Ten year survival according to age at onset, sex, and clinical variables statistically significant in patients with interval between first symptom and diagnosis equal or lower than five years
| Prognostic factors | Number | Dead | Survival2-150 | SEM | Log rank test | p Value |
|---|---|---|---|---|---|---|
| Age at onset (y) | ||||||
| <60 | 28 | 6 | 0.712 | 0.104 | ||
| 60 | 9 | 3 | 0.583 | 0.186 | 2.87 | 0.090 |
| Sex | ||||||
| Male | 5 | 1 | 0.500 | 0.354 | ||
| Female | 32 | 8 | 0.687 | 0.972 | 0.01 | 0.903 |
| Skin involvement groups | ||||||
| Limited | 24 | 4 | 0.750 | 0.146 | ||
| Diffuse | 13 | 5 | 0.514 | 0.116 | 4.13 | 0.0421 |
| FC < 70% expected value | ||||||
| Absent | 30 | 3 | 0.825 | 0.097 | ||
| Present | 6 | 6 | 0.000 | 0.000 | 37.24 | <0.001 |
| Interstitial pattern | ||||||
| Absent | 23 | 1 | 0.909 | 0.087 | ||
| Present | 14 | 8 | 0.305 | 0.142 | 17.65 | <0.001 |
| PHT | ||||||
| Absent | 32 | 4 | 0.853 | 0.069 | ||
| Present | 5 | 5 | 0.000 | 0.000 | 15.33 | <0.001 |
| PHT detected by Doppler | ||||||
| Absent | 31 | 5 | 0.797 | 0.893 | ||
| Present | 4 | 3 | 0.250 | 0.216 | 4.91 | 0.026 |
| Pericardial effusion | ||||||
| Absent | 24 | 2 | 0.890 | 0.741 | ||
| Present | 11 | 6 | 0.254 | 0.197 | 9.21 | 0.024 |
| Renal involvement | ||||||
| Absent | 31 | 3 | 0.884 | 0.063 | ||
| Present | 6 | 6 | 0.000 | 0.000 | 23.03 | <0.001 |
↵2-150 Survival estimated according Kaplan-Meier method. FVC: forced vital capacity in pulmonary functional testing. PHT: pulmonary hypertension.