7
Table 3. Selected Fibrotic Lung Diseases That Can Manifest
PPF
Potentially Fibrotic Interstitial
Lung Diseases Histologic Patterns
Fibrotic non-specific interstitial
pneumonia (F-NSIP)
F-NSIP
Pleuroparenchymal fibroelastosis
(PPFE)
• Intraalveolar fibrosis and elastosis (IAFE)
• May coexist with other patterns such as UIP
in patients with other forms of concomitant
ILD (e.g., IPF)
Fibrosing organizing pneumonia
(FOP)
• Cicatricial organizing pneumonia
• Organizing pneumonia with concomitant
interstitial fibrosis, (sometimes secondary to
diffuse alveolar damage/acute IP)
DIP
DIP
a
Fibrotic CTD-ILD F-NSIP, FOP, UIP (use histopathologic criteria
for idiopathic diseases)
Fibrotic HP • HP and probable HP
• Fibrotic element may be that of either UIP,
F-NSIP, or bronchiolocentric fibrosis
Fibrotic occupational ILD Dependent on occupational lung
disease (asbestosis, fibrotic HP, silicosis,
pneumoconiosis, other)
Fibrotic Langerhan's cell histiocytosis
(F-LCH)
F-LCH
Fibrotic sarcoidosis Discrete non-necrotizing granulomas with a
lymphatic distribution with coexistent fibrosis
Unclassified fibrotic ILD Cases should ideally only be termed
"unclassifiable" after multidisciplinary
discussion. Most cases represent combined or
overlapping patterns of classifiable interstitial
pneumonias and these should be reported as
such.
Other Fibrosis in association with inborn errors of
metabolism, surfactant protein disorders,
pulmonary involvement by systemic disorders,
others
a
Terminolog y for fibrotic interstitial pneumonias with DIP-like features is controversial, this
overlapping with F-NSIP.
