Idiopathic pulmonary fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is scarring of the scaffolding framework of the lung where the air sacs are attached leading to inability of the lung to function properly. The cause of IPF is unknown but 10% of the patients affected with this condition have family members that are similarly affected suggesting a genetic susceptibility to this condition.

PATH NETWORK: IPF

How is Idiopathic Pulmonary Fibrosis Diagnosed?
The diagnosis of pulmonary fibrosis usually requires a consultation with a lung specialist. In patients who are complaining of unremitting shortness of breath for more than 3 months, an abnormal breathing test, coupled with a typical scarring pattern on high resolution computed tomography (CT scan) of the chest are usually enough to confirm the diagnosis of pulmonary fibrosis. Because there are many other causes of pulmonary fibrosis, additional testing including lung biopsy may be necessary in some patients.

How is Idiopathic Pulmonary Fibrosis Treated?
Currently, there are two medications approved for the treatment of IPF: Esbriet (Pirfenidone) and OFEV (Nintedanib). These drugs slow down the progression of IPF, but they are not a cure. Additional research studies to find a cure for IPF are ongoing. In patients with moderate and advanced disease, supplemental oxygen and pulmonary rehabilitation are helpful in relieving breathlessness and maintaining physical activity. Lung transplantation is a proven therapy in some patients with IPF. Early referral to a lung transplant center for an evaluation is recommended.

Support – Where can patients find info about support groups or local resources?
Johns Hopkins Medicine: Nadine Rattray, 410-955-4176, ILD Program Coordinator
UPMC: The Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease at UPMC
Penn State Milton S. Hershey Medical Center: Call Anne Dimmock (717) 531-0003 X 284024
Temple Health: Call Michelle Vega, CRNP – (215) 707-9574
Every Breath Counts: This documentary takes an up close look at IPF.

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