Putting It All Together: Typical Scenarios for Patients with Lung Fibrosis
| IPF | NSIP | CHP | S4 | |
|---|---|---|---|---|
| Clinical presentation | 74-year-old male with history of smoking, cough, and progressive DOE | 45-year-old female with history of connective tissue disease and shortness of breath | 68-year-old female. male with shortness of breath and parakeet/parrot (hobbies/occupations) | 30-year-old male with cough, DOE |
| Absent history of smoking | ||||
| Exams | Crackles at lung bases on physical exam | Evidence of rash on extensor surfaces. | Squeaks and airway sounds as well as rales testing for sensitivity to antigens causing fibrosis | Restrictive, obstructive or mixed pulmonary function tests |
| Digital clubbing | Capillary testing of fingers | Restrictive pulmonary function tests with decreased DLCO | Hx of sarcoidosis or evidence of granulomatous inflammation and multisystemic involvement | |
| Restrictive pulmonary function tests with decreased DLCO | Restrictive pulmonary function tests with decreased DLCO | |||
| Radiology | Subpleural basilar predominant fibrosis with honeycombing compatible with ATS criteria for a UIP pattern | Lower lobe fibrosis which follows the bronchovascular bundles and is homogeneous. Sometimes there is peripheral sparring | Heterogeneous fibrosis that follows the bronchovascular bundles with slight upper lobe predominance | Upper lobe posterior fibrosis sometimes with calcified mediastinal lymph nodes |
| There is usually air trapping | ||||
| Treatment | Anti-fibrotic medication | Anti-inflammatory medications | Anti-inflammatory medications | Anti-inflammatory medications |
| Pulmonary rehabilitation | Rheumatology consult | Removal of antigen causing disease if known | ||
| Treat esophageal reflux disease | ||||
| Role of transplant | Referral for transplant evaluation early after diagnosis or for progressive disease | Referral for transplant evaluation for advanced disease | Referral for transplant evaluation for advanced disease | Referral 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.