Table 3.

Putting It All Together: Typical Scenarios for Patients with Lung Fibrosis

Clinical presentation74-year-old male with history of smoking, cough, and progressive DOE45-year-old female with history of connective tissue disease and shortness of breath68-year-old female. male with shortness of breath and parakeet/parrot (hobbies/occupations)30-year-old male with cough, DOE
Absent history of smoking
ExamsCrackles at lung bases on physical examEvidence of rash on extensor surfaces.Squeaks and airway sounds as well as rales testing for sensitivity to antigens causing fibrosisRestrictive, obstructive or mixed pulmonary function tests
Digital clubbingCapillary testing of fingersRestrictive pulmonary function tests with decreased DLCOHx of sarcoidosis or evidence of granulomatous inflammation and multisystemic involvement
Restrictive pulmonary function tests with decreased DLCORestrictive pulmonary function tests with decreased DLCO
RadiologySubpleural basilar predominant fibrosis with honeycombing compatible with ATS criteria for a UIP patternLower lobe fibrosis which follows the bronchovascular bundles and is homogeneous. Sometimes there is peripheral sparringHeterogeneous fibrosis that follows the bronchovascular bundles with slight upper lobe predominanceUpper lobe posterior fibrosis sometimes with calcified mediastinal lymph nodes
There is usually air trapping
TreatmentAnti-fibrotic medicationAnti-inflammatory medicationsAnti-inflammatory medicationsAnti-inflammatory medications
Pulmonary rehabilitationRheumatology consultRemoval of antigen causing disease if known
Treat esophageal reflux disease
Role of transplantReferral for transplant evaluation early after diagnosis or for progressive diseaseReferral for transplant evaluation for advanced diseaseReferral for transplant evaluation for advanced diseaseReferral for transplant evaluation for advanced disease
  • CHP, chronic hypersensitivity pneumonitis; DLCO, Diffusion Capacity of Lungs for Carbon Monoxide; DOE, dyspnea on exertion; IPF, idiopathic pulmonary fibrosis; NSIP, nonspecific interstitial pneumonitis; S4, stage 4 sarcoidosis.