Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissue’s ability to transfer oxygen into the bloodstream.It is a progressive interstitial lung disease with an unknown cause.More specifically, IPF is defined as a distinctive type of chronic fibrosing interstitial pneumonia of unknown cause associated with a histological pattern of usual interstitial pneumonia (UIP).
No one knows what causes pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening makes 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.
Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following:
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 – 70 years old.
*Chest pain (occasionally)
*Chronic dry, hacking cough
*Decreased tolerance for activity
*Shortness of breath during activity that lasts for months or years and over time will also occur at rest
*Fatigue and weakness
*Discomfort in the chest
*Loss of appetite
*Rapid weight loss
Prevalence of Pulmonary Fibrosis:-
There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually. This is the same as die from Breast Cancer. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group.
Exams and Tests
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.
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).
The diagnosis of IPF can be made by demonstrating UIP pattern on lung biopsy in a patient without clinical features suggesting an alternate diagnosis (see clinical features, above). Establishing the diagnosis of IPF without a lung biopsy has been shown to be reliable when expert clinicians and radiologists concur that the presenting features are typical of IPF. Based on this evidence, the 2002 ATS/ERS Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias proposes the following criteria for establishing the diagnosis of IPF without a lung biopsy:
Major criteria (all 4 required):
*Exclusion of other known causes of interstitial lung disease (drugs, exposures, connective tissue diseases)
*Abnormal pulmonary function tests with evidence of restriction (reduced vital capacity) and impaired gas exchange (pO2, p(A-a)O2, DLCO)
*Bibasilar reticular abnormalities with minimal ground glass on high-resolution CT scans
*Transbronchial lung biopsy or bronchoalveolar lavage (BAL) showing no features to support an alternative diagnosis
Minor criteria (3 of 4 required):
*Age > 50
*Insidious onset of otherwise unexplained exertional dyspnea
*Duration of illness > 3 months
*Bibasilar inspiratory crackles
Tests that help diagnose idiopathic pulmonary fibrosis include the following:
*Bronchoscopy with transbronchial lung biopsy
*Chest CT scan
*Measurements of blood oxygen level
*Pulmonary function tests
*Surgical lung biopsy
*Tests for connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma
There are currently no effective treatments or a cure for Pulmonary Fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress.
No known cure exists for idiopathic pulmonary fibrosis. Medications such as corticosteroids and cytotoxic drugs may be given to reduce swelling (inflammation), but these treatments usually don’t work. Oxygen is given to patients who have low blood oxygen levels.
There is a lack of large, randomized placebo-controlled trials of therapy for IPF. Moreover, many of the earlier studies were based on the hypothesis that IPF is an inflammatory disorder, and hence studied anti-inflammatory agents such as corticosteroids. Another problem has been that studies conducted prior to the more recent classification of idiopathic interstitial pneumonias failed to distinguish IPF/UIP from NSIP in particular. Hence, many patients with arguably more steroid-responsive diseases were included in earlier studies, confounding the interpretation of their results.
Small early studies demonstrated that the combination of prednisone with either cyclophosphamide or azathioprine over many months had very modest, if any, beneficial effect in IPF, and were associated with substantial adverse effects (predominantly myelotoxicity). Other treatments studied have included interferon gamma-1b, the antifibrotic agent pirfenidone and bosentan. Pirfenidone and bosentan are currently being studied in patients with IPF while interferon gamma-1b is no longer considered a viable treatment option. Finally, the addition of the antioxidant N-acetylcysteine to prednisone and azathioprine produced a slight benefit in terms of FVC and DLCO over 12 months of follow up. However, the major benefit appeared to be prevention of the myelotoxicity associated with azathioprine
Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly.
Some patients with advanced pulmonary fibrosis may need a lung transplant. Lung rehabilitation will not cure the lung disease, but it can help maintain exercise capacity.
There are a number of new trials testing drugs to treat Pulmonary Fibrosis. For more information contact us at:
Pulmonary Fibrosis Foundation
1332 North Halsted Street Suite 201
Chicago, Illinois 60642-2642
(312) 587-9272 fax (312) 587- 9273
You can ease the stress of illness by joining a support group where members share common experiences and problems.
See also: Lung disease – support group
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.
*Chronic hypoxemia (low blood oxygen level)
*Polycythemia (abnormally high levels of red blood cells)
When to Contact a Medical Professional
Call for an appointment with your health care provider if you develop a regular cough or shortness of breath.
Avoiding smoking may help prevent this condition, but how to prevent the cause is not exactly known.
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose