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

From: Respiratory symptoms as initial manifestations of interstitial lung disease in clinically amyopathic juvenile dermatomyositis: a case report with literature review

PATIENT CHARACTERISTIC CASE 1 CASE 2 CASE 3 CASE 4 CASE 5 CASE 6 CASE 7
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
  1. WBC white blood cell, AST aspartate aminotransferase, ALT alanine aminotransferase, CK creatine kinase, LDH lactate dehydrogenase, CRP C-reactive protein, ESR erythrocyte sedimentation rate, chest CT Chest computed tomography, PSL prednisolone, AZA azathioprine, CSA cyclosporin A, MP methylprednisolone, CTX cyclophosphamide, IVIG intravenous immunoglobulin, PFD pirfenidone, MMF mycophenolate mofetil, HCQ hydroxychloroquine