Table 1.
Spectrum of interstitial lung diseases according to the ILD registry.
| ILD Variants | ILD without PF, n (%) |
ILD with PF, n (%) |
ILD with PPF, n (%) |
|---|---|---|---|
| All ILD | 166 (61.5) | 104 (38.5) | 62 (23.0) |
| ILD with known etiology | 38 (40.9) | 55 (59.1) | 30 (32.3) |
| CHP | 2 (9.1) | 20 (90.9) | 16 (72.7) |
| CTD-ILD | 22 (40.7) | 32 (59.3) | 13 (24.1) |
| SSc-ILD | 0 | 13 (100.0) | 5 (38.5) |
| RA-ILD | 8 (40.0) | 12 (60.0) | 4 (20.0) |
| Other autoimmune ILD # | 14 (66.7) | 7 (33.3) | 4 (19.0) |
| DI-ILD | 14 (82.4) | 3 (17.6) | 1 (5.9) |
| IIP (total) | 24 (38.1) | 39 (61.9) | 26 (41.3) |
| IPF | 0 | 16 (100.0) | 16 (100.0) |
| iNSIP | 6 (28.6) | 15 (71.4) | 5 (23.8) |
| Unclassifiable IP | 4 (44.4) | 5 (55.6) | 3 (33.3) |
| IPAF | 2 (40.0) | 3 (60.0) | 2 (40.0) |
| COP | 12 (100.0) | 0 | 0 |
| Sarcoidosis | 68 (91.9) | 6 (8.1) | 3 (4.1) |
| Rare ILD | 22 (100.0) | 0 | 0 |
| ICEP | 9 (100.0) | 0 | 0 |
| LCH | 5 (100.0) | 0 | 0 |
| LAM | 5 (100.0) | 0 | 0 |
| PAP | 3 (100.0) | 0 | 0 |
| Other ILD * | 14 (77.8) | 4 (22.2) | 3 (16.7) |
ILD—interstitial lung diseases; PF—pulmonary fibrosis; PPF—progressive pulmonary fibrosis; CHP—chronic hypersensitive pneumonitis; SSc—systemic sclerosis; RA—rheumatoid arthritis; DI-ILD—drug-induced ILD; IIP—idiopathic interstitial pneumonia; IPF—idiopathic pulmonary fibrosis; iNSIP—idiopathic nonspecific interstitial pneumonia; IP—interstitial pneumonia; IPAF—interstitial pneumonia with autoimmune features; COP—cryptogenic organizing pneumonia; ICEP—idiopathic chronic eosinophilic pneumonia; LCH—langerhans cell histiocytosis; LAM—lymphangioleiomyomatosis; PAP—pulmonary alveolar proteinosis; # ILD, associated with ankylosing spondylitis, mixed connective tissue disease, systemic lupus erythematosus, Sjogren ‘s disease, dermatopolymiositis, and vasculitis. * other ILD included pneumoconiosis, ILD associated with HIV, lymphocytic interstitial pneumonia in patient with general variable immune insufficiency, ILD associated with nonspecific ulcerative colitis, lymphoid interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, pulmonary amyloidosis, and pulmonary alveolar microlithiasis.