Idiopathic Pulmonary Fibrosis Flashcards
what is Idiopathic Pulmonary Fibrosis
A progressive lung disorder characterized by chronic inflammation and fibrosis (scarring) of the lung connective tissue, leading to impaired gas exchange and progressive respiratory failure.
what are the symptoms
Exertional dyspnea (shortness of breath with activity)
Dry, nonproductive cough
Inspiratory crackles (Velcro-like)
Clubbing of fingers (due to chronic hypoxia)
Fatigue, weakness
Anorexia, weight loss
what are the risk factors?
Smoking (strongest environmental risk factor)
Exposure to wood and metal dust
Male gender
Age 50-70 years
what does x ray show
Findings are often nonspecific.
what does High-Resolution CT (HRCT) Scan show
Shows honeycombing (fibrotic lung changes).
what does PFT show
Shows restrictive lung disease (↓ vital capacity, ↓ total lung capacity, ↓ diffusion capacity).
what does Open Lung Biopsy (VATS - Video-Assisted Thoracic Surgery) do?
Confirms the diagnosis.
what are the medications given?
Corticosteroids (e.g., prednisone) → Reduces inflammation.
Immunosuppressants (e.g., methotrexate, cyclosporine, azathioprine) → Modulates immune response.
Kinase Inhibitors (e.g., nintedanib, pirfenidone) → Helps slow lung scarring
what is supportive therapy for IPF
Oxygen therapy → Helps manage hypoxia and reduces dyspnea.
Pulmonary rehabilitation → Improves exercise tolerance and quality of life.
when is lung transplant done
Lung Transplant (for eligible patients) → Only curative option; early referral is crucial due to disease progression.
what should pt do?
No cure, but medications help slow progression.
O₂ therapy and pulmonary rehab improve symptoms.
Avoid respiratory infections (vaccinations: flu & pneumococcal).
Smoking cessation and avoiding environmental pollutants.
Early lung transplant evaluation is essential for eligible patients.
