Pulmonary Fibrosis: Causes, Symptoms, Diagnosis & Treatment
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| Pulmonary Fibrosis: Causes, Symptoms, Diagnosis & Treatment |
Pulmonary Fibrosis: A Complete Medical Guide
Authored by Dr. Ali K. Jawad, MD – Cardiologist & Medical Science Communicator
Pulmonary fibrosis (PF) is a progressive lung disorder in which the tissue deep in the lungs becomes scarred, thickened, and stiff over time. This scarring—known as fibrosis—impairs the lungs’ ability to transfer oxygen into the bloodstream, leading to chronic shortness of breath, fatigue, and reduced quality of life.
This guide provides a detailed, evidence-based overview of pulmonary fibrosis, including its causes, symptoms, diagnostic criteria, treatment options, and supportive care strategies.
What Is Pulmonary Fibrosis?
Pulmonary fibrosis belongs to a group of more than 200 interstitial lung diseases (ILDs). The hallmark is the accumulation of extracellular matrix proteins (like collagen) in the lung interstitium—the space around the air sacs (alveoli). As fibrosis advances, the lungs lose elasticity and compliance, making breathing increasingly difficult.
Types of Pulmonary Fibrosis
- Idiopathic Pulmonary Fibrosis (IPF): The most common and severe form; cause unknown; typically affects adults over 60.
- Hypersensitivity Pneumonitis: Caused by repeated inhalation of allergens (e.g., mold, bird proteins).
- Connective Tissue Disease-Associated PF: Seen in rheumatoid arthritis, scleroderma, or lupus.
- Occupational/Environmental PF: From asbestos (asbestosis), silica (silicosis), or coal dust (black lung disease).
- Drug- or Radiation-Induced PF: Linked to chemotherapy, amiodarone, or thoracic radiation.
Common Causes & Risk Factors
- Advanced age (>50 years)
- Cigarette smoking (current or former)
- Genetic predisposition (e.g., mutations in TERC, TERT)
- Male sex
- Chronic microaspiration or GERD
- Viral infections (e.g., Epstein-Barr virus)
Symptoms
- Progressive dyspnea (shortness of breath), initially with exertion
- Dry, hacking cough unresponsive to typical remedies
- Constant fatigue
- Unexplained weight loss
- Finger or toe clubbing (enlargement of fingertips)
Diagnosis
Diagnosis requires a multidisciplinary approach involving pulmonologists, radiologists, and sometimes pathologists:
- High-Resolution Computed Tomography (HRCT): Key imaging test—shows reticular opacities, honeycombing, and traction bronchiectasis.
- Pulmonary Function Tests (PFTs): Reveal a restrictive pattern—reduced FVC, TLC, and DLCO.
- 6-Minute Walk Test: Assesses oxygen desaturation during activity.
- Bronchoalveolar Lavage (BAL) or Surgical Lung Biopsy: Used in select cases for definitive diagnosis.
Treatment & Management
While no cure exists, several strategies can slow progression and improve symptom control:
- Antifibrotic Medications:
- Pirfenidone (Esbriet®) – reduces fibroblast proliferation and TGF-β activity.
- Nintedanib (Ofev®) – tyrosine kinase inhibitor that blocks fibrotic signaling pathways.
- Supplemental Oxygen Therapy: For patients with resting or exertional hypoxemia.
- Pulmonary Rehabilitation: Structured program including exercise training, breathing techniques, and nutritional counseling.
- Lung Transplantation: For eligible patients <65 advanced="" disease.="" li="" with="" years="">
- Symptom Management: Cough suppressants (e.g., low-dose morphine), GERD treatment, and vaccinations (flu, pneumococcal).
Prognosis
IPF has a median survival of 3–5 years from diagnosis. However, early diagnosis, antifibrotic therapy, and comprehensive care can significantly improve outcomes and quality of life.
Scientific References
- Raghu G, et al. ATS/ERS/JRS/ALAT Clinical Practice Guideline: Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2022;205(9):e18–e47. DOI
- American Thoracic Society – PF Guidelines
- National Center for Biotechnology Information (NCBI) – Pulmonary Fibrosis Overview
- Pulmonary Fibrosis Foundation (PFF) – Patient Resources
Frequently Asked Questions (FAQs)
Q: Is pulmonary fibrosis the same as COPD?
A: No. COPD is an obstructive lung disease (air can’t exit easily), while PF is restrictive (lungs can’t expand fully due to scarring).
Q: Can pulmonary fibrosis be reversed?
A: No—existing scar tissue is permanent. However, antifibrotic drugs can slow or halt further scarring.
Q: Are there clinical trials available?
A: Yes. Visit ClinicalTrials.gov and search “pulmonary fibrosis” for active studies.
Q: Should family members be screened?
A: Only if there’s a known family history of PF (familial PF). Genetic counseling may be recommended.
We Value Your Input
If you’ve been diagnosed with pulmonary fibrosis—or care for someone who has—we warmly invite you to share your questions, insights, or experiences in the comments below. Your voice may provide hope or clarity to others navigating this condition.
Thank you for reading with care and curiosity. Knowledge is power, and every patient deserves dignity, support, and evidence-based care.
— Dr. Ali K. Jawad