Table 3 Comparison of patient characteristics according to bronchiectasis or cystic pattern predominance

Demographic data

 Age at onset of symptoms, mean (± SD)

50 ± 6

44 ± 13

0.54

 Age at diagnosis, mean (± SD)

56 ± 6

48 ± 12

0.16

 Female sex, n (%)

6 (85)

6 (40)

0.07

Smoking history, n (%)

4 (57)

12 (80)

0.33

Pneumothorax history, n (%)

0

3 (20)

0.5

Lung function parameters at baseline

 FEV1, % pred, mean (± SD)

107 ± 17

86 ± 26

0.04

 FVC, % pred, mean (± SD)

103 ± 19

93 ± 24

0.33

 FEV1/FVC, %, mean (± SD)

83 ± 4.7

74 ± 10

0.01

 Airflow obstruction, n (%)

0

7 (46)

0.05

 TLC, %pred, mean (± SD)

98 ± 11

108 ± 17

0.17

 DLCO, % pred, mean (± SD)

62.2 ± 19.8

54 ± 24

0.33

 KCO, %pred, mean (± SD)

54.5 ± 33.4

67 ± 20

0.36

 6 min walk test, m, mean (± SD)

565 ± 86.7

496 ± 92

0.16

Change in lung function parameters

 Mean ∆FEV1, ml/year (± SD)

184 ± 139

216 ± 227

0.96

 Mean ∆DLCO, %/year (± SD)

2.76 ± 1.4

4.59 ± 2.7

0.27

Laboratory data

 Mean kappa light chain in serum, mg/L (± SD)

192 ± 205

326 ± 441

0.75

 Mean lambda light chain serum, mg/L (± SD)

9 ± 4.6

11 ± 6.4

0.53