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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.
IPF, as with other interstitial lung diseases, affects each person differently and at different rates.
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.
To help confirm a diagnosis of IPF, UPMC specialists conduct several tests, including:
Additional procedures may include:
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 Simmons Center's multidisciplinary team includes:
The team will re-evaluate the diagnosis and assess treatment strategies once the diagnosis is confirmed.
At present, there are no approved drug therapies for IPF, but patients may benefit from:
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.