Pulmonary fibrosis treatment

Treatment for pulmonary fibrosis (PF) aims to slow disease progression, ease symptoms, and improve quality of life. It may include medications, oxygen therapy, pulmonary rehabilitation, and, in severe cases, a lung transplant.

PF is a type of interstitial lung disease (ILD), a group of more than 200 conditions characterized by damage and inflammation in the tissue surrounding the lungs’ air sacs. In PF specifically, the accumulation of scar tissue (fibrosis) stiffens the lungs, making it harder for oxygen to pass into the bloodstream.

While PF generally worsens over time, appropriate monitoring and treatment can help slow its progression and make living with PF easier.

However, there is no one-size-fits-all approach to PF treatment. A person’s care team will decide on the right treatment depending on a number of factors, including the disease’s underlying cause, how quickly it is progressing, the person’s symptoms, and their overall health.

Goals of pulmonary fibrosis treatment

There is no cure for PF. Instead, treatment aims to slow PF disease progression, support daily activities, and improve overall quality of life.

Specifically, the main goals of treatment for PF include:

• Slowing lung function decline
• Easing symptoms such as shortness of breath, cough, and fatigue
• Preventing complications, such as lung infections or acute exacerbations
• Maintaining physical function and quality of life
• Supporting emotional well-being and everyday activities

The right treatment, started early and adjusted as needs change, may help preserve lung function and, in some cases, prolong survival. After diagnosis, patients should work with a multidisciplinary care team to develop a personalized PF care plan that fits their needs and preferences.

Medications

The medications used to treat PF depend on the disease type and underlying cause. Some medicines slow the loss of lung function, while others target inflammation or an underlying condition. Doctors may also recommend medications to address specific PF symptoms.

Antifibrotic therapy

Antifibrotic medications cannot get rid of existing scar tissue or cure the disease, but they may help slow the progression of certain forms of PF and improve long-term outcomes.

In the U.S., three antifibrotic medications are approved to treat idiopathic pulmonary fibrosis (IPF), the most common form, in which an underlying cause cannot be identified:

• Esbriet (pirfenidone), with generics available
• Jascayd (nerandomilast)
• Ofev (nintedanib), with generics available

Some of these antifibrotic therapies can also be used to treat other types of PF if the disease has been progressing. Ofev is also indicated to slow lung function decline people with scleroderma-associated ILD.

Other medications

For certain PF types, particularly those associated with an autoimmune condition or another immune system problem, medications that suppress or modify the immune response may also be central to the treatment plan. These can include:

• Actemra (tocilizumab), approved for adults with scleroderma-associated ILD
• Azathioprine (sold as Imuran and others)
• Corticosteroids such as prednisone
• Mycophenolate mofetil (sold as CellCept and others)
• Rituximab

These medicines are not appropriate for every type of PF. The choice depends on the underlying condition, potential benefits and risks, and other individual factors. A multidisciplinary team including a lung specialist and an expert in autoimmune or immune system conditions will work together with patients to decide the best treatment plan.

Oxygen therapy

PF can make it harder for oxygen to move from the lungs into the bloodstream. As the disease advances, many people require supplemental oxygen therapy to ease breathing and prevent serious or life-threatening complications. Some individuals need oxygen only at night or during exercise, while others with more advanced disease may need it all day long.

The need for oxygen is determined by certain tests that measure the amount of oxygen in the blood during rest, exercise, and sometimes at night. When levels drop to 88% or lower, a healthcare provider may order supplemental oxygen.

Oxygen therapy for PF can be delivered through a tube placed in the nose or, in some cases, a mask. Various oxygen delivery systems are available, each suited to a person’s lifestyle and needs. Some of these are portable, making it easier for people to go about their daily activities.

Pulmonary rehabilitation

Pulmonary rehabilitation is an education and exercise program that helps individuals with lung disease learn how to breathe more easily and be more active. It can help ease symptoms and improve quality of life.

The supervised program is led by a multidisciplinary team of healthcare professionals who create a program tailored to each person’s needs. Pulmonary rehabilitation is considered most effective and safe when delivered in a hospital or clinic, but some forms of rehabilitation can be offered at home or online.

Depending on the situation, pulmonary rehabilitation for PF may involve:

• Education about lung function and symptom management
• Supervised exercises and strength training
• Breathing techniques
• Nutritional guidance
• Emotional support or counseling

A healthcare professional can help determine whether pulmonary rehabilitation is appropriate and which program best suits a person’s abilities and needs.

Managing symptoms

In addition to PF medications and other treatments,  a healthcare team may recommend a variety of practical strategies and lifestyle adjustments to help manage specific PF symptoms and make daily life more manageable, depending on disease severity:

• Managing shortness of breath: Breathing exercises may help make the lungs more efficient and activity more manageable.
• Managing cough: Doctors may recommend cough-suppressing medicines or assess whether another problem, such as acid reflux, is contributing to the cough. Some people may also benefit from speech therapy to learn how to talk without triggering a cough.
• Managing fatigue: Pacing activities, taking regular breaks, and prioritizing essential tasks can help conserve energy. Asking for help with everyday activities such as cleaning or cooking may also help with energy management.
• Staying active: Appropriate physical activity can help maintain strength and function, while improving energy and sleep. A healthcare professional can advise on safe activity levels.
• Supporting general health: Eating a balanced diet, staying hydrated, avoiding smoking, and keeping up with recommended vaccinations may support overall well-being.

These interventions are often encompassed under palliative care, a branch of medicine focused on easing symptoms and improving quality of life for people with serious diseases.

Lung transplant

For some people with advanced PF, a lung transplant may be an option when other treatments are no longer enough to manage the disease. A transplant can significantly improve breathing and quality of life for eligible patients, but it is a major surgery with significant risks so it is not suitable for everyone.

While a lung transplant for PF is performed at advanced disease stages, patients should be evaluated by a team of transplant specialists soon after diagnosis, as assessing eligibility and waiting for a donor organ can take time. A transplant team will consider several factors to determine transplant eligibility, including whether a patient is generally healthy enough to undergo the procedure and if they have an adequate support system in place.

Once deemed eligible for the chronic lung scarring treatment, patients will be placed on a transplant list to wait for a donor. After a transplant, patients must will need lifelong medical follow-up and medicines to reduce the risk of organ rejection.

Ongoing care and follow-up

Treatment needs for a person living with PF may change over time as the disease progresses. Patients should adhere to regular follow-up with their care team, and promptly report any changes in their symptoms, so doctors can determine whether the current care plan remains appropriate.

Routine PF monitoring may include:

• Pulmonary function tests, which measure lung function and help doctors track changes over time.
• Oxygen checks, which assess whether the body is getting enough oxygen, including during activity.
• Imaging scans, which may help doctors assess changes in the lungs over time.
• Symptom reviews, in which doctors discuss changes in breathing, cough, fatigue, and the ability to carry out daily activities.

Sometimes, patients may experience a significant worsening of symptoms over days to weeks, known as an acute exacerbation. This can be life-threatening, so people should seek urgent medical attention if they experience:

• Sudden or severe shortness of breath and fatigue
• New or worsening chest pain
• Bluish lips or fingernails, which may indicate dangerously low oxygen levels
• New confusion, dizziness, or fainting

Exacerbations are often triggered by respiratory infections. For that reason, it is important for people with PF to receive all recommended vaccinations and practice good hygiene to help prevent infections.

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Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.