Pulmonary Fibrosis

Also called: IPF, Idiopathic pulmonary fibrosis

Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. This tissue gets thick and stiff. That makes it hard for you to catch your breath, and your blood may not get enough oxygen.

Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. In most cases, the cause cannot be found. This is called idiopathic pulmonary fibrosis.

Symptoms include

  • Shortness of breath
  • A dry, hacking cough that doesn't get better
  • Fatigue
  • Weight loss for no known reason
  • Aching muscles and joints
  • Clubbing, which is the widening and rounding of the tips of the fingers or toes

Your doctor may use your medical history, imaging tests, a biopsy, and lung function tests to diagnose pulmonary fibrosis. There is no cure. Treatments can help with symptoms and improve your quality of life. They include medicines, oxygen therapy, pulmonary rehabilitation, or a lung transplant.

NIH: National Heart, Lung, and Blood Institute

Symptoms of Pulmonary Fibrosis

The following features are indicative of Pulmonary Fibrosis:
  • shortness of breath
  • dry cough
  • fatigue
  • unexplained weight loss
  • aching muscles and joints
  • widening and rounding of the tips of the fingers or toes
It is possible that Pulmonary Fibrosis shows no physical symptoms and still is present in a patient.

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Common Causes of Pulmonary Fibrosis

The following are the most common causes of Pulmonary Fibrosis:
  • long-term exposure to silica dust, asbestos fibers, hard metal dusts, coal dust and grain dust
  • exposure to radiation therapy
  • exposure to chemotherapy drugs, heart medications such as amiodarone, antibiotics such as nitrofurantoin or ethambutol and anti-inflammatory drugs such as rituximab or sulfasalazine

Other Causes of Pulmonary Fibrosis

The following are the less common causes of Pulmonary Fibrosis:
  • dermatomyositis
  • polymyositis
  • mixed connective tissue disease
  • systemic lupus erythematosus
  • rheumatoid arthritis
  • sarcoidosis
  • scleroderma
  • pneumonia

Risk Factors for Pulmonary Fibrosis

The following factors may increase the likelihood of Pulmonary Fibrosis:
  • radiation exposure
  • genetic factors
  • middle-aged and older adults

Prevention of Pulmonary Fibrosis

Yes, it may be possible to prevent Pulmonary Fibrosis. Prevention may be possible by doing the following:
  • avoid smoking
  • maintain a healthy diet
  • regular exercise

Occurrence of Pulmonary Fibrosis

Number of Cases

The following are the number of Pulmonary Fibrosis cases seen each year worldwide:
  • Common between 1 - 10 Million cases

Common Age Group

Pulmonary Fibrosis most commonly occurs in the following age group:
  • Aged between 15-60 years

Common Gender

Pulmonary Fibrosis can occur in any gender.

Lab Tests and Procedures for Diagnosis of Pulmonary Fibrosis

The following lab tests and procedures are used to detect Pulmonary Fibrosis:
  • Chest X-ray: To generate the images of the chest
  • Computerized tomography (CT) scan: To produce the cross-sectional images of internal structures in the body
  • Echocardiogram: To visualize the heart
  • Pulmonary function testing: To measure how much air the lungs can hold and how quickly the air move in and out
  • Pulse oximetry: To measure the oxygen saturation in the blood
  • Exercise stress test: To monitor the lung function when you're active
  • Arterial blood gas test: To test a sample of the blood, usually taken from an artery in the wrist
  • Biopsy: Microscopical examination of a small lung tissue

Doctor for Diagnosis of Pulmonary Fibrosis

Patients should visit the following specialists if they have symptoms of Pulmonary Fibrosis:
  • Thoracic Surgeon
  • Pulmonologist

Complications of Pulmonary Fibrosis if untreated

Yes, Pulmonary Fibrosis causes complications if it is not treated. Below is the list of complications and problems that may arise if Pulmonary Fibrosis is left untreated:
  • pulmonary hypertension
  • right-sided heart failure
  • respiratory failure
  • lung cancer
  • lung infections

Procedures for Treatment of Pulmonary Fibrosis

The following procedures are used to treat Pulmonary Fibrosis:
  • Lung transplant: Improves the quality of life and allow you to live a longer life
  • Oxygen therapy: Makes breathing easier and prevent complications from low blood oxygen levels
  • Pulmonary rehabilitation: Manage the symptoms and improve your daily functioning

Self-care for Pulmonary Fibrosis

The following self-care actions or lifestyle changes may help in the treatment or management of Pulmonary Fibrosis:
  • Regular physical exercise: Improves your endurance
  • Use breathing techniques: Improves the lung efficiency
  • Stop smoking: Keep the lungs healthy
  • Eat well: Avoid trans fat and saturated fat diet to lessen the chances of getting disease
  • Get pneumonia vaccination: Receive pneumonia vaccine and an annual flu shot

Patient Support for Treatment of Pulmonary Fibrosis

The following actions may help Pulmonary Fibrosis patients:
  • Education about your condition: Learning more about the disease can help you and your family coping with disease
  • Attend pulmonary rehabilitation: Manage the symptoms and improve your daily functioning
  • Spend time with family and friends: Helps in coping with fear and stress during the disease
  • Join support group: Talk to other people who have had similar symptoms and discuss more coping strategies

Time for Treatment of Pulmonary Fibrosis

While time-period of treatment for each patient may vary, below is the typical time-period for Pulmonary Fibrosis to resolve if treated properly under an expert supervision:
  • In 3 - 6 months

Last updated date

This page was last updated on 2/04/2019.
This page provides information for Pulmonary Fibrosis.
Interstitial Lung Diseases
Pulmonary Rehabilitation

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