Table 1 Clinical and serological features of 190 patients with IIM stratified by ILD

n (%)

89 (46.84)

101 (53.16)

Age of onset (years), mean (SD)

56.6 (12.7)

49.0 (16.7)

< 0.001

Male, n (%)

25 (28.09)

34 (33.66)

0.502

CK (U/L), median (IQR)

389.0 (113.0, 1687.5)

585.5 (151.0, 4021.0)

0.100

LD (U/L), median (IQR)

367.0 (264.0, 538.0)

360.0 (244.0, 662.0)

0.997

AST (U/L), median (IQR)

60.0 (35.0, 159.0)

69.0 (38.0, 204.0)

0.472

IIM subgroup, n (%)

< 0.001

DM

19 (21.35)

33 (32.67)

ASS

56 (62.92)

7 (6.93)

IMNM

1 (1.12)

15 (14.85)

PM

1 (1.12)

13 (12.87)

Overlap myositis

12 (13.48)

32 (31.68)

IBM

0

1 (0.99)

Concurrent autoimmune diseases, n (%)

0.494

None

59 (66.29)

64 (63.37)

Systemic lupus erythematosus

3 (3.37)

12 (11.88)

Primary Sjögren’s syndrome

13 (14.61)

10 (9.90)

Systemic sclerosis

3 (3.37)

4 (3.96)

Rheumatoid arthritis

5 (5.62)

5 (4.95)

Mixed CTD

1 (1.12)

1 (0.99)

Undifferentiated CTD

5 (5.62)

5 (4.95)

Malignancy, n (%)

8 (8.99)

10 (9.90)

1.000

Mortality, n (%)

12 (13.48)

3 (2.97)

0.016

MSAs, n (%)

Mi-2α

0 (0.00)

4 (3.96)

0.124

Mi-2β

1 (1.12)

3 (2.97)

0.624

TIF1γ

1 (1.12)

11 (10.89)

0.014

MDA5

8 (8.99)

4 (3.96)

0.261

SAE1

1 (1.12)

1 (0.99)

1.000

NXP2

4 (4.49)

1 (0.99)

0.188

SRP

1 (1.12)

10 (9.90)

0.023

Jo-1

23 (25.84)

5 (4.95)

< 0.001

PL-7

10 (11.24)

2 (1.98)

0.020

PL-12

9 (10.11)

1 (0.99)

0.007

EJ

10 (11.24)

0 (0.00)

< 0.001

OJ

2 (2.25)

1 (0.99)

0.601

MAAs, n (%)

Ku

4 (4.49)

9 (8.91)

0.360

PM-Scl-75

0 (0.00)

2 (1.98)

0.499

PM-Scl-100

0 (0.00)

1 (0.99)

1.000

Ro-52

57 (64.04)

19 (18.81)

< 0.001

Muscle weakness

56 (62.92)

80 (79.21)

0.020

Dermatologic manifestations

Skin rash

30 (33.71)

40 (39.60)

0.490

Heliotrope rash

12 (13.48)

14 (13.86)

1.000

Gottron’s papules/signs

23 (25.84)

26 (25.74)

1.000

Calcinosis cutis

3 (3.37)

1 (0.99)

0.342

Mechanic’s hands

20 (22.47)

3 (2.97)

< 0.001