Pulmonary fibrosis (PF) describes a group of chronic, progressive fibrotic interstitial lung diseases. The average survival rate of the most common form, idiopathic pulmonary fibrosis (IPF), is 3-5 years from diagnosis. Rapid diagnosis and timely symptom management are important.
Symptoms of pulmonary fibrosis
Symptoms tend to develop gradually and become progressively worse over time. They include:
- shortness of breath
- a persistent dry cough
- tiredness
- loss of appetite and weight loss
- finger clubbing (rounded and swollen fingertips)
- bibasal, inspiratory crackles (dry or “Velcro” type in quality) when auscultating the chest
Risk factors for pulmonary fibrosis
- Older age
- Tobacco smoking (past or present)
- Autoimmune diseases, such as rheumatoid arthritis, sarcoidosis, scleroderma (systemic sclerosis), mixed connective tissue disease (MCTD) and autoimmune myositis
- Occupations where patients may have been persistently exposed to asbestos, dust from wood or stone, metals or coal
- Hobbies and pets where patients may have been exposed to organic dust
- Family history of pulmonary fibrosis, particularly a first-degree relative
- History of radiation or chemotherapy
- Gastroesophageal reflux
Diagnosis and tests
If you suspect pulmonary fibrosis, carry out the following tests and refer your patient to a local respiratory or ILD clinic for further tests as soon as possible:
- spirometry: look for restriction, i.e. reduction in FVC with a normal FEV1/FVC ratio; note that patients with mild pulmonary fibrosis may have normal spirometry
- chest x-ray: interstitial shadowing, reticulation, interstitial fibrosis, or low lung volumes may indicate pulmonary fibrosis
- a high-resolution CT scan of the thorax will demonstrate the pattern of pulmonary fibrosis which may include reticulation, traction bronchiectasis, and honeycombing with or without ground-glass opacities
Key guidelines on IPF diagnosis
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