Characteristics of all patients during followup.
| Characteristics | Patients | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | |
| Followup, mos | 45 | 120 | 18 | 165 | 46 | 47 | 51 | 20 | 18 | 114 |
| FVC (%) at 6 mos | 85 | 90 | — | 52 | 80 | 98 | 45 | 26 | 45 | 51 |
| TLCO (%) at 6 mos | 46 | 45 | — | 39 | 70 | 72 | 40 | 80 | 46 | 31 |
| FVC (%) at end of followup | 61 | 88 | 75 | 33 | 130 | 96 | 63 | 35 | 46 | 70 |
| TLCO (%) at end of followup | 40 | 48 | 42 | 29 | 43 | 73 | 65 | 75 | 46 | 30 |
| ILD progression at end of study | Yes | Yes | No | Yes | Yes | No | Yes | No | No | Yes |
| Fibrosis at end of followup | Yes | Yes | No | Yes | Yes | Yes | Yes | Yes | Yes | Yes |
| PH, confirmed with RHC | Yes | No | No | No | No | No | No | No | No | Yes |
| mPAP, mmHg | 37 | — | — | — | — | — | — | — | — | 51 |
| PWP, mmHg | 17 | — | — | — | — | — | — | — | — | 8 |
| Death | Yes | No | No | No | No | No | No | No | No | No |
| No. treatment lines | 2 | 4 | 2 | 3 | 3 | 1 | 3 | 2 | 3 | 3 |
| Cumulative treatment | Steroid CYC | Steroid, MTX, AZA, HCQ | Steroid, MTX | Steroid, MTX, CYC | Steroid, MTX, AZA | Steroid | Steroid, AZA, HCQ | Steroid, MTX | Steroid, MTX, HCQ | Steroid, MTX, MMF |
| Pulmonary evolution | Worsened | Stable | Stable | Worsened | Worsened | Improved | Worsened | Improved | Stable | Worsened |
FVC: forced vital capacity; TLCO: carbon monoxide transfer factor; ILD: interstitial lung disease; MMF: mycophenolate mofetil; MTX: methotrexate; CYC: cyclophosphamide; AZA: azathioprine; HCQ: hydroxychloroquine; PH: pulmonary hypertension; RHC: right heart catheterization; mPAP: mean pulmonary arterial pressure; PWP: pulmonary wedge pressure.