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Table 1 Demographic data of cohort 1 at the time of first available serum sample*

From: Longitudinal assessment of reactivity and affinity profile of anti-Jo1 autoantibodies to distinct HisRS domains and a splice variant in a cohort of patients with myositis and anti-synthetase syndrome

 

IIM/ASSD (n=44)

Anti-Jo1+ (n=19)

Anti-Jo1 (n=25)

Age, mean years (SD)

57 (13)

52 (14)

61 (12)a

Women, n (%)

24 (55)

9 (47)

15 (60)

Disease duration in months, median (25–75th percentiles)**

0 (0–1)

1 (0–10)

0 (0–1)

Anti-synthetase syndrome (ASSD), n (%)

28 (64)

19 (100)

9 (36)b

Muscular manifestations, n ever (%)

 Muscle weakness (pathological MMT8 and/or FI-2)

35 (83)

15 (79)

20 (87)

 Muscle enzymes elevation (CK, LD, ASAT, ALAT)

35 (83)

15 (79)

20 (87)

 Muscle inflammatory infiltrates

26 (62)

11 (58)

15 (65)

Extra-muscular manifestations, n ever (%)

 Interstitial lung disease (ILD)

25 (57)

16 (84)

9 (36)c

 Skin rash***

14 (32)

5 (26)

9 (36)

 Arthritis

18 (41)

11 (58)

7 (28)

 Dysphagia

9 (21)

3 (16)

6 (24)

 Raynaud’s phenomenon

2 (5)

2 (11)

0 (0)

Smoking status, n ever (%)

24 (55)

10 (53)

14 (56)

Laboratory tests

 CK, median μcat/L (25–75th percentiles)

4.3 (1.4–14.2)

3.8 (1.1–9.0)

4.4 (1.6–16.2)

 CRP, median mg/L (25–75th percentiles)

4.0 (0.9–8.3)

7.0 (2.0–9.0)

2.0 (0.5–8.0)

Autoantibodies

 Positive anti-PL7, n (%)

2 (5.1)

0

2 (8.3)

 Positive anti-PL12, n (%)

2 (5.1)

0

2 (8.3)

 Positive anti-EJ, n (%)

1 (2.6)

0

1 (4.2)

 Positive anti-OJ, n (%)

3 (7.7)

0

3 (12.5)

 Positive anti-Mi-2, n (%)

3 (7.9)

1 (7.1)

2 (8.3)

 Positive anti-SRP, n (%)

2 (5.1)

0

2 (8.3)

 Positive anti-MDA5, n (%)

3 (7.9)

0

3 (12.5)

 Positive anti-TIF1g, n (%)

3 (7.9)

0

3 (12.5)

 Positive anti-SSA, n (%)

16 (36.4)

10 (52.6)

6 (24.0)

 Positive anti-Ro52, n (%)

12 (38.7)

8 (47.1)

4 (28.6)

 Positive anti-SSB, n (%)

0

0

0

 Positive anti-U1 RNP, n (%)

5 (11.4)

2 (10.5)

3 (12.0)

 Positive anti-Ku, n (%)

1 (2.5)

0

1 (4.0)

 Positive anti-PmScl, n (%)

2 (4.9)

1 (6.3)

1 (4.0)

Physician VAS, median (25–75th percentiles)

40 (25– 60)

45 (32–60)

40 (17–50)

Patient VAS, median (25–75th percentiles)

40 (16–69)

44 (19–70)

32 (11–68)

HAQ (1–3), median (25–75th percentiles)

0.88 (0.00–1.50)

0.75 (0.19–1.25)

1.00 (0.00–1.63)

MMT-8 (0–80), median (25–75th percentiles)

78 (67–80)

79 (77–80)

75 (64–80)

Muscle activity score VAS, median (25–75th percentiles)

15 (0 37.5)

4.5 (0–35.5)

15 (0–37.5)

MDAAT, median (25–75th percentiles)

0.07 (0.05–0.16)

0.12 (0.05–0.17)

0.06 (0.03–0.16)

Extra-muscular activity, median (25–75th percentiles)

32 (15–40)

40 (11–43)

24 (16–34)

Immunosuppressive (IS) treatment, n (%)

 No treatment

10 (26)

4 (25)

6 (26)

 1 treatment

10 (26)

2 (13)

8 (35)

 2 or 3 concomitant treatments

19 (49)

10 (63)

9 (39)

 

Healthy controls (n=24)

  

Age, mean years (SD)

59.3 (13.0)

  

Women, n (%)

12 of 24 (50)

  
  1. *First available serum samples collected: (i) at diagnosis (0 months), sera were available from 6 anti-Jo1+ and 14 anti-Jo1 patients; (ii) before diagnosis, sera were available from 3 anti-Jo1+ and 4 anti-Jo1 patients (median months [25–75th percentile], −1 [−3 to −1] and −10[−19.5 to −4.25], respectively); (iii) after diagnosis, sera were available from 10 anti-Jo1+ and 7 anti-Jo1− patients (9 [1–99] and 1 [1–4], respectively).
  2. IIM idiopathic inflammatory myopathies; ASSD anti-synthetase syndrome; CK creatinine kinase (reference values: 0.6–3.5 μkat/L); CRP C-reactive protein (0–3 mg/L); VAS visual analogue scale; MDDAT Myositis Disease Activity Assessment Tool; HAQ Health Assessment Questionnaire; MMT-8 Manual Muscle Testing
  3. 1 treatment designates one of the following: methotrexate (Mtx), glucocorticoids (GC), intravenous immunoglobulin, or abatacept; 2 or 3 concomitant treatments designate all the possible following combinations: GC + azathioprine (Aza), GC + cyclophosphamide, GC + Mtx, GC + mycophenolate mofetil (MMF), GC + rituximab, GC + cyclophosphamide + rituximab, GC + Mtx + rituximab, or GC + MMF + rituximab
  4. **Disease duration was calculated based on month and year of clinical diagnosis; ***Skin rash features: Periungual erythema, mechanic’s hand, Gottron’s sign, Gottron’s papules, V-sign, shawl sign, alopecia, erythroderma, periorbital edema, heliotrope rash
  5. ap = 0.0236; bp < 0.0001; cp = 0.0020 vs anti-Jo1+ (Mann-Whitney’s test for quantitative variables and Fisher’s exact test for analysis of categorical variables were employed)