Table 2.
Change in values from baseline to the end of the 52nd week in the prospective investigation group
| Characteristic | Baseline value | Value at week 52a | P-value |
|---|---|---|---|
| FVC (% of predicted), mean (s.d.) | 69.1 (14.0) | 81.2 (16.4) | 0.0023 |
| DLCO (% of predicted), mean (s.d.) | 43.7 (8.7) | 49.5 (10.2) | 0.1428 |
| PaO2 at rest (room air) (mmHg), mean (s.d.) | 79.5 (14.2) | 90.7 (20.7) | 0.0118 |
| AaDO2 at rest (mmHg), mean (s.d.) | 29.5 (33.0) | 27.2 (48.6) | 0.8409 |
| Score on Mahler Dyspnoea Index | |||
| Baseline Dyspnoea Index, mean (s.d.) | 6.3 (2.5) | ||
| Transition Dyspnoea Index, mean (s.d.) | 3.0 (5.1) | ||
| CK (IU/l), mean (s.d.) | 948.2 (1259.0) | 115.3 (181.7) | 0.0020 |
| KL-6 (U/l), mean (s.d.) | 1118.8 (740.3) | 957.2 (580.6) | 0.3744 |
| SP-D (μg/ml), mean (s.d.) | 182.8 (132.7) | 124.4 (81.5) | 0.0265 |
| Score for HAQ disability index, mean (s.d.) | 0.95 (0.74) | 0.69 (0.84) | 0.1336 |
| Whole-lung high attenuation score, mean (s.d.) | 52.2 (26.2) | 33.9 (20.4) | 0.0005 |
| SF-36v2 Health Survey score, mean (s.d.) | |||
| Physical functioning score | 48.8 (22.9) | 59.6 (30.6) | 0.0757 |
| Role—physical score | 42.0 (27.8) | 59.1 (31.8) | 0.0377 |
| Bodily pain score | 48.5 (29.2) | 66.3 (24.2) | 0.0214 |
| General health perception score | 50.1 (14.3) | 51.5 (13.3) | 0.7957 |
| Vitality score | 35.8 (23.3) | 59.1 (22.0) | 0.0010 |
| Social functioning score | 46.0 (28.1) | 69.3 (29.0) | 0.0054 |
| Role—emotional score | 50.0 (30.4) | 67.0 (34.5) | 0.0505 |
| Mental health score | 49.6 (23.4) | 67.5 (21.5) | 0.0045 |
| Glucocorticoid dose (mg/kg/day of prednisolone), mean (s.d.)b | 0.81 (0.21) | 0.21 (0.16) (N = 17) | <0.0001 |
The analysis included 25 patients in the prospective investigation group. Scores for the Mahler Transition Dyspnoea Index can range from –9 to +9, with the plus/minus sign indicating improvement/worsening, respectively.
The last-observation-carried-forward method was employed for patients who withdrew before the end of the 52nd week or with missing values for all parameters except for glucocorticoid dose, as explained below.
Average daily glucocorticoid doses during the first 2 weeks of study (for baseline values) and during the 52nd week (for week 52 values) were presented, which do not include pulse glucocorticoid treatment. FVC: forced vital capacity; DLCO: diffusing capacity for carbon monoxide; AaDO2: alveolar-arterial oxygen gradient; CK: creatine kinase; KL-6: Krebs von den Lungen 6; SP-D: surfactant protein D.