Idiopathic Pulmonary Fibrosis (IPF)

Overview
Diagnosis
Treatment

Idiopathic Pulmonary Fibrosis (IPF) Overview

Idiopathic pulmonary fibrosis (IPF) is a disease marked by progressive scarring of the lungs. The disease is called idiopathic because there is currently no known cause for IPF.

Fibrosis, or scar tissue, develops and prevents the lungs from working normally. This gradually interferes with a person’s ability to breathe and function.

IPF belongs to a family of more than 100 related diseases, called interstitial lung diseases (ILDs), which often have similar characteristics and can result in lung scarring.

Symptoms of idiopathic pulmonary fibrosis

IPF, as with other interstitial lung diseases, affects each person differently and at different rates.

Typical early symptoms of IPF include shortness of breath during periods of activity and a dry cough.
Other possible symptoms include weight loss and fatigue.
With later-stage IPF, enlargement or clubbing of the fingertips may develop.
As the disease progresses, shortness of breath may occur at rest, making even normal activities — such as walking, taking on the phone, and eating — difficult.

The University of Pittsburgh Simmons Center for Interstitial Lung Disease at UPMC, was created in 2001 because of a generous gift from the Simmons family. The center is dedicated to providing the highest quality of health care, education, and support for people with interstitial lung diseases and their caregivers and loved ones.

Diagnosing Idiopathic Pulmonary Fibrosis

To help confirm a diagnosis of IPF, UPMC specialists conduct several tests, including:

High resolution CT scans of the chest — to provide a more detailed picture of the lungs.
Breathing tests — to show how the lungs are working.
Oxygen saturation tests — to check oxygen levels at rest, with exertion, and during sleep.

Additional procedures may include:

Lab tests, including rheumatologic studies — to exclude other causes of lung scarring.
An echocardiogram — to assess any heart involvement. As a result of low oxygen levels, the heart may be stressed and cause pulmonary hypertension (high blood pressure in the lungs). A lung biopsy or video-assisted thoracic surgery — to find usual interstitial pneumonia, the tissue (histological) diagnosis for IPF.

Idiopathic Pulmonary Fibrosis Treatment

At the University of Pittsburgh Simmons Center for Interstitial Lung Disease at UPMC, a multidisciplinary team of experts offers people with IPF the full capabilities of UPMC.

Patients are invited for an evaluation and ongoing management of their diseases in conjunction with their local doctors. Researchers and clinicians collaborate to translate basic research on interstitial lung disease into new treatments that may save lives.

The idiopathic pulmonary fibrosis treatment team

The Simmons Center's multidisciplinary team includes:

Pulmonary physicians — who are experts in the management of IPF
Cardiologists — who evaluate the involvement of the heart and pulmonary vessels
Rheumatologists — who assess the immune system
Pathologists — who evaluate the biopsies
Chest radiology experts — who evaluate imaging results
Rehabilitation and quality-of-life experts — who optimize supportive therapy and assess the psychological impact of IPF
Transplant surgeons
Leaders in clinical research — who provide access to novel IPF therapies

The team will re-evaluate the diagnosis and assess treatment strategies once the diagnosis is confirmed.

Treatment options for idiopathic pulmonary fibrosis

At present, there are no approved drug therapies for IPF, but patients may benefit from:

Pulmonary rehabilitation
Oxygen therapy
Participating in clinical research
Monitoring for acute flare-ups
Joining an IPF support group
Referral to UPMC’s Lung Transplantation Program, if necessary

Lung transplantation is the only effective therapy to treat IPF and is considered in the management of most patients.

The UPMC Lung Transplantation Program is nationally renowned. Our experts have performed more than 1,400 lung transplants since the program’s inception.

Doctors at the Simmons Center work closely with members of the Lung Transplant Program to refer patients for evaluation, when the time is right.