Sex | Female | Female | Female | Female | Male | Female |
Current age yrs | 68 | 46 | 41(died) | 18 | 16 | 14 |
Age of diagnosis yrs | 66 | 46 | 34 | 18 | 16 | 14 |
Age of first evidence yrs | 57 | 44 | 34 | 18 | 16 | 14 |
Age of first symptoms yrs | 63 | None | 34 | None | None | None |
Serum biomarker | | | | | | |
KL-6 U·mL−1 (normal range <500) | 1560 | 386 | NA | 245 | 309 | 332 |
LDH IU·L−1 (normal range 119–229) | 303 | 147 | 166 | 161 | 141 | 144 |
Pulmonary function tests | | | | | | |
VC % pred (normal range >80) | 42.5 | 101.9 | 65.3 | 72.2 | 85 | 96.6 |
FEV1 % pred (normal range >70) | 92.9 | 92.8 | 83.3 | 84.1 | 90.3 | 85 |
DLCO % pred (normal range >80) | 38.5 | 72.2 | NA | 69.3 | NA | 65.2 |
Blood gas analysis (room air) | | | | | | |
Pa,O2 Torr (normal range 75–100) | 68.3¶ | 83.7 | 90.6 | 113 | 109 | 111 |
P(A-a),O2 Torr (normal range <10) | 24.2¶ | 16.2 | 6.1 | -8.5 | -14 | -10.8 |
BAL | | | | | | |
Cell count 105·mL−1 | 1.21 | NA | 3.85 | 2.4 | 2 | 1.4 |
Alveolar macrophages % | 54.2 | NA | 80 | 90 | 86 | 91 |
Lymphocytes % | 10.1 | NA | 17.3 | 7.5 | 12 | 5.8 |
Neutrophils % | 34.5 | NA | 1.1 | 2.5 | 1 | 2.4 |
Eosinophils % | 1.2 | NA | 1.6 | 0 | 1 | 0.8 |
CD4/CD8 ratio | 0.25 | NA | 0.6 | 1.7 | 1.6 | 1.5 |
Histological pattern | UIP | NA | UIP | UIP | UIP | UIP |