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Table 4 Disease activity and inflammatory markers in clinical AID without pathogenic gene variants treated with colchicine

From: Colchicine – an effective treatment for children with a clinical diagnosis of autoinflammatory diseases without pathogenic gene variants

 

FMF

N = 9

CAPS

N = 8

PFAPA

N = 14

Unclassified AID

N = 2

Total cohort

N = 33

Disease activity (PGA) in cm (0 to 10 cm), median (range)

 Baseline

4 (2–5)

4.5 (3–8)

4.5 (3–6)

3.5 (3–4)

4 (2–8)

 First follow-up

2 (0–4)

2.5 (0–4)

2 (0–5)

1 (0–2)

2 (0–5)

 Last follow-up

1 (0–3)

1.5 (0–3)

2 (0–4)

0.5 (0–1)

1 (0–4)

CRP values in mg/dL (N < 0.5 mg/dL)

 Baseline, mean ± SD (tested)

18 ± 30.35 (9/9)

14.7 ± 27.49 (8/8)

8.5 ± 8.72 (14/14)

0.05 ± 0.06 (2/2)

12.1 ± 21.28 (33/33)

 First follow-up, mean ± SD (tested)

1.2 ± 2.15 (8/9)

2 ± 3.38 (6/8)

1.7 ± 5.15 (13/14)

0.01 ± 0.00 (2/2)

1.5 ± 3.85 (29/33)

 Last follow-up, mean ± SD (tested)

1.7 ± 3.13 (6/9)

1 ± 2.55 (8/8)

3 ± 10.22 (13/14)

0.49 ± 0.67 (2/2)

2 ± 7.01 (29/33)

SAA values in mg/L (N < 10 mg/L)

 Baseline, mean ± SD (tested)

442.9 ± 668.45 (8/9)

97.0 ± 117.22 (8/8)

349.5 ± 320.26 (14/14)

22.1 ± 28.21 (2/2)

289.2 ± 410.98 (32/33)

 First follow-up, mean ± SD (tested)

66.8 ± 147.73 (6/9)

6.7 ± 9.70 (7/8)

39.1 ± 95.33 (13/14)

0.8 ± 0 (1/2)

35.4 ± 94.42 (27/33)

 Last follow-up, mean ± SD (tested)

6.7 ± 9.54 (7/9)

51.7 ± 125.56 (7/8)

16.9 ± 34.22 (13/14)

56 ± 77.78 (2/2)

25.6 ± 66.90 (29/33)

Proteinuria, N (%)

 Baseline (tested)

0 (0) (5/9)

0 (0) (3/8)

0 (0) (12/14)

0 (0) (2/2)

0 (0) (22/33)

 First follow-up (tested)

0 (0) (6/9)

0 (0) (5/8)

0 (0) (10/14)

0 (0) (2/2)

0 (0) (23/33)

 Last follow-up (tested)

0 (0) (7/9)

0 (0) (7/8)

0 (0) (11/14)

0 (0) (2/2)

0 (0) (27/33)

  1. Abbreviations: AID autoinflammatory disease, FMF Familial Mediterranean Fever, CAPS Cryopyrin-associated periodic syndromes, PFAPA periodic fever, aphthosis, pharyngitis and adenitis, w weekly, d days, CRP C-reactive protein, SAA serum amyloid A, mg milligram, dL decilitre, L litre