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What is Idiopathic Pulmonary Fibrosis (IPF): Symptoms, Diagnosis & Management

By Dr. Praveen Kumar Pandey in Pulmonology

Oct 28 , 2024 | 1 min read

Idiopathic Pulmonary Fibrosis (IPF) is a chronic and progressive lung disease characterised by scarring of the lung tissue. This leads to impaired lung functions and difficulty breathing. IPF affects approximately 100,000 people every year, with a median survival rate of 2–5 years after diagnosis. In this article, we deep-dive into the diagnosis, treatment, and management of IPF.

What is Idiopathic Pulmonary Fibrosis (IPF)?

IPF is a type of interstitial lung disease (ILD) in which the lung tissue becomes scarred and stiff, making it difficult for oxygen to pass through the lungs and into the bloodstream. The term idiopathic means that the cause of the disease is unknown. IPF typically affects people over 60 years old, with men more likely to develop the condition.

Symptoms of Idiopathic Pulmonary Fibrosis (IPF)

The symptoms of IPF can vary in severity and progress gradually over time. Common symptoms include:

Shortness of breath (dyspnea)
Coughing
Fatigue
Weight loss
Chest tightness or discomfort
Blue-tinged skin (cyanosis)

Diagnosis of Idiopathic Pulmonary Fibrosis (IPF)

Diagnosis of IPF involves a combination of clinical evaluation, imaging tests and pulmonary function tests:

High-Resolution Computed Tomography (HRCT) Scan: Provides detailed images of lung tissue.
Pulmonary Function Tests (PFTs): Measure lung function, including forced vital capacity (FVC) and 7 diffusing capacity of the lung for carbon monoxide (DLCO).
Bronchoscopy: Examines the airway and collects tissue samples.
Biopsy: Obtains lung tissue for histopathological examination.

Treatment Options for IPF

While there is no cure for IPF, treatment focuses on slowing disease progression, managing symptoms and improving quality of life.

Medications:

Pirfenidone (Esbriet) & nintedanib (Ofev) slow disease progression
Corticosteroids & immunosuppressants are utilised to reduce & manage inflammation.

Oxygen Therapy: Supplements of oxygen to alleviate shortness of breath.
Pulmonary Rehabilitation: Combines exercise, education and support to improve lung functions
Lung Transplantation: Considered for advanced cases.

Management and lifestyle changes :

To effectively manage IPF, patients can benefit from combining medical treatment with targeted lifestyle modifications :

Quit Smoking: Essentials for slowing disease progression.
Exercise Regularly: Improves lung function and overall health.
Healthy Diet: Focus on nutrition-rich diet & food
Stress Management: Follow techniques like meditation & yoga.
Influenza and Pneumonia Vaccinations: Prevent respiratory infections.

Conclusion

Idiopathic Pulmonary Fibrosis (IPF) is a complex and debilitating disease requiring a comprehensive treatment approach. Early diagnosis, medication, oxygen therapy, pulmonary rehabilitation and lifestyle modification can improve quality of life and slow disease progression. Ongoing research offers hope for further treatment advancements.