Table 1 Clinical manifestations and course of illness in 7 children with CAJDM-associated ILD

AUTHOR (YEAR)

I. Kobayashi (2001)

I. Kobayashi (2003)

Yoshifusa Abe (2010)

Wang Tingjie (2015)

Jiang Lu (2017)

Our Case (2018)

Hou Jun (2019)

GENDER

Male

Male

Male

Female

Male

Male

Male

AGE AT ONSET Y

8

8

16

10

8

10

13

CUTANEOUS MANIFESTATIONS

Gottron papules

Gottron papules

Gottron papules

Gottron papules

Gottron papules

Edema

Gottron papules

Gottron papules

MUSCLE MANIFESTATIONS

–

–

–

–

–

–

–

RESPIRATORY SYMPTOMS

Dry cough

Dyspnea

Cough

Dyspnoea

Cough

Dyspnoea

–

–

Cough

Chest pain

Chest pain

OTHER SYMPTOMS

Fever

–

–

Fever

Fever

Fever

–

WBC *10^9/L

3.9

Unknown

7.7

Unknown

Unknown

6.1

Normal

AST U/L

71

77

50

629

75

50

97

ALT U/L

38

Unknown

95

448

Normal

28

Unknown

CK U/L

1250

65

61

229

233

36

74

LDH U/L

647

539

392

Unknown

811

425

425

CRP MG/L

Negative

Unknown

0.3

Unknown

77

Negative

2.4

ESR MM/H

6

Unknown

Unknown

Normal

Normal

12

9

ANTI-MDA5

–

–

–

–

–

–

+

ANTI-RO52

–

–

–

+

–

+

–

OTHER ANTIBODIES

–

–

–

–

–

–

–

ELECTROMYOGRAPHY

Unknown

Unknown

Unknown

Myogenic damage

–

Normal

Unknown

CHEST CT

Interstitial pneumonia

Interstitial pneumonia

Interstitial pneumonia

Interstitial pneumonia

Interstitial pneumonia

Interstitial pneumonia

Interstitial pneumonia

LUNG FUNCTION

Unknown

Unknown

Unknown

Restrictive ventilatory dysfunction

Unknown

Restrictive ventilatory dysfunction

Unknown

SKIN BIOPSY

DM

Unknown

DM

DM

DM

DM

DM

TREATMENT

PSL AZA CSA

PSL AZA

MP CSA CTX

PSL IVIG PFD HCQ

MP IVIG MMF HCQ Tocilizumab

MP PSL MMF IVIG

MP CTX

PROGNOSIS

Died

Died

Died

Died

Improved

Improved

Not Improved