What Is Idiopathic Pulmonary Fibrosis?
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Medically reviewed by Susan Russell, MD
Idiopathic pulmonary fibrosis is a severe, chronic lung 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.
Lung damage caused by this disease cannot be changed. The illness becomes worse over time, making it harder to breathe. Treatment can help improve symptoms and slow the progress of the damage.
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 of interstitial 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 of pulmonary function tests.
A more specific classification system for idiopathic pulmonary fibrosis includes the following:
Stage 1: A recently diagnosed person with idiopathic pulmonary fibrosis
Stage 2: A person with idiopathic pulmonary fibrosis who needs oxygen with activity but not while at rest
Stage 3: A person with idiopathic pulmonary fibrosis who needs oxygen 24 hours a day, with activity, at rest, and during sleep
Stage 4: A person with idiopathic pulmonary fibrosis who needs advanced oxygen treatment, such as high-flow oxygen, or has progressed to the state of which a portable delivery system is inadequate for their oxygen needs
Learn More: What Are the Types of Fibrosis?
Idiopathic Pulmonary Fibrosis Symptoms
Idiopathic pulmonary fibrosis symptoms can vary by individual and can include the following:
Tachypnea (rapid, shallow breathing)
Dyspnea (shortness of breath) that begins during exercise and worsens to occur even at rest
Chronic dry cough that lasts longer than eight weeks and worsens to repeated, uncontrollable coughing episodes
Unintended weight loss
A general feeling of being unwell
Clubbed fingers and nails (widening and rounding of soft tissue around your fingernails and toenails)
Learn More: Pulmonary Fibrosis vs. Cystic Fibrosis: What Are the Differences?
Causes
While the cause is unknown, idiopathic pulmonary fibrosis often occurs in people who have one of the following comorbidities (simultaneous disease):
Ischemic heart disease
Having one or more of the following factors can increase your chances of developing idiopathic pulmonary fibrosis with or without another disease present:
Male sex
Being in your 60s or 70s
Smoking
A first-degree relative, such as a parent or sibling, with idiopathic pulmonary fibrosis
Exposure to hazardous materials such as asbestos or silica
Learn More: Causes and Risk Factors of Pulmonary Fibrosis
Diagnosis
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:
Chest X-ray: Uses small amounts of electromagnetic radiation to create images of your lungs and other internal organs
Computed tomography (CT) scan: Uses multiple X-rays to create a three-dimensional (3D) image of your lungs
Bronchoscopy
A bronchoscopy uses a bronchoscope (a type of endoscope made 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 your trachea (windpipe), and into your lungs to collect a small sample of lung tissue or fluid for analysis.
Bronchoalveolar Lavage
A bronchoalveolar lavage removes 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:
Spirometry: Measures how much air you can breathe in and out of your lungs and how fast and easily you can exhale out
Lung diffusion test: Measures how well carbon dioxide and oxygen are diffused, or transferred, between your lungs and blood
Lung plethysmography test: Determines how much air your lungs can hold
Blood Tests
Blood tests can detect signs of an autoimmune disease or environmental factors contributing to your breathing problems.
Lung Biopsy
A lung 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 via fine needle aspiration or as an open biopsy using an incision.
Treatment
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:
Esbriet (pirfenidone)
Ofev (nintedanib)
Lifestyle Changes
Lifestyle changes can involve a wide range of approaches for managing symptoms. These can include the following:
Quit smoking.
Avoid situations or environments where breathing is more difficult due to poor air quality, pollution, or dust.
Remain active with regular physical activity to maintain your strength and lung function.
Address anxiety and depression.
Eat a heart-healthy diet.
Oxygen Therapy
Oxygen therapy is administering supplemental oxygen to increase oxygen levels to healthy, normal levels. This can help you achieve the following:
Reduce shortness of breath
Reduce fatigue
Achieve better sleep
Remain more active
Pulmonary Rehabilitation
Pulmonary rehabilitation is a multifaceted type of lung therapy. It can include exercises and education to help you achieve the following:
Strengthen breathing
Eat and drink safely
Make your lung muscles less susceptible to fatigue
Mental health counseling for coping with the disease
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.
Learn More: Treatment Options for Idiopathic Pulmonary Fibrosis
Prognosis
Idiopathic pulmonary fibrosis is a rare disease that varies significantly from person to person. This can make a prognosis hard to determine. While some people may rapidly decline in health and quality of life, others experience periods of relative calm followed by acute deterioration.
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.
Coping
Everyone copes with chronic illness differently. Many people benefit from palliative 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:
Participation in an in-person or online support group in which you can discuss your situation with others who have similar first-hand experiences
Education through healthcare professionals or organizations dedicated to the treatment of pulmonary diseases
Following sources such as blogs, podcasts, and social media groups that address the challenges of coping with chronic lung 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.
