Pulmonary Fibrosis Care at Arizona Pulmonary Specialists

What is Pulmonary Fibrosis?

Pulmonary fibrosis (PF) is a type of interstitial lung disease (ILD) characterized by scarring (fibrosis) of the lung tissue. As the scarring worsens, the lungs become less flexible and less efficient at delivering oxygen to the bloodstream.

There are several types of pulmonary fibrosis, including:

• Idiopathic Pulmonary Fibrosis (IPF) – The most common form, with no known cause
• Usual Interstitial Pneumonia (UIP) – A specific lung pattern often associated with IPF
• Secondary Pulmonary Fibrosis – Caused by conditions such as autoimmune diseases, chronic infections, or exposure to harmful environmental factors, such as asbestos, silica, or certain medications

While pulmonary fibrosis primarily affects adults over 50, it can occur at any age. Early diagnosis is key to slowing progression and managing symptoms effectively.

Symptoms of Pulmonary Fibrosis

Pulmonary fibrosis symptoms often develop gradually and worsen over time. Early recognition of symptoms is crucial for prompt diagnosis and treatment. Common symptoms include:

• Shortness of breath (dyspnea) – Initially with exertion, later even at rest
• Chronic dry cough – Persistent and non-productive
• Fatigue – Feeling constantly tired despite rest
• Leg or ankle swelling – A sign of potential complications such as pulmonary hypertension
• Unintended weight loss – Due to increased energy demands from breathing difficulty
• Clubbing of fingers – Enlargement of the fingertips caused by low oxygen levels

Because symptoms overlap with other lung diseases such as COPD or asthma, specialized testing is needed for an accurate diagnosis.

Diagnosing Pulmonary Fibrosis

At Arizona Pulmonary Specialists, we use state-of-the-art diagnostic tools to confirm pulmonary fibrosis and determine its severity. An accurate diagnosis is critical for selecting the most effective treatment strategy. Our diagnostic tools include:

• High-resolution CT (HRCT) scan – The most important test for identifying lung scarring patterns
• Pulmonary function tests (PFTs) – Measure lung capacity and oxygen exchange efficiency
• Oxygen saturation testing – Determines if supplemental oxygen is needed
• Blood tests – To rule out autoimmune diseases or infections that can mimic PF
• Lung biopsy – In some cases, a surgical or minimally invasive biopsy may be required for a definitive diagnosis

Treatment of Pulmonary Fibrosis

While pulmonary fibrosis has no cure, there are FDA-approved treatments that can help slow disease progression and improve quality of life. Treatment options include:

• Antifibrotic medications – Newer drugs such as pirfenidone (Esbriet) and nintedanib (Ofev) can help slow lung scarring
• Oxygen therapy – Helps reduce breathlessness and improves energy levels
• Pulmonary rehabilitation – A specialized exercise and education program to strengthen respiratory muscles
• Lung transplant evaluation – For eligible patients with advanced disease

Since pulmonary hypertension can complicate pulmonary fibrosis, our doctors carefully monitor and adjust treatment as needed. We also recommend:

• Annual flu vaccinations
• Pneumonia vaccines every five years
• Early intervention for respiratory infections

Clinical Research and Trials

At Arizona Pulmonary Specialists, we are committed to advancing pulmonary fibrosis treatment through clinical research. We have participated in key studies that led to the approval of new antifibrotic medications, and we continue to offer access to clinical trials, giving patients the opportunity to try emerging therapies before they become widely available. If you’re interested in participating in research studies for pulmonary fibrosis, ask your doctor about current clinical trial opportunities.

Why Choose Arizona Pulmonary Specialists?

• Pulmonary fibrosis experts – Specialized physicians with experience in rare lung diseases
• Advanced testing and treatment – State-of-the-art assessments and tailored therapies
• Access to clinical trials – Opportunities to access the latest treatments
• Coordinated hospital and outpatient care – Seamless management whether you need office visits or hospitalization

Physicians Specializing in Pulmonary Fibrosis

Our team of pulmonologists, nurses, and respiratory therapists is dedicated to providing expert, compassionate care for pulmonary fibrosis patients.