Table 1.
Demographic and Clinical Information | |
Total n. of patients | 129 |
Age (years) | 56 (47-63) |
Gender (Male/Female) | 54/75 |
Smoking habit (Never/Former) | 8 (100/21) |
Median years to diagnosis | 5 (1-10) |
Symptomatic/asymptomatic at disease onset | 99/30 |
GenPhenReSa | |
Abdominal | 16 |
Ocular-cardiac-cutaneous-CNS | 17 |
Musculoskeletal-cutaneous | 10 |
Pulmonary and intrathoracic lymph node | 86 |
Extrapulmonary | 0 |
Lung Function Test parameters (median IQR) | |
FVC% | 104 (90-115) |
FEV1% | 98 (83-108) |
DLCO% | 86 (75-105) |