (480) | — | — | 0.0214 |
| NR=6 | NR=3 |
| . | Eosinophilic fasciitis . | Localized scleroderma . | (Dermato) myositis . | Spinal muscular atrophy . | Healthy controls . | . |
|---|---|---|---|---|---|---|
| . | EF . | LoS . | (J)DM+MCTD . | SMA . | HC . | . |
| . | Netherlands . | Netherlands . | Netherlands, France & Singapore . | Netherlands . | Netherlands . | . |
| . | n=8 . | n=30 . | n=33+1 . | n=43 . | n=22 . | P-value . |
| Age at diagnosis (years), median (IQR) | 64.9 (17.1) | 35.5 (40.4) | 8.4 (9.5) | 1.5 (1.7) | — | <0.0001 |
| Age at sampling (years), median (IQR) | 65.1 (12.8) | 41.9 (35.1) | 8.4 (9.5) | 35.7 (28.4) | 31 (21) | <0.0001 |
| Sex, % female | 62.5 | 66.7 | 55.9 | 60.5 | 68.2 | 0.8750 |
| Pediatric patients,% | 0.0 | 16.7 | 91.2 | 23.3 | 0.0 | |
| Clinical disease activity scores | ||||||
| Muscle weakness (% of patients) | — | — | 91.2 | — | — | n/a |
| (J)DM skin symptoms (% of patients) | — | — | 94.1 | — | — | n/a |
| CMAS (0–52), median (IQR) | — | — | 28.0 (22.3) | — | — | n/a |
| NR=12 | ||||||
| PGA (0–10), median (IQR) | — | — | 6.0 (1.8) | — | — | n/a |
| NR=9 | ||||||
| EF/LoS VAS activity (0–100), median (IQR) | 46.5 (11.8) | 20.5 (21.3) | — | — | — | 0.0054 |
| NR=2 | NR=2 | |||||
| EF/LoS mLoSSI (0–162), median (IQR) | 42.5 (18.3) | 16 (12.5) | — | — | — | 0.0035 |
| NR=2 | NR=2 | |||||
| EF/LoS VAS damage (0–100), median (IQR) | 17.5 (20) | 12 (17) | — | — | — | 0.5839 |
| NR=2 | NR=1 | |||||
| EF/LoS LoSDI (0–162), median (IQR) | 8.5 (6.8) | 6 (6.5) | — | — | — | 0.8560 |
| NR=2 | NR=2 | |||||
| SMA motor score (HMFSE, 0–66), median (IQR) | — | — | — | 4 (36) | — | n/a |
| NR=4 | ||||||
| Laboratory parameters | ||||||
| % ANA positive | 42.9 | 60.0 | 59.4 | — | — | 0.7006 |
| NR=1 | NR=10 | NR=2 | ||||
| CRP (mg/l), median (IQR) | 10 (1) | 5 (1.5) | 1 (2) | — | — | 0.0172 |
| NR=6 | NR=26 | NR=3 | ||||
| ESR (mm/hour), median (IQR) | 16 (7) | 5 (7.8) | 16 (10) | — | — | 0.0126 |
| NR=5 | NR=18 | NR=5 | ||||
| ALT (IU/l), median (IQR) | 20.5 (9.5) | 20.5 (7.8) | 48.5 (83.3) | — | — | 0.0031 |
| NR=4 | N=18 | |||||
| AST (IU/l), median (IQR) | 24 (20.5) | — | 102 (480) | — | — | 0.0060 |
| NR=5 | NR=1 | |||||
| CK (IU/l), median (IQR) | 37 (2) | — | 659 (2865.3) | — | — | 0.0032 |
| NR=6 | ||||||
| LDH (IU/l), median (IQR) | 214.5 (20.5) | — | 642 (480) | — | — | 0.0214 |
| NR=6 | NR=3 |
Three patients (two JDM, one LoS) in whom treatment was started max 1 week before sampling, were also considered treatment-naive. For continuous variables, medians and interquartile ranges (IQR) are shown. For categorical variables, frequencies are shown. For comparison between two groups, the Mann–Whitney U test was used for continuous variables and the Fisher’s exact test for categorical variables. For comparison between more than two groups, the Kruskal–Wallis test was used for continuous variables and the chi-squared test for categorical variables. (J)DM: (juvenile) dermatomyositis (n = 33); EF: eosinophilic fasciitis (n = 8); HC: healthy controls (n = 22); LoS: localized scleroderma (n = 30); MCTD: mixed connective tissue disease (n = 1); NR: not reported; SMA: spinal muscular atrophy (n = 43); ALT: alanine aminotransferase; AST: aspartate aminotransferase; CK: creatine kinase; LDH: lactate dehydrogenase; VAS: visual analogue scale; HMFSE: Hammersmith Functional Motor Scale Expanded.
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