- Overview of Interstitial Lung Diseases
- Overview of Idiopathic Interstitial Pneumonias
- Acute Interstitial Pneumonia
- Cryptogenic Organizing Pneumonia
- Idiopathic Pleuroparenchymal Fibroelastosis
- Idiopathic Pulmonary Fibrosis
- Lymphoid Interstitial Pneumonia
- Nonspecific Interstitial Pneumonia
- Respiratory Bronchiolitis–Associated Interstitial Lung Disease and Desquamative Interstitial Pneumonia
- Drug-Induced Pulmonary Disease
- Eosinophilic Pneumonia
- Hypersensitivity Pneumonitis
- Lymphangioleiomyomatosis
- Pulmonary Alveolar Proteinosis
- Pulmonary Langerhans Cell Histiocytosis
Idiopathic pulmonary fibrosis affects mostly people over the age of 50 years, usually people who have formerly smoked.
People may cough, have difficulty breathing, and feel tired.
Pulmonary rehabilitation, lung transplantation, and medications such as pirfenidone and nintedanib, are treatments.Pulmonary rehabilitation, lung transplantation, and medications such as pirfenidone and nintedanib, are treatments.
(See also Overview of Idiopathic Interstitial Pneumonias and Overview of Interstitial Lung Diseases.)
In idiopathic pulmonary fibrosis, the lungs develop progressive scarring because of many reasons. There seems to be a genetic component because some families have more than one person affected by the disorder. Specific gene mutations have been identified in some people with idiopathic pulmonary fibrosis. People who smoke or who used to smoke are more likely to be affected. Occupational exposures such as inhalation of toxic fumes can cause damage as well.
Symptoms of Idiopathic Pulmonary Fibrosis
Symptoms depend on the extent of the lung damage, the rate at which the disease progresses, and whether complications, such as lung infections and right-sided heart failure (cor pulmonale) develop.
The main symptoms start insidiously as shortness of breath during exertion, cough, and diminished stamina. In most people, symptoms worsen over a period ranging from months to several years.
As the disease progresses, the level of oxygen in the blood decreases, and the skin may take on a bluish tinge (called cyanosis) or the hands and feet may be discolored and cold (acrocyanosis) and the ends of the fingers may become thick or club-shaped (see figure Recognizing Finger Clubbing). In people with dark skin, a subtle grayish instead of bluish discoloration may be present. Strain on the heart may cause the right ventricle to enlarge, eventually resulting in right-sided heart failure. Through a stethoscope, doctors often hear crackling sounds in the lungs.
Diagnosis of Idiopathic Pulmonary Fibrosis
Chest computed tomography
Sometimes lung biopsy
A chest x-ray may show lung damage, mostly in the lower parts of both lungs. Computed tomography (CT) typically shows the damage and thick scarring in more detail. Pulmonary function tests show that the amount of air the lungs can hold is below normal. Analysis of a blood sample (see Arterial Blood Gas Analysis) or use of an oximeter shows a low level of oxygen during minimal exercise (walking at a normal pace) and, as the disease progresses, even when the person is resting.
To confirm the diagnosis, doctors may do a lung biopsy with use of a thoracoscope.
Blood tests cannot confirm the diagnosis but are done as part of the search for other disorders that may cause a similar pattern of inflammation and scarring. For example, doctors do blood tests to look for certain autoimmune disorders.
Treatment of Idiopathic Pulmonary Fibrosis
Pirfenidone or nintedanibPirfenidone or nintedanib
Pulmonary rehabilitation
Treatment of symptoms
Most people continue to get worse despite treatment. Antifibrotic medications may increase the number of people who survive for more than 5 years after diagnosis.
Pirfenidone and nintedanib appear to slow the decline in lung function. Other medications are being studied in clinical trials.Pirfenidone and nintedanib appear to slow the decline in lung function. Other medications are being studied in clinical trials.
Other treatments are aimed at relieving symptoms:
Pulmonary rehabilitation for improving ability to carry out activities of daily life
Oxygen therapy for low blood oxygen levels
Antibiotics for infection
Medications for the heart failure that is caused by cor pulmonale
Lung transplantation (in some cases with a single lung) has been successful in some people with severe idiopathic pulmonary fibrosis.
More Information
The following English-language resource may be useful. Please note that The Manual is not responsible for the content of this resource.
Pulmonary Fibrosis Foundation: Support and educational resources for patients, caregivers, family members, and health care providers
