Skip to main content

Big news from the 2008 American Thoracic Society meeting - Japanese share Pirfenidone results

This is a report on one of the sessions that took place at the American Thoracic Society meeting. The Japanese trial is actually somewhat ahead of the trials in the United States - both the IPF trial and the HPS trial. It's so great to hear that the drug appears to be having some effect. While at the booth a doctor almost came bouncing by to ask what we thought of Pirfenidone. "Do you think it works?" he asked. "I hope so!" I answered, "I've been part of the clinical trial for 20 months." He looked a little surprised. I don't think he was expecting to find a patient for some reason. Anyway, thought you all would be interested in the results. I sure was!

New treatment option for idiopathic pulmonary fibrosis

Published: Tuesday, 20-May-2008



Medical Research News
Patients with idiopathic pulmonary fibrosis (IPF) may have a new treatment option, according to researchers in Japan.


In a Phase III, double-blind, placebo-controlled clinical trial, the investigators discovered that a daily dose of pirfenidone could slow the progression of IPF, reducing the loss of lung capacity. The results will be announced at the
American Thoracic Society's 2008 International Conference in Toronto on Tuesday, May 20.

"The most common treatment for IPF is anti-inflammatory agents such as steroids," said lead researcher Takashi Ogura, M.D., of Kanagawa Cardiovascular and Respiratory Center in Yokohama, Japan. "However our study confirmed that pirfenidone, the main action of which is thought to be antifibrotic, achieved a therapeutic effect on IPF. I expect that our study will serve as a guide to develop a new therapy for IPF in the future."

The researchers recruited a total of 275 Japanese patients with mild to moderate IPF and randomized them to a high dose pirfenidone (1,800 mg/day) group, a low dose pirfenidone group (1,200 mg/day) and a placebo group. They measured lung capacity (vital capacity or VC) and progression-free survival, defined as a period without death or a greater than 10 percent decrease in VC, to determine the effectiveness of the regimens.

At the end of one year, they found that patients who had been randomized to the high dose regimen had significantly lower loss of VC than the placebo group. Furthermore, pirfenidone slowed the overall deterioration of IPF compared to the placebo.

"Taken altogether, our study demonstrated positive clinical effects of pirfenidone that suppresses the progress of IPF and potentially contributes to improving the outcomes of patients with IPF," said Dr. Ogura.

Pirfenidone represents new hope, not only for IPF patients who currently have no curative treatment options, but because it is thought to be an antifibrotic agent, it may be able to treat other fibrotic lung diseases, such as interstitial pneumonia with collagen vascular disease and extrapulmonary fibrosis.

"We will continue the follow-up of the patient cohort included in this study to identify whether pirfenidone can contribute to prolonged survival in patients with IPF," said Dr. Ogura. "Other clinical studies of pirfenidone are also being conducted in the U.S. and Europe, and we hope that our results will be replicated there."

http://www.thoracic.org/

Comments

Popular posts from this blog

Ratner's Cheesecake

Here's another recipe from Toby! Thanks Toby......and I'll get the others posted soon! Ratner's Cheesecake and plain cookies Ratner's was a Jewish dairy restaurant in the lower East Side of Manhattan. This recipe, from my disintegrating, no longer in print Ratner's cookbook, is the closest I've ever gotten to reproducing the rich, heavy cheesecake my mother made when I was a kid. It's worth the time it takes to prepare and every last calorie. Dough Can be prepared in advance. Makes enough for two cakes. Can be frozen or used to make cookies – see recipe below. 1 cup sugar 1 teaspoon vanilla extract 1 cup shortening 1 teaspoon lemon extract 1 cup butter 2 eggs 3 cups sifted cake flour ½ teaspoon salt 2 cups all purpose flour 1 teaspoon baking powder 1. In a bowl, combine all ingredients with hands. Refrigerate 3 -4 hours, or preferably overnight. Filling (for

The next generation with Hermansky-Pudlak Syndrome

I'm so behind on posting about the trip to Puerto Rico. Since the episode of Mystery Diagnosis on Hermansky-Pudlak Syndrome ran right after we got home, it's been a little busy. These, however, are my favorite pictures from Puerto Rico. I know, not pretty senery etc - but these little guys and gals inspire me. They are the next generation of folks with HPS, and if we keep up the hard work, they will live better lives because of it. They motivate me.

Help for one of our newest HPS friends

As many of you know, I have a google alert set up for all sorts of key terms like albinism, pulmonary fibrosis etc. The following was posted on the blog of a friend of Melanie's. Melanie is one of our newest members of the Hermansky-Pudlak Syndrome family. Unfortunately, like so many she didn't know she had HPS and the lung disease sort of snuck up on her. Melanie will be 26 years old next week. Perhaps some of us would like to contribute to the fund set up to help her and her family for her birthday. Here's the post: Melanie Hernandez, beloved sister and daughter to Pete, Monique, Cookie, and Bugie, has been diagnosed with Pulmonary Fibrosis, a lung disease occurring in people with Hermansky-Pudlak Syndrome, a very rare disease.Hermansky-Pudlak Syndrome (“HPS”) is a genetic metabolic disorder. It is characterized by: Albinism, Vision Impairment, Bleeding Disorder, Inflammatory Bowel Disease and Lung Disease – Pulmonary Fibrosis. With the family’s research and the recollect