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Idiopathic Pulmonary Fibrosis — Antifibrotic Therapy

Disease-modifying antifibrotic therapy with pirfenidone and nintedanib for IPF, slowing lung function decline, reducing acute exacerbations, and improving survival in this progressive fibrosing interstitial lung disease.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Göğüs Hastalıkları department. Book Appointment →

What is Idiopathic Pulmonary Fibrosis — Antifibrotic Therapy?

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung disease of unknown etiology with usual interstitial pneumonia (UIP) histopathology, primarily affecting older adults (median age at diagnosis 65). Without treatment, median survival is 3-5 years from diagnosis, with progressive decline in lung function, exercise capacity, and quality of life. Acute exacerbations significantly increase mortality.

Antifibrotic therapy revolutionized IPF management. Pirfenidone (Esbriet) is an oral antifibrotic with anti-inflammatory and antioxidant properties inhibiting TGF-β-mediated fibroblast proliferation and collagen synthesis. Nintedanib (Ofev) is an oral multi-tyrosine kinase inhibitor targeting VEGFR (vascular endothelial growth factor receptor), FGFR (fibroblast growth factor receptor), and PDGFR (platelet-derived growth factor receptor) pathways involved in fibrogenesis.

Both drugs slow annual FVC decline by approximately 50% (from -200 mL/year untreated to -100 mL/year with therapy), reduce risk of acute exacerbations and hospitalizations, and improve progression-free survival. Nintedanib has FDA approval expanded to include systemic sclerosis-associated ILD (SSc-ILD) and progressive pulmonary fibrosis (PPF) of any cause. Treatment is lifelong with monitoring for side effects (GI, hepatic) and disease progression.

Symptoms

Progressive exertional dyspnea (most common presenting symptom)
Dry, persistent, non-productive cough
Bilateral inspiratory crackles ('Velcro' rales) on auscultation
Digital clubbing (50-65% of patients)
Fatigue, weight loss, decreased exercise tolerance
Resting or exertional hypoxemia in advanced disease
Acute exacerbation: rapidly worsening dyspnea, new ground-glass on imaging

Risk Factors

Age >50 years (peak incidence 65-75)
Male sex (3:2 male:female)
Tobacco smoking (current or former)
Family history of pulmonary fibrosis (familial IPF, surfactant gene mutations)
Genetic predisposition: TERT/TERC telomere mutations, MUC5B promoter polymorphism
Environmental/occupational exposure: metal dust, wood dust, silica, agricultural exposures
Gastroesophageal reflux disease (microaspiration association)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Progressive exertional dyspnea persisting >6 months
  • Persistent dry cough without explanation
  • Bilateral inspiratory crackles on examination
  • Abnormal chest imaging suggesting interstitial lung disease
  • Family history of pulmonary fibrosis with respiratory symptoms
  • Newly diagnosed IPF for antifibrotic therapy initiation
  • Acute worsening of dyspnea in established IPF (acute exacerbation evaluation)

Treatment Methods

01
Diagnostic workup: HRCT chest with characteristic UIP pattern (subpleural, basal predominant honeycombing with traction bronchiectasis), pulmonary function tests, 6-minute walk test, autoimmune serology to exclude CTD-ILD
02
Multidisciplinary discussion (pulmonologist, radiologist, pathologist) for IPF diagnosis confirmation
03
Pirfenidone 2,403 mg/day (titrated up over 14 days) with food to minimize GI side effects (nausea, photosensitivity, weight loss)
04
Nintedanib 150 mg twice daily with food, dose-adjust for diarrhea and liver enzyme elevation
05
Supportive care: smoking cessation, vaccinations, pulmonary rehabilitation, oxygen therapy for hypoxemia, GERD management
06
Lung transplantation evaluation for eligible candidates (age <70, no significant comorbidities)
07
Acute exacerbation management: high-dose corticosteroids, supportive ICU care, palliative care for advanced disease

Which Department to Visit?

You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.