Table 1 Patient characteristics at baseline
From: Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease
Value | Patients with available data, n | |
|---|---|---|
Demographic data | ||
Age at symptom onset, years, median (IQR) | 42.5 (17.5) | 30 |
Age at diagnosis, years, median (IQR) | 50 (20) | 29 |
Female sex, % | 68 | 31 |
Smoking history, % | 67 | 30 |
Pack-years, median (IQR) | 15 (17.5) | 19 |
Clinical data, n (%) Dyspnea, % | 91 | 30 |
Cough, % | 80 | 30 |
Sputum, % | 70 | 30 |
Haemoptysis, % | 30 | 30 |
History of recurrent bronchitis or pneumonia, % | 64.5 | 30 |
History of extrapulmonary infectious disease, % | 6.7 | 30 |
History of spontaneous pneumothorax (≥ 1), % | 17 | 30 |
Palpable lymphadenopathy, % | 10 | 30 |
Laboratory data | ||
IgG, mg/L, mean ± SD (normal reference) | 9.8 ± 4.57 (7.00—16.00) | 22 |
IgA, mg/L, mean ± SD (normal reference) | 1.9 ± 0.82 (0.78- 4.11) | 21 |
IgM, mg/L, mean ± SD (normal reference) | 3.66 ± 3.78 (0.40- 2.80) | 22 |
Increased serum kappa/lambda ratio, % | 91 | 23 |
Mean serum kappa/lamba ratio ± SD | 30.3 (± 43.8) | 22 |
Increased kappa light chain in serum, % | 87 | 23 |
Mean kappa light chain in serum, mg/L ± SD | 236.2 (± 344.4) | 23 |
Increased lambda light chain in serum, % | 0 | 22 |
Mean lambda light chain in serum, mg/L ± SD | 11.4 (± 5.8) | 21 |
Alpha 1 anti-trypsin deficiency, % | 0 | 11 |
Lung function parameters at baseline | ||
FEV1, L, mean ± SD | 2.5 ± 0.78 | 29 |
FEV1, % pred, mean ± SD | 86.2 ± 26.7 | 27 |
FVC, L, mean ± SD | 3.3 ± 1 | 28 |
FVC, % pred, mean ± SD | 92.3 ± 23 | 27 |
FEV1/FVC, %, mean ± SD | 74.9 ± 11.8 | 27 |
TLC, L, mean ± SD | 5.93 ± 1.78 | 26 |
TLC, %, pred, mean ± SD | 105.9 ± 16.8 | 23 |
DLCO, % pred, mean ± SD | 52.5 ± 23.9 | 22 |
KCO, % pred, mean ± SD | 57 ± 27 | 18 |
PaO2, mmHg, mean ± SD | 73.3 ± 13.4 | 19 |
6 min walk test, m, mean ± SD | 503 ± 103 | 12 |