Table 2.

Overview of Common Fibrotic Lung Diseases

UIPOlder ageSubpleuralAnti fibrotic medications
Male sexBasilar predominantConsider referral for lung transplant soon after diagnosis as course is unpredictable and inexorable
Smoking historyHoneycombingAnti-inflammatory and immunosuppressive Rx NOT indicated (may be harmful)
Crackles at lung bases
NSIPYounger ageHomogeneousAnti-inflammatory medications
Female sexBronchovascularConsider referral for lung transplant if advanced disease
Connective tissue diseaseLower lobe
Positive serologiesDilated esophagus
Peripheral sparring
CHPOlder agePeribronchiolar fibrosisAnti-inflammatory medications
FemaleAir trappingRemoval of causative antigen
Bird ownerUpper lobeConsider referral for lung transplant if advanced disease
Mold exposure
S4Younger ageUpper lobe posteriorAnti-inflammatory medications
Less symptomaticCalcified lymph nodesConsider transplantation for advanced disease (PHN, hypoxemia, declining PFT's)
Peribronchiolar fibrosis
  • CHP, chronic hypersensitivity pneumonitis; NSIP, non-specific interstitial pneumonitis; S4, stage 4 sarcoidosis; PFT, Pulmonary Function Test; PHN, pulmonary hypertension; UIP, usual interstitial pneumonitis.