Idiopathic pulmonary fibrosis (IPF)

What is Idiopathic Pulmonary Fibrosis?

The term idiopathic means of causes unknown. Pulmonary fibrosis refers scarring and production of fibrotic tissue in the lungs. The disease also sometimes goes by the name Usual interstitial pneumonitis (UIP). There is no currently known cause of IPF, nor are there any known treatments. The disease is progressive and it has no medical therapies which have been shown to slow its advancement in clinical trials. It is one of two lung disease processes that transplant has been shown to prolong the length of a patient’s life, the other being Cystic Fibrosis.

These realizations have led to new recommendations that patients diagnosed with the disease be immediately referred for clinical trials and transplant consideration.

 

How to diagnose IPF/UIP:

IPF/UIP does NOT require open lung biopsy for diagnosis in the majority of cases. The operation, in the face of classic radiographic and clinical findings, only delays referral and can worsen the patient’s clinical status. The thoracotomy can also complicate future lung transplant surgeries.

 

When to refer for Transplant:

Timing for referral is critical. Once a patient’s total lung capacity is less than 70% in a patient who requires oxygen at rest or with ambulation, 2 year survival is 50% or less. Additionally, as the restrictive process continues the size of the donor lung has to be reduced as well. In patients who are small in stature the chances of acquiring lungs 2 standards deviations below the national average is significantly less likely. Even though the new lung allocation score favors patients with UIP, new statistics reveal that those patients with high lung allocation scores (>46) do not have post-transplant survival rates as those who have lower scores. For optimal outcomes, prompt referral for transplant for appropriate candidates should be made at the time of diagnosis.

A brief presentation is available below to help answer questions.