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Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.
Alternative NamesIdiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
Causes, incidence, and risk factorsNo one knows what causes pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening may make it increasingly difficult to breathe. In some people the disease gets worse quickly (over months to a few years), but other people have little worsening of the disease over time.
The condition is believed to result from an inflammatory response to an unknown substance. "Idiopathic" means no cause can be found. The disease occurs most often in people between 50 and 70 years old.
Symptoms- Chest pain(occasionally)
- Cough (usually dry)
- Decreased tolerance for activity
- Shortness of breath during activity (this symptom lasts for months or years, and over time may also occur when at rest)
The health care provider will perform a physical exam and ask questions about your medical history. Your doctor will ask whether you have been exposed to asbestos and if you have been a smoker.
Patients with idiopathic pulmonary fibrosis have abnormal breath sounds called crackles. Patients with advanced disease may have blue-colored skin (cyanosis) around the mouth or in the fingernails due to low oxygen.
Examination of the fingers and toes may show abnormal enlargement of the fingernail bases (clubbing).
Tests that help diagnose idiopathic pulmonary fibrosis include the following:
- Bronchoscopy with transbronchial lung biopsy
- Chest CT scan
- Chest x-ray
- Measurements of blood oxygen level
- Pulmonary function tests
- Surgical lung biopsy
- Tests for connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma
No known cure exists for idiopathic pulmonary fibrosis. Unfortunately, no medication has been shown to improve the outcome of patients with this condition.
- For some people, medications such as corticosteroids and cytotoxic drugs may help reduce swelling (inflammation).
- Ongoing clinical studies are experimenting with new treatments that have been shown to help some people with idiopathic pulmonary fibrosis.
- Oxygen may be necessary in patients who have low blood oxygen levels.
- Lung rehabilitation will not cure the disease, but can help maintain exercise capacity (the ability to exercise without breathing difficulty).
Some patients with advanced pulmonary fibrosis may need a lung transplant.
Support GroupsYou can ease the stress of illness by joining a support group where members share common experiences and problems.
See also: Lung disease - support group
Expectations (prognosis)Some patients may improve when they are treated with corticosteroids or cytotoxic drugs, but in most people the disease can get worse even with treatment. This worsening can happen quickly, or very slowly.
Complications- Chronic hypoxemia (low blood oxygen level)
- Cor pulmonale
- Pneumothorax
- Polycythemia (abnormally high levels of red blood cells)
- Pulmonary hypertension
- Respiratoryfailure
Call for an appointment with your health care provider if you develop:
- A regular cough
- Shortness of breath
Avoiding smoking may help prevent this condition, but how to prevent the cause is not known.
ReferencesAmerican Thoracic Society (ATS) and European Respiratory Society (ERS). Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement. Am J Respir Crit Care Med. 2000; 161:646-664.
Noth I, Martinez FJ. Recent advances in idiopathic pulmonary fibrosis. Chest. 2007; 132(2).
What else can I help you with?
What did evil cannieval die from?
Idiopathic pulmonary fibrosis
Is alpha 1 anTitrypsin deficiency the same as idiopathic pulmonary fibrosis?
no
Your father was diagnosed with idiopathic pulmonary fibrosis What is the prognosis of his life expectancy?
About 5 to 7 years.
What is the treatment for idiopathic pulmonary fibrosis?
The treatment for idiopathic pulmonary fibrosis (IPF) typically involves a combination of medications to help slow down the progression of the disease and manage symptoms. Common medications include pirfenidone and nintedanib, which are both anti-fibrotic drugs. Supplemental oxygen therapy may also be prescribed to help with breathing difficulties. In some cases, lung transplant may be considered for advanced stages of the disease. Regular monitoring and follow-up with a healthcare provider specializing in pulmonary fibrosis is essential for managing IPF effectively.
Are lingular and pulmonary fibrosis the same?
No, lingular and pulmonary fibrosis are not the same. Pulmonary fibrosis is a condition where the lung tissue becomes thick and stiff, and the air sacs of the lungs become scarred. This can cause difficulty breathing, coughing, and chest pain. Lingular fibrosis is a specific type of pulmonary fibrosis. It is a form of idiopathic interstitial pneumonia and affects the middle lobe of the lung. It is more common in women than in men and is seen in people who are between the ages of 40 and 70. Pulmonary fibrosis is a progressive and often fatal condition that is caused by an unknown etiology and is characterized by scarring of the lung tissue. This scarring can cause the tissue to become thick and stiff, which can make it difficult for air to move in and out of the lungs, leading to shortness of breath and other respiratory symptoms. Common causes of pulmonary fibrosis include environmental exposures, medications, radiation therapy, and autoimmune conditions. Lingular fibrosis is a form of idiopathic interstitial pneumonia that affects the middle lobe of the lung. It is more common in women than in men, and is seen in people who are between the ages of 40 and 70. The cause of lingular fibrosis is unknown, but it is thought to be related to an autoimmune condition. It is characterized by scarring of the lung tissue, and can cause difficulty breathing, coughing, and chest pain. The symptoms of both pulmonary fibrosis and lingular fibrosis are similar, but the two conditions are distinct. Pulmonary fibrosis affects the entire lung, while lingular fibrosis only affects the middle lobe. In addition, the causes of the two conditions are different. While the cause of pulmonary fibrosis is unknown, lingular fibrosis is thought to be related to an autoimmune condition. Therefore, lingular and pulmonary fibrosis are not the same.
Can a person with pulmonary fibrosis have general anesthesia?
can a person with pulmonary fibrosis have general anathesia
What does idf normally stand for?
There are a lot of things that the acronym IDF can represent. Some options are Israel Defense Force, International Dairy Federation, and Idiopathic Pulmonary Fibrosis.
What are some symptoms of a pulmonary fibrosis?
Some symptoms of pulmonary fibrosis are cough, reduced physical endurance and difficulty when breathing. Lung transplantation is the only option of treatment for pulmonary fibrosis.
When and how did Evel Knievel die?
He had been suffering from diabetes and idiopathic pulmonary fibrosis for many years.
Can pulmonary fibrosis be reversed to normal?
No.
What is the difference between pulmonary fibrosis and pulmonary sclerosis?
They are essentially the same.
Can a pulmonary fibrosis be totally eliminated in the lungs?
no
