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Table 1 Summary of demographic and baseline characteristics

From: Efficacy and safety of baricitinib in Japanese patients with autoinflammatory type I interferonopathies (NNS/CANDLE, SAVI, And AGS)

Parameter

NNS/CANDLE

(N = 5)

SAVI

(N = 3)

AGS

(N = 1)

Total

(N = 9)

Age, years

  Mean (SD)

39.2 (13.8)

9.7 (9.8)

9.0 (NA)

26.0 (19.1)

  Range (minimum, maximum)

15–48

4–21

9–9

4–48

Sex, n (%)

  Female

1 (20.0)

3 (100.0)

1 (100.0)

5 (55.6)

eGFR (mL/min/1.73m2)

  Mean (SD)

124.7 (19.3)

109.4 (22.5)

106.7 (NA)

117.6 (19.6)

Weight, kg

  Mean (SD)

45.8 (5.9)

22.7 (17.3)

14.30 (NA)

34.6 (16.6)

Weight, n (%)

   ≥ 10 to < 20 kg

0

2 (66.7)

1 (100.0)

3 (33.3)

   ≥ 40 to < 50 kg

4 (80.0)

1 (33.3)

0

5 (55.6)

   ≥ 50 to < 60 kg

1 (20.0)

0

0

1 (11.1)

Height (cm)

  Mean (SD)

150.9 (11.2)

112.9 (31.2)

112.2 (NA)

133.9 (26.6)

BMI (kg/m2)

  Mean (SD)

20.2 (2.3)

15.7 (3.2)

11.4 (NA)

17.7 (4.0)

Corticosteroid use

  n (%)

5 (100.0)

1 (33.3)

0

6 (66.7)

Corticosteroid total daily dose (mg)a

  Mean (SD)

8.9 (4.3)

4 (NA)

0

8.08 (4.3)

Corticosteroid dose per weight (mg/kg)a

  Mean (SD)

0.2 (0.1)

0.4 (NA)

0

0.2 (0.1)

  1. AGS Aicardi-Goutières syndrome, BMI body mass index, eGFR estimated glomerular filtration rate, n number of patients with non-missing data, N number of patients in the safety population within each disease subpopulation, NA not applicable, NNS/CANDLE Nakajo-Nishimura syndrome/chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature, SAVI STING-associated vasculopathy with onset during infancy, SD standard deviation, STING stimulator of interferon genes
  2. aThe mean corticosteroid doses are reported as prednisone equivalent doses in patients taking corticosteroids