c/o exertional sob, renal cardiac normal , suggest management

| Investigation | Purpose |
|---|---|
| HRCT chest (high priority) | Mandatory for ILD characterization — look for honeycombing, traction bronchiectasis, subpleural basilar reticulation (UIP pattern) |
| PFTs (spirometry + DLCO) | Establishes severity — restrictive defect with reduced DLCO |
| 6-minute walk test | Baseline exercise capacity + O₂ desaturation |
| Autoimmune screen (ANA, RF, anti-CCP, Scl-70, Jo-1) | Rule out CTD-ILD (treatable with immunosuppression) |
| Occupational/drug history | Rule out hypersensitivity pneumonitis or drug-induced ILD |
| BAL ± surgical lung biopsy (VATS) | If HRCT is indeterminate; VATS preferred over TBBx |
HRCT showing a definite UIP pattern in the right clinical context is sufficient to diagnose IPF without biopsy.
| Drug | Dose | Mechanism | Effect |
|---|---|---|---|
| Pirfenidone | Up to 2403 mg/day (3 divided doses) | Anti-inflammatory, anti-fibrotic, anti-oxidant | Slows FVC decline, reduces respiratory hospitalizations; pooled data suggest survival benefit |
| Nintedanib | 150 mg orally twice daily | Tyrosine kinase inhibitor (PDGFR, VEGFR, FGFR) | Reduces FVC decline by similar magnitude to pirfenidone; comparable mortality benefit |
| Intervention | Indication/Notes |
|---|---|
| Supplemental O₂ | If SpO₂ <88% at rest or on exertion — improves exercise tolerance |
| Pulmonary rehabilitation | Improves exercise capacity and quality of life |
| Vaccinations | Influenza, pneumococcus, herpes zoster — all appropriate |
| Treat infections promptly | Respiratory tract infections accelerate decline |
| Screen & treat pulmonary hypertension | If present: inhaled treprostinil 72 µg QID can improve exercise capacity; avoid endothelin receptor antagonists (ambrisentan is harmful in IPF) |
| GI/GERD management | PPI/H2 blocker for symptomatic reflux — no proven survival benefit |
| Issue | Comment |
|---|---|
| Lung transplantation | Age >70 is a relative contraindication; not likely suitable here |
| Palliative care | Should be introduced early — dyspnea management (low-dose opioids), goals of care discussion |
| Opioids for refractory dyspnea | Low-dose oral morphine is appropriate for palliation |
Exertional SOB + bilateral basal reticulation on CXR
→ Cardiac/renal excluded ✓
→ HRCT chest (definite UIP = IPF diagnosis)
→ PFTs + DLCO → grade severity
→ Autoimmune screen → CTD-ILD ruled out
→ Start antifibrotic: Pirfenidone OR Nintedanib
→ O₂, pulmonary rehab, vaccines, treat comorbidities
→ Palliative care discussion (age 85)
Sources: Goldman-Cecil Medicine, p. 190–200; Washington Manual of Medical Therapeutics, p. 7187–7219