Signs & Symptoms of Pulmonary Fibrosis

We often don’t think about our breathing until something takes our breath away. When the lungs become scarred, for example in pulmonary fibrosis, it is hard to get oxygen to the organs and body. The lack of oxygen can cause different signs and symptoms. Knowing what to look for is important so you and your health care provider can come up with a plan to diagnose the problem. 

People with pulmonary fibrosis may have some or all of the following signs and symptoms: 

• feelings of shortness of breath, especially during or after physical activity 
• a dry cough 
• inspiratory crackles – this is a ‘velcro-like’ crackling sound in the lungs that can be heard when listening with a stethescope 
• toe/finger clubbing – about one half of people with idiopathic pulmonary fibrosis or IPF have rounding and widening of the fingers and toes 
• Unintended weight loss and feeling unwell (fatigue and weakness) 

Speak with your doctor or nurse practitioner if you have these signs and symptoms. 

Causes of Pulmonary Fibrosis 

There are a number of known causes of pulmonary fibrosis as well as unknown causes. Known causes of lung scarring (fibrosis) include: 

• Medical tests and treatments 
  • Pulmonary fibrosis that could be caused by a medical test or treatment. Certain medications for inflammation (anti-inflammatory), infections (antibiotic), and cancer (chemotherapy) for example, can cause scarring in the lungs, and medical tests like radiation therapy are also linked to pulmonary fibrosis.  
• Occupational or Environmental Exposures 
  • Inhaling toxic compounds such as coal (coal workers’ pneumoconiosis), silica (silicosis), and asbestos (asbestosis) can cause scarring in the lungs that may lead to pulmonary fibrosis and certain inhaled drugs or chemicals like cocaine, zinc chloride, and ammonia are also known to cause scarring in the lungs. 
• Autoimmune Disease 
  • There is some proof that pulmonary fibrosis can be caused by autoimmune diseases like rheumatoid arthritis, scleroderma, and systemic lupus erythematosus (SLE). In autoimmune disease the body’s natural defense system mistakenly attacks normal cells and causes the disease. 

When no cause for pulmonary fibrosis can be identified the disease is termed idiopathic pulmonary fibrosis or IPF. Idiopathic simply means ‘of unknown cause. 

Risk Factors for Idiopathic Pulmonary Fibrosis 

Although IPF is a disease of unknown cause, there are a number of potential risk factors for the development of IPF that are known. They include: 

• Smoking 
• Environmental Exposures 
  • Certain jobs and occupations such as farming, raising birds, hair dressing, and stone cutting/polishing may be connected. Contact with livestock and different kinds of dust (metal, wood, vegetable and animal) are also risk factors. 
• Viruses 
• Gastroesophageal Reflux Disease (GERD) 
  • GERD is a chronic digestive disease. GERD occurs when stomach acid or content flows back into your food pipe (esophagus). This backwash (reflux) irritates the lining of your esophagus and is what causes GERD. GERD may also be related to IPF, however, more research is still needed in this area. 
• Genetics 
  • Genetics can play a role in IPF. A very small percentage of people with IPF have reported two or more family members with the disease. 
• Gender 
  • IPF is more common in men than women. 
• Age 
  • IPF is more common in people 50 years and older. The risk of getting IPF for both women and men increases with age.