From: Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease
Bronchiectasis pattern (n = 7) | Cystic pattern (n = 15) | p | |
---|---|---|---|
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 |