Pulmonary Fibrosis

Pulmonary Fibrosis, often referred to as IPF or UIP, is a specific type of interstitial lung disease (ILD).  This is an uncommon lung disease that usually affects adults over 50 years old.  Symptoms are shortness of breath and cough.  As the disease progresses, fatigue and leg swelling can become important symptoms. 

Pulmonary Fibrosis is diagnosed in one of two ways.  Some patients can be diagnosed without a lung biopsy just on the basis of a characteristic CT scan of the chest.  Other patients will require a lung biopsy.  Making a specific diagnosis is important as pulmonary fibrosis is a particularly difficult disease to treat.  There are no currently approved therapies that improve survival.  A small percentage of patients will respond to very strong medicines that suppress the immune system.  When appropriate, lung transplantation is an important consideration.

Pulmonary hypertension may complicate pulmonary fibrosis.  Your doctors will be looking for this as your treatment may be adjusted.  If your oxygen is low, you will be prescribed oxygen to use.  Annual influenza vaccinations and pneumonia vaccinations every five years are generally encouraged. 

After decades of research we finally have medications available that slow the progression and improve survival for patients with IPF.  At Arizona Pulmonary Specialists, we are proud to have participated in the research studies that brought the two new drugs to market. 

The lung doctors at Arizona Pulmonary Specialists are experts in the diagnosis and treatment of pulmonary fibrosis.   Our team of doctors, nurses and respiratory therapists will work with you to ensure you understand your medications and other treatments. 

At Arizona Pulmonary Specialists, we are actively involved in many research studies.  We often have studies of new medications for pulmonary fibrosis.  Ask your doctor if you are interested.

To learn more about pulmonary fibrosis, please visit WWW.PulmonaryFibrosisMD.com