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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
 MAS1 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