ILD cases | Population >15 years of age# | |||
Subjects n | Prevalence per 100 000 | Subjects n | Incidence per 100 000 per year | |
All identified cases | 1170 | 97.9 | 232 | 19.4 |
Reviewed cases | 848 | 71.0 | 219 | 18.3 |
ILDs of known cause | 260 | 21.8 | 77 | 6.5 |
CTDs/vasculitis | 145 | 12.1 | 39 | 3.3 |
Pneumoconioses | 42 | 3.5 | 9 | 0.8 |
Drug-induced ILDs | 31 | 2.6 | 14 | 1.2 |
HP | 28 | 2.3 | 11 | 0.9 |
Radiation-induced pneumonitis | 7 | 0.6 | 1 | 0.1 |
Others¶ | 7 | 0.6 | 2 | 0.3§ |
IIPs | 145 | 12.14 | 52 | 4.4 |
IPF | 98 | 8.2 | 33 | 2.8 |
NSIP | 20 | 1.7 | 10 | 0.8 |
Desquamative interstitial pneumonia | 10 | 0.8 | 3 | 0.3 |
Organising pneumonia | 9 | 0.8 | 1 | 0.1 |
Unclassified (despite SLB) | 6 | 0.5 | 5 | 0.4 |
RBILD | 2 | 0.2 | 0 | 0.0 |
LIP | 0 | 0.0 | 0 | 0.0 |
Sarcoidosis | 361 | 30.2 | 58 | 4.9 |
Particular ILDs | 22 | 1.8 | 10 | 0.8 |
LAM | 9 | 0.8 | 4 | 0.3 |
CIEP | 5 | 0.4 | 1 | 0.1 |
PLCH | 4 | 0.3 | 2 | 0.2 |
PAP | 2 | 0.2 | 1 | 0.1 |
Others+ | 2 | 0.2 | 2 | 0.1 |
Undetermined diagnosis | 60 | 5.0 | 22 | 1.8 |
Differential diagnosis between IPF and NSIP | 34 | 2.9 | 13 | 1.1 |
CTDs: connective tissue diseases; HP: hypersensitivity pneumonitis; IIPs: idiopathic interstitial pneumonias; IPF: idiopathic pulmonary fibrosis; NSIP: nonspecific interstitial pneumonia; SLB: surgical lung biopsy; RBILD: respiratory bronchiolitis with ILD; LIP: lymphoid interstitial pneumonia; LAM: lymphangioleiomyomatosis; CIEP: chronic idiopathic eosinophilic pneumonia; PLCH: pulmonary Langerhans’ cell histiocytosis; PAP: pulmonary alveolar proteinosis. #: 1 194 601 inhabitants; ¶: including chronic inflammatory colitis (n=4), amyloidosis (n=1), immunoglobulin G4 syndrome (n=1) and progeria (n=1); +: including alveolar microlithiasis (n=1) and Erdheim Chester's disease (n=1).