Idiopathic pulmonary fibrosis (IPF) is also called cryptogenic fibrosing alveolitis (CFA). This is a progressive and chronic form of the lung disorder. It is characterized by the fibrosis of supporting interstitium (framework) of lungs. Idiopathic term is used when the pulmonary fibrosis cause is not known.
Idiopathic pulmonary fibrosis affects nearly 100,000 Americans and also 30 – 40 thousand new cases are diagnosed every year. Typically, IPF is identified in people between the age group of 50 – 70 years. Men are more affected than women. Many patients are the former smokers. There are no evidences about the risk factors of IPF, but few patients have family history of scarring of lungs.
Symptoms of Idiopathic Pulmonary Fibrosis:
Signs and symptoms of idiopathic pulmonary fibrosis appear gradually and include:
- Crackles in lungs can be heard through stethoscope.
- Shortness of breath, especially during or after physical activities
- Clubbing, that is, rounding of finger nails
- Dry, chronic hacking cough
- Difficulty with exertion
IPF symptoms may be similar to other lung disorders like lung scarring. Hence, often diagnosis for the disease involves curing the other conditions.
Diagnosis for Idiopathic Pulmonary Fibrosis:
Inputs are required from radiologists, pulmonologists and in most cases, pathologists who are experienced in evaluating interstitial lung disease patients to diagnose IPF. A discussion is must among these experts to perform accurate diagnosis.
Doctor performs physical examination and enquires about the medical history. IPF patients have abnormal breath sounds known as “crackles”. Patients in advanced stage have blue colored skin around fingernails or mouth because of low oxygen levels. Examination of toes and fingers reveals clubbing. Diagnosing tests include:
- Computed Tomography Chest
- Lung biopsy
- Pulmonary function tests
- Chest X-ray
- Tests for connective tissues disorders, like lupus, rheumatoid arthritis and scleroderma
- Measurement of oxygen levels in blood
- Bronchoscopy
Complications of Idiopathic Pulmonary Fibrosis:
Following are some complications of IPF.
- Pulmonary hypertension
- Respiratory failure
- Polycythemia, that is, abnormally high levels of red blood cells
- Chronic hypoxemia. That is low levels of oxygen in blood
- Cor pulmonale (failure of right side of heart).
