Pulmonary fibrosis

Causes of pulmonary fibrosis

In a small number of pulmonary fibrosis cases, it’s possible to identify a specific cause.

Some causes are:

  • being exposed to certain types of dust - including wood or metal dusts or asbestos
  • being exposed to allergens - such as bird feathers or mould
  • a side effect of a drug

Some types of pulmonary fibrosis occur when you have another condition such as rheumatoid arthritis or scleroderma.

In a very few cases, two or more members of a family may develop pulmonary fibrosis. But current research suggests the genetics of familial interstitial lung disease is complex and there is no clear inherited predisposition to develop pulmonary fibrosis.

In most types of pulmonary fibrosis a specific cause cannot be found. One of the most frequently occurring forms of fibrosis is called idiopathic pulmonary fibrosis (IPF). The word ‘idiopathic’ means there is no known cause.

Researchers recently set out some common types of interstitial lung disease, grouped by what causes them, in the diagram below. It is not agreed by all doctors and there’s lots of research underway to improve our understanding of the causes.

Classification of common interstitial lung diseases

Interstitial lung disease Idiopathic disorders
  • Idiopathic pulmonary fibrosis
  • Acute interstitial pneumonia (AIP)
  • Idiopathic non-specific interstitial pneumonia (NSIP)
  • Sarcoidosis
  Connective tissue and autoimmune disease
  • Scleroderma / progressive systemic sclerosis
  • Systemic Lupus erythematosis (Lupus)
  • Rheumatoid arthritis
  • Polymyositis / dermatomyositis
  Occupational and environmental
  • Inorganic dust
  • Organic dust
  • Gases and fumes
  • Radiation
  Drug-induced
  • Chemotherapeutic agent
  • Radiation therapy
  • Antiarrhythmics
  • Antibiotics
  • Anticonvulsants
  Infections
  • Viral infections
  • Bacterial infections
  Genetic / inherited
  • Familial pulmonary fibrosis
  • Hermasky-Pudlak syndrome

Adam Wallis and Katherine Spinks: The diagnosis and management of ILDs
British Medical Journal 2015

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Last medically reviewed: August 2016. Due for review: August 2019

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.