Idiopathic Pulmonary Fibrosis (IPF)
IPF is a chronic, progressive fibrosing interstitial pneumonia primarily affecting older adults. It is characterized by usual interstitial pneumonia (UIP) pattern on high-resolution CT (HRCT) and histology. IPF leads to worsening dyspnea, cough, and eventual respiratory failure.
Symptoms
- Insidious onset of exertional dyspnea
- Persistent dry cough
- Fatigue, weight loss, digital clubbing
- Velcro-like inspiratory crackles on auscultation
- Acute cough/dyspnea spikes due to exacerbations
Diagnosis
- HRCT showing UIP pattern (basal, subpleural reticulation, honeycombing)
- Autoimmune serologies to rule out connective tissue disease
- Pulmonary function tests: restrictive pattern, reduced DLCO
- Six-minute walk test (6MWT) for functional capacity
- Surgical lung biopsy if imaging is indeterminate
Treatment & Management
Antifibrotic Medications
- Pirfenidone: slows FVC decline; monitor for GI upset, photosensitivity, LFTs
- Nintedanib: multi-tyrosine kinase inhibitor; slows progression; monitor for diarrhea, LFTs
- Continue therapy unless severe adverse events
Supportive Care
- Oxygen therapy for hypoxemia
- Pulmonary rehabilitation for conditioning and breathing techniques
- Vaccinations (influenza, COVID-19, pneumococcal, RSV if eligible)
- Treat comorbidities (GERD, pulmonary hypertension, sleep apnea)
- Manage cough with neuromodulators (gabapentin), speech therapy
Acute Exacerbations
- Hospitalization, high-dose steroids, supportive care; prognosis guarded
Lung Transplantation
- Refer early for evaluation (age, comorbidities, psychosocial support)
- Bridge therapies (ECMO) for transplant candidates with rapid decline
Lifestyle & Monitoring
- Avoid smoking and lung irritants
- Balanced nutrition, maintain BMI
- Track FVC, DLCO, 6MWT results, oxygen needs
- Palliative care involvement for symptom control and advance care planning
Complications
- Respiratory failure, pulmonary hypertension, right heart failure
- Increased risk of lung cancer
- Depression/anxiety due to chronic breathlessness
Research & Future Directions
Research focuses on pathways driving fibrosis (TGF-β, integrins, telomerase), precision medicine, and regenerative therapies.
Experimental & Emerging Treatments
- Anti-Integrin & Anti-CTGF Therapies: Agents like pamrevlumab and PLN-74809 aim to halt fibrotic signaling.
- Inhaled Antifibrotics: Delivering drugs directly to lungs to reduce systemic side effects.
- Gene & Telomerase Therapies: Target TERT/TERC mutations in familial pulmonary fibrosis.
- Cell-Based Regeneration: Mesenchymal stem cell infusions investigated for anti-inflammatory, pro-repair effects.
Track IPF with Diagnoza.care
Stay Ahead of Fibrosis – Log PFTs, 6MWT distances, oxygen saturation, medications, side effects, pulmonary rehab sessions, and transplant evaluations; schedule CT scans and specialty visits; capture symptoms and let the AI companion flag accelerating decline.
Medical Disclaimer: Informational only. Follow your pulmonologist or interstitial lung disease specialist for antifibrotic therapy, transplant decisions, and supportive care.
Sources: American Thoracic Society, European Respiratory Society, Pulmonary Fibrosis Foundation