Idiopathic Pulmonary Fibrosis

Signs and Symptoms

Pulmonary fibrosis (PF) is a condition that causes progressive scarring of the lungs. The cause of idiopathic pulmonary fibrosis (IPF) is unknown. IPF involves chronic injury to the lung's tissues, which then prompts an exaggerated healing response. As a result, over time, fibrous scar tissue builds up in the lungs, affecting the lung's ability to provide the body with necessary amounts of oxygen.

Although the cause of IPF is unknown, certain risk factors may increase a person's chance of developing the condition. These include occupational exposure to metal dust or wood dust, smoking cigarettes, viral infection and a family history of PF.

IPF affects 83,000 people in the United States with 15,000 new cases diagnosed each year. Typically, the disease is diagnosed in people between the ages of 50 and 70 and affects men more frequently than women.

Symptoms of IPF may occur gradually and include:

• Shortness of breath, particularly during or after physical activity
• Chronic dry hacking cough
• Fatigue
• Weight loss
• Build-up of tissue in the fingertips, a condition called clubbing

Over time, symptoms of IPF may worsen and lead to other complications, such as high blood pressure, heart attack, respiratory failure, stroke, blood clot in the lungs, lung infection and lung cancer.

Diagnosis

In making a diagnosis of idiopathic pulmonary fibrosis (IPF), your doctor will first start by conducting a thorough physical examination. This will include closely listening to your chest with a stethoscope to check for any abnormal sounds while you breathe. Your doctor will also ask about any symptoms you are experiencing, including when they started and how they've progressed or eased over time. Your full medical history will also be recorded.

Symptoms of IPF may mimic those of other lung diseases. Therefore, the condition may be difficult to diagnosis and may require significant time and testing to make an accurate diagnosis. Several visits with your doctor may be needed to finalize your diagnosis and treatment approach.

Diagnosing IPF is often a process of ruling out other conditions. The following tests may be conducted to help make an accurate diagnosis.

• Pulmonary Function Testing (PFT) -- This test involves a series of breathing maneuvers that measure the airflow and volume of air in your lungs. This allows your doctor to objectively assess the function of your lungs. PFT is recommended for all patients.
• High Resolution Computed Tomography (HRCT) -- This is a special type of CT scan that provides your doctor with high-resolution images of your lungs. These images are extremely valuable in determining which type of ILD you have. Having a HRCT is no different than having a regular CT scan; they both are performed on an open-air table and take only a few minutes. HRCT is recommended for all patients.
• Six Minute Walk Testing -- This test is a formal evaluation of the distance you can walk and the oxygen saturations (measured by finger or ear probe) you achieve while walking. This is an important marker of functional status used by your doctor in managing your condition. The six minute walking test is recommended for all patients.
• Bronchoscopy -- This test involves the passage of a flexible fiberoptic scope about the diameter of a pencil into the lungs to obtain fluid and tissue samples to aid in diagnosis. This test performed by your doctor is an outpatient procedure that does not require an overnight stay in the hospital. Bronchoscopy is recommended for some patients.
• Surgical Lung Biopsy -- This test is required for some patients to definitively diagnose the cause of their lung disease. Surgical lung biopsy is usually performed with small tools and cameras through three to four inch long incisions. A surgical lung biopsy is performed by a cardiothoracic surgeon and requires a few days stay in the hospital.

Treatment

Idiopathic pulmonary fibrosis (IPF) is treated at the Interstitial Lung Disease Center of Excellence at UCSF. World-renowned lung specialists, chest radiologists, pathologists and in some cases, lung transplant specialists, work together as a team to design the most effect treatment plan for your condition.

The exact cause of IPF is unknown at this time. Therefore, there is no specific, curative treatment currently available.

However, medications are available that reduce symptoms and slow disease progression. These include corticosteroids, usually prednisone, taken in combination with drugs to suppress the bodys immune response, such as azathioprine and cyclophosphamide. It is important to note that these medications are only effective in a small number of people, typically those whose disease is not advanced.

A lung transplant may be an effective treatment option for some patients. Our lung transplant staff has performed more than 100 transplants since the program started in 1991, with an average one-year survival rate of 80 percent compared to a national average one-year survival rate of 70 percent. Our team is dedicated to providing the most comprehensive medical and surgical care found anywhere. The support group, social work and psychological services are committed to supporting the patients and their families during the entire transplant process from the initial evaluation through the transplant surgery and thereafter.