Table of ContentsView AllTable of ContentsTypesSymptomsCausesDiagnosisTreatmentPrognosisCoping
Table of ContentsView All
View All
Table of Contents
Types
Symptoms
Causes
Diagnosis
Treatment
Prognosis
Coping
Idiopathic pulmonary fibrosis is a severe,chroniclung disease. It involves lung inflammation and/or scarring of your lungs' alveoli, or air sacs. The scarring causes stiffness in your lungs. This affects their ability to absorb oxygen from the air, making breathing difficult.
The causes of this illness are not fully known. As a result, this disease is called idiopathic, meaning it has no known cause. However, having certain risk factors can make you more likely to get the disease.
This article describes idiopathic pulmonary fibrosis symptoms, causes, diagnosis, treatment, and outlook.
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Types of Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is one of over 200 different types ofinterstitial lung disease (ILD), a condition that results in inflammation and scarring of the interstitium, a system of tissues that run through your lungs.
The impact of the disease makes the lung tissue stiff and thick. While other idiopathic types of interstitial lung disease exist, idiopathic pulmonary fibrosis is the most common.
Idiopathic pulmonary fibrosis is broken down into stages of illness. These stages are considered mild, severe, or advanced based on the results ofpulmonary function tests.
A more specific classification system for idiopathic pulmonary fibrosis includes the following:
What Are the Types of Fibrosis?
Idiopathic Pulmonary Fibrosis Symptoms
What Is Idiopathic Pulmonary Fibrosis?
While the cause is unknown, idiopathic pulmonary fibrosis often occurs in people who have one of the followingcomorbidities(simultaneous disease):
Having one or more of the following factors can increase your chances of developing idiopathic pulmonary fibrosis with or without another disease present:
What Is Pulmonary Fibrosis?
The diagnosis of idiopathic pulmonary fibrosis can be difficult. Since this disease is rare, it may not be familiar to many healthcare providers. In addition, many symptoms of idiopathic pulmonary fibrosis are also present in other lung diseases. As a result, many people may not get an accurate diagnosis until the disease progresses and symptoms become more specific and harmful with time.
The diagnosis of idiopathic pulmonary fibrosis typically requires more than one test. The following tests are commonly used:
Medical Examination
A medical examination involves a physical exam using a stethoscope to listen to your lungs. It also includes a discussion of the type, duration, and extent of your symptoms and information about your family history. A discussion of your risk factors and other medical conditions is also included.
Imaging Tests
The following imaging tests may be used to look for scarring in your lungs:
Bronchoscopy
Abronchoscopyuses a bronchoscope (a type ofendoscopemade up of a thin tube with a light and camera on the end) to examine your airways and lungs. During the procedure, the bronchoscope is inserted into your nose, down through yourtrachea(windpipe), and into your lungs to collect a small sample of lung tissue or fluid for analysis.
Bronchoalveolar Lavage
Abronchoalveolar lavageremoves fluids from the lower part of your respiratory tract to analyze the fluid for signs of infection or bleeding.
Pulmonary Function Tests
Pulmonary function tests can help diagnose idiopathic pulmonary fibrosis and distinguish it from other lung diseases. These tests include the following:
Blood Tests
Blood testscan detect signs of anautoimmune diseaseor environmental factors contributing to your breathing problems.
Lung Biopsy
Alung biopsy(removing a sample tissue for evaluation in a lab) may be used when the results of other procedures do not provide the data necessary to make a definitive diagnosis. A lung biopsy can be done viafine needle aspirationor as anopen biopsyusing an incision.
Treatment for idiopathic pulmonary fibrosis can slow disease progression and help your lungs work better. The type of treatment you receive depends on several factors that include your age, the presence of unrelated illnesses, your symptoms, and the severity and stage of your disease.
Treatment for idiopathic pulmonary fibrosis can involve any of the following:
Medications
The following antifibrotic medications are used to help slow the rate of scar tissue that causes scarring in the lungs and help patients achieve a better quality of life with idiopathic pulmonary fibrosis:
Lifestyle Changes
Lifestyle changes can involve a wide range of approaches for managing symptoms. These can include the following:
Oxygen Therapy
Oxygen therapyis administering supplemental oxygen to increase oxygen levels to healthy, normal levels. This can help you achieve the following:
Pulmonary Rehabilitation
Pulmonary rehabilitation is a multifaceted type of lung therapy. It can include exercises and education to help you achieve the following:
Lung Transplant
Occasionally, a lung transplant may be the recommended course of treatment. It is typically reserved for those with a severe form of the disease that has not responded to other treatments. A lung transplant is most often used to treat patients without significant complications. People who receive a lung transplant must pass a stringent evaluation process to determine the potential for success of the procedure.
Comorbidity can significantly impact the prognosis of your idiopathic pulmonary fibrosis. Having another disease simultaneously can contribute to delays in diagnosing and treating idiopathic pulmonary fibrosis. This can advance the loss of quality of life and worsen outcomes.
Chronic Thromboembolic Pulmonary Hypertension (CTEPH): What Happens?
Everyone copes with chronic illness differently. Many people benefit frompalliative care, also known as supportive care. This type of care focuses on relieving and preventing symptoms that cause discomfort.
Palliative care aims to improve your quality of life with idiopathic pulmonary fibrosis. It usually involves a team approach that includes physicians, nurses, social workers, psychologists, pharmacists, and other healthcare professionals.
Palliative care does not mean you can’t also receive curative care, which can involve treatment, consideration for lung transplantation, and participation in idiopathic pulmonary fibrosis research studies.
You may also benefit from the following strategies when coping with this disease:
Summary
Idiopathic pulmonary fibrosis is a rare but severe type of chronic lung disease. The illness can have an extreme medical and psychological impact. The effect stiffens and thickens lung tissue, making it harder to breathe during movement. As the condition advances, it can also cause breathing hard during rest and sleep.
The causes of this disease are not fully known, though having certain risk factors can make you more likely to develop it. With symptoms common to many other types of lung disease, this illness can be hard to diagnose. It can sometimes take years if the disease moves slowly and its impact gradually.
Treatment can help reduce symptoms, but the damage caused cannot be improved. The prognosis is usually poor. In severe cases, lung replacement may be a treatment option after other failed therapies.
14 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Lung Association.Idiopathic pulmonary fibrosis (IPF).Pulmonary Fibrosis Foundation.Idiopathic pulmonary fibrosis.Kolb M, Collard HR.Staging of idiopathic pulmonary fibrosis: past, present and future.Eur Respir Rev. 2014;23(132):220-224. doi:10.1183/09059180.00002114National Jewish Health.Idiopathic pulmonary fibrosis (IPF) overview.National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: living with.Alfaro TM, Robalo Cordeiro C.Comorbidity in idiopathic pulmonary fibrosis - what can biomarkers tell us?Ther Adv Respir Dis. 2020;14:1753466620910092. doi:10.1177/1753466620910092National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: causes and risk factors.St. Vincent’s Hospital Lung Health.IPF (idiopathic pulmonary fibrosis).National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: diagnosis.Meyer KC.Diagnosis and management of interstitial lung disease.Transl Respir Med. 2014;2:4. doi:10.1186/2213-0802-2-4Pulmonary Fibrosis Foundation.Supplemental oxygen.Pulmonary Fibrosis Foundation.Pulmonary rehabilitation.Pulmonary Fibrosis Foundation.Lung transplantation.Fujimoto H, Kobayashi T, Azuma A.Idiopathic pulmonary fibrosis: treatment and prognosis.Clin Med Insights Circ Respir Pulm Med. 2016;9(Suppl 1):179-185. doi:10.4137/CCRPM.S23321
14 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Lung Association.Idiopathic pulmonary fibrosis (IPF).Pulmonary Fibrosis Foundation.Idiopathic pulmonary fibrosis.Kolb M, Collard HR.Staging of idiopathic pulmonary fibrosis: past, present and future.Eur Respir Rev. 2014;23(132):220-224. doi:10.1183/09059180.00002114National Jewish Health.Idiopathic pulmonary fibrosis (IPF) overview.National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: living with.Alfaro TM, Robalo Cordeiro C.Comorbidity in idiopathic pulmonary fibrosis - what can biomarkers tell us?Ther Adv Respir Dis. 2020;14:1753466620910092. doi:10.1177/1753466620910092National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: causes and risk factors.St. Vincent’s Hospital Lung Health.IPF (idiopathic pulmonary fibrosis).National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: diagnosis.Meyer KC.Diagnosis and management of interstitial lung disease.Transl Respir Med. 2014;2:4. doi:10.1186/2213-0802-2-4Pulmonary Fibrosis Foundation.Supplemental oxygen.Pulmonary Fibrosis Foundation.Pulmonary rehabilitation.Pulmonary Fibrosis Foundation.Lung transplantation.Fujimoto H, Kobayashi T, Azuma A.Idiopathic pulmonary fibrosis: treatment and prognosis.Clin Med Insights Circ Respir Pulm Med. 2016;9(Suppl 1):179-185. doi:10.4137/CCRPM.S23321
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
American Lung Association.Idiopathic pulmonary fibrosis (IPF).Pulmonary Fibrosis Foundation.Idiopathic pulmonary fibrosis.Kolb M, Collard HR.Staging of idiopathic pulmonary fibrosis: past, present and future.Eur Respir Rev. 2014;23(132):220-224. doi:10.1183/09059180.00002114National Jewish Health.Idiopathic pulmonary fibrosis (IPF) overview.National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: living with.Alfaro TM, Robalo Cordeiro C.Comorbidity in idiopathic pulmonary fibrosis - what can biomarkers tell us?Ther Adv Respir Dis. 2020;14:1753466620910092. doi:10.1177/1753466620910092National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: causes and risk factors.St. Vincent’s Hospital Lung Health.IPF (idiopathic pulmonary fibrosis).National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: diagnosis.Meyer KC.Diagnosis and management of interstitial lung disease.Transl Respir Med. 2014;2:4. doi:10.1186/2213-0802-2-4Pulmonary Fibrosis Foundation.Supplemental oxygen.Pulmonary Fibrosis Foundation.Pulmonary rehabilitation.Pulmonary Fibrosis Foundation.Lung transplantation.Fujimoto H, Kobayashi T, Azuma A.Idiopathic pulmonary fibrosis: treatment and prognosis.Clin Med Insights Circ Respir Pulm Med. 2016;9(Suppl 1):179-185. doi:10.4137/CCRPM.S23321
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National Jewish Health.Idiopathic pulmonary fibrosis (IPF) overview.
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National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: causes and risk factors.
St. Vincent’s Hospital Lung Health.IPF (idiopathic pulmonary fibrosis).
National Heart, Lung, and Blood Institute.Idiopathic pulmonary fibrosis: diagnosis.
Meyer KC.Diagnosis and management of interstitial lung disease.Transl Respir Med. 2014;2:4. doi:10.1186/2213-0802-2-4
Pulmonary Fibrosis Foundation.Supplemental oxygen.
Pulmonary Fibrosis Foundation.Pulmonary rehabilitation.
Pulmonary Fibrosis Foundation.Lung transplantation.
Fujimoto H, Kobayashi T, Azuma A.Idiopathic pulmonary fibrosis: treatment and prognosis.Clin Med Insights Circ Respir Pulm Med. 2016;9(Suppl 1):179-185. doi:10.4137/CCRPM.S23321
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