Pulmonary fibrosis

Overview

Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes scarred and loses its function. The cause is unknown in 50% of cases. The other 50% are caused by autoimmune diseases, the inhalation of pollutants, or infections. The disease presents with growing shortness of breath and a dry tickly cough. Avoiding triggering substances and giving up smoking can have a preventive effect.

Symptoms

Main symptom

• Increasing shortness of breath
  • Initially only on exertion
  • As the disease progresses, also when at rest
• Dry tickly cough

Other symptoms

• Rapid breathing
• Chest pain
• Episodes of fever
• Cyanosis (bluish discolouration)
• Weight loss

Complications

• Cardiac insufficiency
• Lung cancer
• Respiratory failure

Causes and treatment

Causes

• Increase in dysfunctional lung tissue
• The cause is unclear in approx. 50% of cases (idiopathic pulmonary fibrosis)

Secondary causes

• Autoimmune diseases and rheumatic diseases
  • Sarcoidosis (most common cause), lupus, scleroderma
  • More rarely, rheumatoid arthritis, etc.
• Inhalation of pollutants
• Inorganic: silicone, charcoal, asbestos, talcum powder
• Organic allergens: mould or faecal dust (e.g. “pigeon breeder's lung” and “farmer's lung”).
  • Gases, fumes: petrol fumes, organic solvents
• Medication
  • Cytostatics (“cancer drugs”), some antibiotics, cardiac medication and antidepressants
• Chronic cardiac or renal insufficiency
• Pathogens
  • Various viruses, bacteria, fungi, parasites

Further treatment by your doctor / in hospital

Possible tests

• Listening to the lungs
• Lung function tests
• X-rays and CT scan (computed tomography)
• Bronchoscopy
• Blood tests (blood gas analysis)
• Removal of lung tissue samples, if necessary

Possible therapies

• Prevent or delay the progression of the disease
  • Eliminate the impact of the pollutant
  • Cortisone preparations, immunosuppressants
  • Pulmonary rehabilitation (“lung training”)
• Oxygen therapy
• Treatment of cardiac insufficiency
• Last resort: lung transplant

What can I do myself?

• Avoid the triggering factors
• Stop smoking
• Workplace contact with pollutants
  • Occupational hygiene measures (wear a face mask)
  • Regular occupational health checks are essential
  • Consider changing professions, if necessary
• Exercise programme, in particular endurance training

When to see a doctor?

• Shortness of breath
• Persistent tickly cough
• Coughing up blood, blood in sputum
• Chest pressure
• Pain when breathing
• Substantial reduction in physical capacity
• Regular check-ups for workplace contact with pollutants

Further information

Verein für Asbestopfer und Angehörige (Association for Asbestos Victims and their Families)
www.asbestopfer.ch

Swiss Lung Association (Lungenliga Schweiz)
www.lungenliga.ch

Synonyms

pulmonary fibrosis, lung fibrosis, silicosis, asbestosis, interstitial lung disease, diffuse parenchymal lung disease