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Table 2 Clinical characteristics of IMNM and other types of IIM

From: Clinical and pathological features of immune-mediated necrotising myopathies in a single-centre muscle biopsy cohort

Characteristics

IMNM(n = 68)

DM(n = 346)

ASS(n = 82)

PM(n = 32)

sIBM(n = 3)

Female

45 (66.2)

236 (68.2)

57 (69.5)

25 (78.1)

1 (33.3)

Age of onset

41.57 ± 15.46

44.32 ± 15.61

50.06 ± 12.12

42.75 ± 15.30

55 ± 11.13

Duration (months)

21.94 ± 25.94

23.60 ± 49.18

23.50 ± 42.35

22.08 ± 35.86

70 ± 45.03

Fever

5 (7.4)

87 (25.1)

36 (43.9)

6 (18.8)

0

Loss of weight

19 (27.9)

94 (27.2)

19 (23.2)

8 (25.0)

0

Muscle weakness

62 (91.2)

253 (73.1)

54 (65.9)

22 (68.8)

3 (100)

Severe muscle weakness

30 (44.1)

59 (17.1)

4 (4.9)

3 (9.4)

1 (33.3)

Dysphagia

24 (35.3)

94 (27.2)

14 (17.1)

6 (18.8)

0

Muscular atrophy

2 (2.9)

1 (0.3)

0

0

0

Myalgia

22 (32.4)

169 (48.8)

34 (41.5)

12 (37.5)

0

Arthralgia

6 (8.8)

123 (35.5)

40 (48.8)

9 (28.1)

0

Skin involvement

15 (22.1)

334 (96.5)

52 (63.4)

3 (9.4)

1 (33.3)

 Heliotrope rash

6 (8.8)

238 (68.8)

21 (25.6)

0

0

 Mechanics’ hands

1 (1.5)

125 (36.1)

25 (30.5)

0

0

 Gottron’s sign

3 (4.4)

214 (61.8)

27 (32.9)

0

0

 V sign

6 (8.8)

198 (57.2)

16 (19.5)

1 (3.1)

1 (33.3)

 Shawl sign

5 (7.4)

143 (41.3)

14 (17.1)

1 (3.1)

0

Raynaud phenomenon

1 (1.5)

27 (7.8)

9 (11.0)

3 (9.4)

0

Interstitial lung diseases

22 (32.4)

161 (46.5)

63 (76.8)

11 (34.4)

0

Malignancies

5 (7.4)

29 (8.4)

3 (3.7)

0

0

Other connective tissue diseases

5 (7.4)

39 (11.3)

16 (19.5)

10 (31.3)

0

ALT (0-40 U/L)

117 (64,241)

38 (23,76)

32 (24,87)

43 (30,50)

–

AST (0-40 U/L)

85 (42,153)

37 (22,76)

32 (18,55)

34 (21,75)

–

LDH (100-250 IU/L)

569 (347,836)

264 (200,378)

264 (209,398)

291 (217,393)

–

CK (26-200 IU/L)

2289 (894,5505)

103 (43,520)

422 (54,1066)

333 (35,1090)

–

ANA (> 1:40)

40/66 (60.6)

198/323 (61.3)

55/78 (70.5)

19 (59.4)

0

MSA

N = 68

N = 334

N = 82

N = 26

N = 3

 Anti-MDA5

0

70 (20.9)

0

0

0

 Anti-NXP2

0

40 (12.0)

0

0

0

 Anti-TIF1-γ

0

57 (17.1)

0

0

0

 Anti-Mi-2

0

26 (7.8)

0

0

0

 Anti-SAE

0

7 (2.1)

0

0

0

 Anti-Jo-1

0

0

40 (48.8)

0

0

 Anti-PL-7

0

0

22 (26.8)

0

0

 Anti-PL-12

0

0

8 (9.8)

0

0

 Anti-EJ

0

0

12 (14.6)

0

0

 Anti-SRP

35 (51.5)

0

0

0

0

 Anti-HMGCR

13 (19.1)

0

0

0

0

 MSA negative

20 (29.4)

134 (40.1)

0

26 (100)

0

MAA

 Anti-Ro-52

16 (24.2)

64 (19.8)

28 (35.9)

4 (12.5)

0

 Anti-Ku

1 (1.5)

0

4 (4.9)

0

0

 Anti- PM/Scl

1 (1.5)

4 (1.2)

1 (1.3)

0

0

 Anti-AMA-M2

6 (9.2)

11 (3.4)

1 (1.3)

3 (9.4)

0

  1. IIM Idiopathic inflammatory myopathies, IMNM Immune-mediated necrotising myopathy, DM Dermatomyositis, ASS Anti-synthetase syndrome, PM Polymyositis, sIBM Sporadic inclusion body myositis, ALT Alanine aminotransferase, AST Aspartate aminotransferase, LDH Lactate dehydrogenase, CK Creatine kinase, ANA Anti-nuclear antibodies, MSA Myositis specific antibodies, MAA Myositis associated antibodies