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Table 1 The clinical features of JDM complicated with MAS

From: Macrophage activation syndrome in juvenile dermatomyositis: a case report and a comprehensive review of the literature

Patient number

Study (year)

Patient/

gender/age

Skin lesions

MS, and CK level (U/L)

Arthritis

ILD

MSA

From JDM

onset to MAS

1

Kobayashi

et al. (2000) [5]

1/M/14

Gottron’s papules and heliotrope eruption

Weakness;

CK 26U/L

Yes

Yes

ND

3 months

2

Sterba et al. (2004) [6]

1/M/12

ND

ND

ND

ND

ND

ND

3

Kobayashi

et al. (2006) [7]

1/F/10

Gottron’s papules and heliotrope eruption

Weakness;

CK 579 U/L

No

Yes

ND

2 weeks

4

Yajima et al.

(2008) [8]

1/M/16 ADM

ND

Normal

Yes

Yes

ND

Active phase of DM

5

Bustos et al.

(2012) [9]

1/M/7

Heliotrope sign and facial edema

Weakness;

CK 16,353 U/L

No

ND

ND

4 weeks

6

Wang et al. (2013) [10]

1/M/15,

Heliotrope sign and facial edema

Weakness;

CK 3325 U/L

No

No

ND

2 weeks

7

Yamashita

et al. (2013) [11]

1/F/17

Heliotrope, Gottron’s sign, and nail-fold lesions

Weakness;

CK 1094 U/L

Yes

Yes

No

3 months

8

Lilleby et al. (2014) [12]

1/M/12

Facial erythema and

facial swelling

Weakness;

CK 14,000 U/L

No

Yes

ND

5 weeks

9

Ye et al. (2014) [13]

1/M/16

Heliotrope rash and facial swelling

Weakness;

CK 1071 U/L

No

Yes

No

6 weeks

10

Poddighe

et al. (2014) [14]

1/F/13

ADM

Heliotrope, Gottron’s rash, and peri-orbital edema

Normal

Yes

Yes

ND

3 months

11

Önen et al.

(2014) [15]

1/F/12

Heliotrope rash

Weakness;

CK 25 U/L

No

No

ND

6 months

12

Wakiguchi

et al. (2015) [16]

1/F/4

Heliotrope rash and Gottron’s sign

Weakness;

CK 40 U/L

No

Yes

MDA5

3 months

13

Teshigawara et al. (2016) [17]

1/M/17

Heliotrope eruption

Myalgia;

CK 1869 U/L

No

No

No

6 months

14

Deschamps

et al. (2018) [18]

1/F/17

Right facial swelling

Weakness;

CK 4265 U/L

No

No

NXP2

5 weeks

15

Stewart JA et al. (2022) [19]

1/F/14

Ulcerative lesions on the dorsum of the hands

Weakness;

CK 217 U/L

Yes

Yes

MDA5

3 months

16

Mouri M et al. (2022) [20]

1/M/5

Heliotrope rash and Gottron’s sign

Weakness;

CK 2511 U/L

Yes

No

NXP2

2 weeks

17

Jagwani H et al. (2022) [21]

1/M/11

Heliotrope sign

Weakness;

elevated CK level

ND

No

No

4 weeks

18

Our case

1/F/15

Heliotrope rash and mild edema on upper arm

Weakness,

CK 2914 U/L

No

No

NXP2

3 months

  1. ADM, amyopathic DM; F, female; M male; MSA, myositis-specific antibody; MS, muscle strength; ND, not described