Table 1.
Baseline Characteristics and Outcomes
| Variable | IPF Cohort (n = 216) | IPAF-UIP Cohort (n = 77) | Combined UIP Cohort (n = 293)a |
|---|---|---|---|
| Age, mean (±SD) | 69.3 (7.8) | 63.3 (9.9) | 67.7 (8.8) |
| Male Gender, n (%) | 160 (74.1) | 41 (53.3) | 201 (68.6) |
| Race, n (%) | |||
| White | 182 (84.3) | 59 (76.6) | 241 (82.3) |
| African American | 15 (6.9) | 9 (11.7) | 24 (8.2) |
| Hispanic | 10 (4.6) | 8 (10.4) | 18 (6.1) |
| Asian | 9 (4.2) | 1 (1.3) | 10 (3.4) |
| Any auto-antibody (+) | 93 (43.1) | 74 (96.1) | 167 (57.0) |
| ANA (+) b | 84 (38.9) | 64 (83.1) | 148 (50.5) |
| HRCT, n (%) | |||
| UIP | 156 (72.2) | 41 (54) | 197 (67.5) |
| Possible UIP | 36 (16.7) | 14 (18.4) | 50 (17.1) |
| Inconsistent with UIP | 24 (11.1) | 21 (27.6) | 45 (15.4) |
| UIP by SLB c, n (%) | 93 (97.9) | 55 (100) | 148 (98.7) |
| FVC (% pred), mean (±SD) | 67.5 (17.4) | 63.5 (16.9) | 66.5 (17.4) |
| DLCO (% pred), mean (±SD) | 51.1 (17.1) | 48.1 (16.8) | 50.3 (17.1) |
| GAP Score, mean (±SD) | 4.1 (1.6) | 3.7 (1.6) | 4.0 (1.6) |
| Outcomes | |||
| Death | 108 (50) | 35 (45.5) | 143 (48.8) |
| Transplant | 14 (6.5) | 14 (18.2) | 28 (9.6) |
Abbreviations: IPF idiopathic pulmonary fibrosis, IPAF interstitial pneumonia with autoimmune features, ANA anti-nuclear antibody, HRCT high-resolution computed tomography, UIP usual interstitial pneumonia, SLB surgical lung biopsy, FVC forced vital capacity, DLCO diffusion capacity of the lung for carbon monoxide, GAP gender, age, physiology
aException for n: Other auto-antibody (n = 88); HRCT (n = 292); UIP by SLB (n = 150); DLCO (n = 272)
bANA titer ≥1:320 or nucleolar or centromere staining pattern at any titer
cTwo patients without UIP by SLB were deemed to have IPF due to family history of pulmonary fibrosis