Make an Appointment
Talk to DoctorOverview
Pulmonary fibrosis is a progressive lung disease characterized by the scarring and thickening of lung tissue, including the alveoli (air sacs) and connective tissue. This condition deteriorates over time, causing increasing shortness of breath. While some individuals may remain stable for extended periods, others may experience a rapid decline in lung function.
The exact causes of pulmonary fibrosis are often unclear, and when no specific cause is identified, the condition is referred to as idiopathic pulmonary fibrosis. This form of the disease typically affects middle-aged and older adults, although it is rare in children and infants.
Unfortunately, the lung damage from pulmonary fibrosis is irreversible. Treatment options, such as medications and therapies, are aimed at slowing the progression of the disease, alleviating symptoms, and enhancing the quality of life. In certain cases, a lung transplant may be considered.
Symptoms
Pulmonary fibrosis manifests differently in each individual, and its symptoms can often resemble those of common, more readily treatable ailments such as the common cold or an upper respiratory infection.
The symptoms of pulmonary fibrosis may include:
- Rapid, shallow breathing.
- Muscle and joint aches.
- Persistent dry cough.
- Severe fatigue, regardless of the amount of rest.
- Shortness of breath, especially during or soon after physical activity.
- Unexplained and unintended weight loss.
As the condition progresses, additional symptoms may develop, including:
- Clubbing, characterized by the widening and rounding of the tips of the fingers or toes.
- Cyanosis, seen as a bluish hue to the skin in fair-skinned people or a gray or white discoloration around the mouth or eyes in darker-skinned individuals, resulting from low oxygen levels in the blood.
Causes
Pulmonary fibrosis results from lung damage caused by various factors such as radiation therapy, certain medications, prolonged exposure to specific toxins, and certain medical conditions. These changes impede the blood’s ability to absorb oxygen. In some cases, the origin of pulmonary fibrosis may be unknown.
- Work and environment: Pulmonary fibrosis may be caused entirely or in part by your place of work and residence. Wearing protective gear is especially important if you have frequent or continuous contact with toxins or pollutants, which are substances that degrade the quality of water, air, or land. Among the examples are:
- Asbestos fibers.
- Bird and animal poop.
- Dust from wood, coal or grain.
- Metal dusts.
- Mold.
- Silica dust.
- Radiation treatments: When receiving radiation therapy to the chest for conditions like lung or breast cancer, some patients experience lung damage months or even years following the treatment. The following will determine the severity of the damage:
- Administered chemotherapy.
- Duration of exposure to radiation.
- Overall radiation dose that was provided.
- Underlying lung disease.
- Medicines: Many medications might harm the lungs. Among the examples are:
- Chemotherapy: Lung tissue can be damaged by medications meant to destroy cancer cells, such as bleomycin, cyclophosphamide, and methotrexate.
- Heart medicines: Lung tissue may be damaged by certain medications used to treat irregular heartbeats, such as amiodarone.
- Antibiotics: Damage to the lungs can result from antibiotics such nitrofurantoin or ethambutol.
- Anti-inflammatory medicines: Lung damage can result from taking some anti-inflammatory medications such sulfasalazine or rituximab.
- Medical conditions: There are also other disorders that can cause damage to the lungs, such as:
- Dermatomyositis: The skin rash and weakening of the muscles are indications of an inflammatory condition.
- Lupus: A condition brought on by the immune system of the body attacking its own tissues and organs.
- Mixed connective tissue disease: Exhibits a variety of symptoms common to several illnesses, including polymyositis, scleroderma, and lupus.
- Pneumonia: An infection that causes inflammation in one or both of the lungs’ air sacs.
- Polymyositis: A condition that is inflammatory and results in bilateral muscular weakening.
- Rheumatoid arthritis: A condition characterized by inflammation that impacts the body’s processes and joints.
- Sarcoidosis: An inflammatory condition that typically affects the lymph nodes and lungs.
- Scleroderma: A group of rare diseases that cause internal body issues in addition to skin tightness and hardening.
- Idiopathic pulmonary fibrosis: Pulmonary fibrosis can be caused by a wide range of medications and conditions. However, the cause is never identified in a great number of cases. Even if the etiology of the condition cannot be determined, risk factors like smoking or exposure to air pollution may be linked to it. Idiopathic pulmonary fibrosis is pulmonary fibrosis without a known etiology.
A common condition in many cases of idiopathic pulmonary fibrosis is gastroesophageal reflux disease, or GERD. When stomach acid refluxes back into the esophagus, it causes this disease. Idiopathic pulmonary fibrosis may be made worse more quickly by GERD, or it may be a risk factor for the condition. However, additional research is required.
Risk factors
The following risk factors that may increase your risk of pulmonary fibrosis includes:
- Age: Although it is uncommon, pulmonary fibrosis has been observed in newborns and children. The majority of those who get pulmonary fibrosis do so in their later years, typically between the ages of 50 and 70.
- Cancer treatments: Pulmonary fibrosis can be more likely to occur if you use certain chemotherapy medications or receive radiation treatments to the chest.
- Gender: More males than women are affected by pulmonary fibrosis. Still, the number of female cases has increased recently.
- Genetics: Genes may be involved in some forms of pulmonary fibrosis since they run in families.
- Occupation: If you are employed in construction, farming, or mining, your risk of acquiring pulmonary fibrosis is increased. Additionally, there is an increased risk if you come into constant or frequent contact with contaminants that are known to harm the lungs.
- Smoking: Compared to those who have never smoked, those who smoke currently or in the past are more likely to develop lung fibrosis. Moreover, those who have emphysema are more vulnerable.
Diagnosis
- Physical examination
- Initial assessment: Your healthcare provider will conduct a physical examination, review your medical and family history, and discuss your symptoms and medications.
- Environmental exposure: Questions about exposure to chemicals, dusts, fumes, or similar materials, especially at work.
- Listening to lungs: During the exam, the provider listens for a crackling sound at the base of the lungs, a common indicator of pulmonary fibrosis.
- Blood tests
- Organ function: Tests to assess the health of your kidneys and liver.
- Screening for conditions: Blood tests to rule out or screen for other medical conditions.
- Disease monitoring: Lab tests to monitor the progression of the condition over time.
- Imaging tests
- Chest X-ray: Helps identify scar tissue associated with pulmonary fibrosis.
- Computed tomography (CT) scan: A high-resolution CT scan provides detailed images to diagnose the extent of lung damage.
- Echocardiogram: Uses sound waves to create images and videos of the heart, measuring pressure in the arteries to the right side of the heart and lungs.
- Lung function tests
- Spirometry: Measures lung capacity and airflow.
- Lung volume test: Assesses the total volume of air the lungs can hold.
- Lung diffusion test: Evaluates the efficiency of gas exchange between the lungs and the bloodstream.
- Pulse oximetry: Monitors blood oxygen levels with a device placed on a finger.
- Exercise stress test: Assesses heart and lung function during physical activity on a stationary bike or treadmill.
- Arterial blood gas test: Analyzes oxygen and carbon dioxide levels in a blood sample from a wrist artery.
- Tissue sample
- Surgical biopsy:
- Video-assisted thoracoscopic surgery (VATS): A minimally invasive method using a tiny camera and surgical tools inserted through small incisions between the ribs.
- Thoracotomy: An open surgery that involves a larger incision to access the lungs.
- Bronchoscopy: A less invasive technique where tissue samples are taken using a bronchoscope inserted through the nose or mouth.
- Surgical biopsy:
These diagnostic procedures help not only in diagnosing pulmonary fibrosis but also in monitoring the disease’s progression and the effectiveness of treatment over time.
Treatment
The primary aim of most treatments for pulmonary fibrosis is to enhance quality of life and alleviate symptoms. Unfortunately, the lung thickening and scarring caused by pulmonary fibrosis are irreversible, and there is currently no known medication capable of halting its progression. However, certain treatments may temporarily alleviate symptoms or slow down the rate of progression, while others can improve quality of life. The approach to treating pulmonary fibrosis depends on its underlying cause, assessed severity, and is typically determined collaboratively by healthcare providers and patients.
- Medication: Your healthcare provider could recommend taking pirfenidone or nintedanib if you have idiopathic pulmonary fibrosis. Additionally, nitedanib is authorized for additional forms of rapidly worsening pulmonary fibrosis. These medications may help avoid episodes in which symptoms develop quickly and may also help decrease the progression of pulmonary fibrosis.
Nausea and diarrhea are among the adverse reactions of nitedanib that might occur. Nausea, lack of appetite, and sunburn are some of the side effects of pirfenidone. Your healthcare provider will utilize routine blood tests to monitor liver function while on either medication.
In cases when you exhibit symptoms of gastroesophageal reflux disease (GERD), healthcare providers may prescribe anti-acid medications. GERD is a digestive disorder that is frequently experienced by patients who have idiopathic pulmonary fibrosis.
- Oxygen therapy: Adding supplemental oxygen to your body allows easier breathing and exercise. It could also give you additional energy and power. This could also lessen any complications due to low blood oxygen level.
When you workout or go to sleep, you could utilize oxygen. However, certain people are always in need of oxygen. Being more mobile can be facilitated by using a portable oxygen concentrator or carrying a small oxygen tank.
An acute exacerbation is a quick worsening of symptoms. Patient may require greater oxygen support. You could require hospitalized mechanical ventilation in specific circumstances. During this procedure, a breathing assistance machine is connected to a tube that is inserted into the lungs. When symptoms suddenly worsen, your healthcare provider may prescribe antibiotics, corticosteroids, or other medications.
- Pulmonary rehabilitation: Pulmonary rehab helps you feel better and do more every day. It includes exercises to boost your strength, breathing techniques for better oxygen use, advice on healthy eating, emotional support, and learning about your condition.
- Lung transplant: A lung transplant replaces a healthy lung (or lungs) from a donor for one or both of the damaged lungs. It has the capacity to enhance your well-being and quality of living. Not everyone is a candidate for a lung transplant since it is a major procedure.
For certain patients with pulmonary fibrosis, a lung transplant may be a recommended. However, problems from lung transplants, such rejection and infection, can occur. You need to take medication for the remainder of your life after a lung transplant. If your healthcare provider determines that a lung transplant is the best course of action for treating your condition, you and your healthcare team may discuss about it.
