6
Tables
Table 2. Definition of PPF
In a patient with ILD of known or unknown etiolog y other than IPF who has
radiological evidence of pulmonary fibrosis, PPF is defined as at least two of the
following three criteria occurring within the past year with no alternative explanation:
a
1 Worsening respiratory symptoms.
2 Physiological evidence of disease progression (either of the following ):
1. Absolute decline in the forced vital capacity (FVC) ≥5% predicted within one
year of follow-up, or
2. Absolute decline in the diffusing capacity of the lungs for CO (D
L
CO)
(corrected for hemoglobin [Hb]) ≥10% predicted within one year of follow-up.
3 Radiological evidence of disease progression (one or more of the following ):
1. Increased extent or severity of traction bronchiectasis and bronchiolectasis
2. New ground-glass opacity (GGO) with traction bronchiectasis
3. New fine reticulation
4. Increased extent or increased coarseness of reticular abnormality
5. New or increased honeycombing
6. Increased lobar volume loss.
a
Although it is critical to exclude alternative explanations of worsening features for all patients
with suspected progression, this is particularly important in patients with worsening respiratory
symptoms and/or decline in D
L
CO given the lower specificity of these features for PPF compared
to FVC and chest CT.
