Fibrosis File

Pulmonary Fibrosis News brings us last year’s big stories in the world of Pulmonary Fibrosis. Here is the countdown!

Esbriet Found to Reduce Idiopathic Pulmonary Fibrosis Progression across Disease Stages

An Italian study found that people with idiopathic pulmonary fibrosis (IPF) might benefit from Esbriet (pirfenidone) treatment, regardless of their disease stage and baseline lung function. Current treatment options for IPF, such as Esbriet or OFEV®/Vargatef (nintedanib), may delay disease progression but will not cure it. The study suggests that treatment early in the disease may be the best strategy to preserving functional status and extending a person’s life.

Study Shows Statins Can Improve IPF Patient Outcomes

A study found that statins provide beneficial effects for those with IPF, includ- ing decreased death risk. Statins are a class of drugs used to lower cholesterol levels in the blood to help prevent heart attacks and stroke. IPF is associated with significant cardiovascular conditions.

Can an Immunotherapy Based on Smallpox Vaccine Reverse Lung Fibrosis?

Surprising results from Johns Hopkins School of Medicine showed that the small- pox vaccine may reverse established lung fibrosis in mice, as the vaccine changed the reactivity of various types of immune cells. Immunotherapy is a treatment that uses your body’s own immune system to help fight disease. These findings may be critical to developing new immunotherapies for lung fibrosis.

Common Diabetes Medication Shows Promise in Treating Lung Fibrosis

A study suggested that metformin, a drug used to treat diabetes and which has proven effective and safe, could benefit people with IPF by working to diminish lung fibrosis.

Physical Activity Crucial for Those with Idiopathic Pulmonary Fibrosis

An Israeli study suggested that phys-ical inactivity and low blood-oxygen levels during periods of exercise predict poor survival in those with IPF, underscoring the importance of assessing physical activity and oxygen desaturation in identifying people at high-risk. The study links even low levels of exercise to an increased risk of death, demonstrating that interventions to increase the amount of exercise, as well as lung rehabilitation programs, might change the course of the disease.

Country Music Star Joe Nichols Shares His Dad’s IPF Story in ‘Breath- less,’ a Public Awareness Campaign

Joe Nichols, a country music artist, part- nered with Boehringer Ingelheim to raise awareness about IPF after his father passed away from the condition at age 46. Nich- ols took part in the famous ‘Breathless’ campaign, and talked about how IPF has impacted his and his family’s lives.

Anti-Fibrotic Oral Treatment for IPF Showing Early Promise in Phase 2 Study

A Phase 2 clinical trial led by ProMetic Life Sciences showed that treatment with PBI-4050 might be safe and effective for people with IPF. Preliminary results found that PBI-4050 was safe and well-tolerated, with evidence of clinical effectiveness either alone or in combination with other IPF drugs, like OFEV and Esbriet.

Non-Invasive Diagnostic Test Now Available

Veracyte announced the launch of its non-invasive test to improve the diagnosis of interstitial lung diseases. Envisia Genomic Classifier should reportedly offer a fast and more accurate diagnosis than is currently available without the need for a lung biopsy.

IPF Drug in Phase 2 Study Reduced Lung Fibrosis, Improved Function in 35 Percent of Participants

FibroGen disclosed results from a Phase 2 clinical trial evaluating the effectiveness of the company’s investigational drug FG-3019 in the treatment of IPF. Study data indicated that the antibody was capable of reducing lung fibrosis in a portion of those enrolled.

IPF Review Study Focuses on Effectiveness of OFEV®

OFEV was praised as one of the two new IPF drugs that revolutionized IPF treatment. For information and support on OFEV, you may visit

Kim Fredrickson who copes with having Pulmonary Fibrosis (PF), writes a blog at the web site

When you feel stressed and over- whelmed with PF, she recently recom- mended learning to calm and soothe yourself. The answers to these ques- tions might help!

  • What are some things I’ve done in the past that bring me comfort?
  • What  type  of  soothing  words  do
  • I need to hear from myself right now?
  • What kind of  music  brings  me comfort? Could I make a playlist that is ready to go when I need it? If not, who could I ask to create a playlist for me, so I can listen to it on my smartphone?
  • Which Bible verses are soothing to my soul? Do I have the strength to mark them in my Bible, make a list I can access on my phone, or print out to have by my bedside? If not, is there a friend who might do this for me?
  • Would it help to journal, pray, cry or take a nap?
  • Would  it  help  to  call  a  friend?  Is there a friend who would be will- ing to just listen and be compas- sionate if I told them that’s what I need right now?
  • What helps to reduce my fears and comfort my heart?
  • Would  it  help  to  get  a  massage, enjoy my favorite drink, or watch my favorite show?