Table 2.
Detailed characteristics of patients
| Characteristics | Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | Case 6 | Case 7 | Case 8 | Case 9 | Case 10 | Case 11 |
|---|---|---|---|---|---|---|---|---|---|---|---|
| BMI | 24 | 37 | 23 | 19 | 30 | 23 | 21 | 31 | 25 | 21 | 31 |
| Smoking history (pack years) | Former (12 py) | Former (70 py) | Never | Never | Never | Never | Former (20 py) | Former (15 py) | Former (25 py) | Never | Never |
| ILD relevant exposure | no | no | no | Powders, detergents | no | no | no | Cement powders | powders | no | no |
| Autoantibodies | ANA 1:80; ENA negative | ANA 1:320; ENA negative | ANA 1:640; ENA negative | negative | ANA 1:80; ENA negative | ANA 1:1280; ENA negative | ANA 1:160; ANCA 1:32; ENA negative | ANA 1:320; ENA negative | ANA 1:320; ENA negative | ANA 1:160; ENA negative | ANA 1:160; ENA negative |
| FVC L (%) at baseline | 1.9 (63) | 1.82 (48) | 2.74 (77) | 1.26 (52.7) | 2.1 (49) | 2.31 (52.7) | 4.28 (101) | 3.37 (97.7) | 3 (73) | 0.93 (26.5) | 1.47 (51.5) |
| DLCO % at baseline | 38 | 35 | unable | 27.4 | 27 | 30 | 64 | 29 | 37.1 | 33 | 35 |
| TORVAN index | 19 | 21 | 19 | 22 | 14 | 24 | 13 | 17 | 7 | 12 | 21 |
| BAL findings at initial diagnosis | Macrophages 65%, Lymphocytes 13%, Neutrophils 22% | Macrophages 85%, Lymphocytes 12%, Neutrophils 3%, Eosinophils 0% | Macrophage 76%, Lymphocytes 10%, Neutrophils 9%, Eosinophils 3%, Mastcells 2% | / | Macrophage 89%, Lymphocytes 9%, Eosinophils1% |
Macrophages 89%, Lymphocytes 14%, Neutrophils 2% |
Macrophage 96%, Lymphocytes 2%, Neutrophils 2% | Macrophage 91%, Lymphocytes 9%, Neutrophils 10% | Macrophages 77%, Lymphocytes 5%, Neutrophils 8%, Eosinophils 10% | / | / |
| Histological pattern | NSIP | / | Indeterminate | Compatible with PPFE | Indeterminate | Indeterminate | Indeterminate | UIP-like | Indeterminate | / | Indeterminate |
| LTOT at initiation of antifibrotic therapy | Yes | Yes | No | No | No | No | No | Yes | Yes | No | Yes |
| Distance at 6MWT (meters) | 425 | / | 578 | 363 | 313 | 402 | 540 | 323 | 480 | 418 | / |
| MDT diagnosis | iNSIP | uILD (IPAF) | uILD (IPAF) | PPFE | uILD | uILD (IPAF) | uILD | Asbestos-associated fibrosis | ILD in Hermansky-Pudlak syndrome | PPFE | uILD |
| Treatment before antifibrotics | Pred. 30 mg; AZT 125 mg; NAC 1800 mg | Pred. 15 mg; AZT 150 mg; MTX 7,5 mg | Pred. 30 mg; AZT 150 mg | Pred. 10 mg; | Pred. 10 mg; | Pred. 7,5 mg; AZT 150 mg; NAC 1800 mg | Pred. 7,5 mg; Cycl. (6 cycles) | Pred. 15 mg; AZT 150 mg | Pred. 10 mg; | Pred. 10 mg; | Pred. 40 mg; |
| Antifibrotic treatment | Pirf 2403 mg | Nint. 300 mg | Pirf 2403 mg | Pirf 1602 mg | Pirf 2403 mg | Pirf 2403 mg | Pirf 2403 mg | Pirf 2403 mg | Pirf 1602 mg | Pirf 2403 mg | Pirf 2403 mg |
Footnotes: BMI Body mass index, FVC Forced Vital capacity, DLCO Diffusing Capacity for carbon monoxide, BAL Bronchial alveolar lavage, LTOT long term oxygen therapy, MDT multidisciplinary discussion, iNSIP idiopathic-Non specific interstitial Pneumonia, uILD unclassifiable Interstitial lung disease, PPFE pleuroparenchymal fibroelastosis, Pred. prednisone, AZT Azathioprin, MTX Methotrexate, Cycl. cyclophosphamide, Pirf. Pirfenidone, Nint. Nintedanib