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Table 1 (abstract A11). See text for description

From: Proceedings of the 2019 Childhood Arthritis and Rheumatology Research Alliance (CARRA) Annual Scientific Meeting

Summary of clinical features associated with lung disease in Trisomy 21-sJIA cases

 

Trisomy 21

Pre-Exposed

sJIA-like

sJIA

PAP/ELP+1

Trisomy 21

Not Trisomy 21

Number of cases

2/6

4/6

5/5

6/6

40/55

Age at sJIA onset (years)

0.6, 2.5

0.5, 1.6, 3.2, 9.9

0.5, 0.6, 2.5, 3.2, 9.9

0.5 to 9.9

Median 2.3 (IQR:1.2-5.1)

Prior to lung disease

 MAS≧1 episode

0/2

0/4

0/5

0/6

29/40 (73%)

 Period of disease quiescence on treatment2

0/2

1/4

0/5

1/6

18/40 (45%)

 Atypical clinical findings occurring between 6 and 1 month prior to lung disease

 Atypical rash

2/2

2/4

3/5

4/6

23/40 (58%)

 Acute clubbing

1/2

3/4

3/5

4/6

31/40(78%)

 Lymphopenia <60% lower limit of normal3

1/1

3/4

3/4

4/5

17/36(47%)

 Tocilizumab reaction

1/2

0/4

1/4

1/5

13/28(46%)

 Peripheral eosinophilia w/in prior year4

1/2

2/4

2/5

3/6

17/39(44%)

Clinical features at lung disease

 Unexpected CT pattern(s) of diffuse lung disease5

0/1

4/4

4/5

4/5

35/39 (90%)

 Hyper-enhancing lymph nodes on CT6

0/1

1/2

1/3

1/3

11/24 (46%)

 Ferritin >1000ng/ml

1/1

2/4

2/4

3/5

14/27 (52%)

 Overt MAS within prior 6 months

0/1

0/4

0/4

0/5

11/40 (28%)

 Hypoxia

2/2

3/4

4/5

5/67

14/40 (35%)7

 Abnormal chest auscultation

2/2

3/4

4/5

5/67

5/40 (13%)7

 Viral infection

1/2

4/4

4/5

5/67

11/40 (28%)7

 Pulmonary hypertension or vascular disease8

1/2

4/4

4/5

5/6

17/40(43%)

Features during lung disease

 MAS

1/2

2/4

2/5

3/6

16/40(40%)

 Alive at data close

1/2

2/4

2/5

3/6

28/40(70%)

  1. 1Pathology on lung tissue evaluation showed pulmonary alveolar proteinosis/endogenous lipoid pneumonia
  2. 2At least one period of ≧6 weeks with ferritin normal for age, no MAS, no pulse steroids and oral steroids zero to <0.15m/k/d. Arthritis allowed
  3. 3Van Gent et al Refined characterization and reference values of the pediatric T- and B-cell compartments. Clin Immunol 2009;133:95-107
  4. 4Peripheral eosinophilia in Trisomy 21 cases occurred within 4 months prior to lung disease
  5. 5Omits patterns of crazy paving only or predominantly ground glass opacities (often seen with PAP/ELP pathology)
  6. 6Evaluable on contrast enhanced CT
  7. 7Significant difference by odds ratio (95% CI): Hypoxia 9.3(1,87), Abnormal chest auscultation 35(3.4,364), Viral infection 13.2(1.4,126)
  8. 8Pulmonary vascular disease on lung tissue and/or clinical pulmonary hypertension