5-μm lung sections were cut from lung biopsies obtained from a patient with IPF. The section reveals marked inflammatory foci within the parenchyma (arrowheads), surrounded by dense collagen deposits (arrows)
As promised, here is the information that I received from the Coalition for Pulmonary Fibrosis regarding the guidelines about treatment decisions for people diagnosed with Idiopathic Pulmonary Fibrosis (IPF).
Also below is the article from the New England Journal of Medicine regarding the genetic connection of IPF. It is a beginning to understanding the cause of the disease and possible treatment in the future.
1. Here is a copy of an e-mail I received from the wonderful people at CPF:
| The Coalition for Pulmonary Fibrosis (CPF) would like to inform you about the first evidence-based guidelines on PF that were recently published. These guidelines will help your physician(s) help you and will provide the guidance they need to make recommendations for treatment decisions with you. Also, the CPF worked with the American Thoracic Society to create a new patient information sheet on PF (linked below), as well. |
| The guide and information sheet can assist you in understanding the options available to you as well as help you make the most of your visits with your doctor by being as fully informed as possible. |
Please see the CPF's full press release regarding the guidelines below.
Sincerely,
Mishka Michon, CPF CEO |
Pulmonary Fibrosis Applauds First Evidence-based IPF Clinical Guidelines
Guidelines Update 11-Year-Old Consensus Statement for Deadly Lung Disease
The Coalition for Pulmonary Fibrosis (CPF) applauds the joint statement issued by the American Thoracic Society for the first evidence-based clinical guidelines for the treatment of idiopathic pulmonary fibrosis (IPF). The guidelines represent progress in understanding of the disease and a milestone for patients. The devastating and deadly disease also called simply “Pulmonary Fibrosis” or “PF” has no FDA approved treatment and no cure.
The new guidelines replace the ATS consensus statement on PF issued in 2000 and reflect the progress in the disease that has changed medical understanding of the complex disease.
“We are pleased to see the new guidelines and hope it will not only help physicians to provide better care to PF patients, but will help them to empower their patients and family members to take an active role in their treatment decisions,” said Mishka Michon, CEO of the Coalition for Pulmonary Fibrosis.
The statement reviews current knowledge in the epidemiology, etiology, diagnosis and management of IPF, as well as available treatment options, including pharmacologic and non-pharmacologic therapies and palliative care. It appears in the March 15, 2011, American Journal of Respiratory and Critical Care Medicine.
“In the decade since the publication of the previous statement on IPF, studies have used the criteria for the diagnosis of IPF and recommendations published in the previous consensus-based statement to further our understanding of the clinical manifestations and course of IPF, and there has been an increasing body of evidence pertinent to its clinical management,” said Ganesh Raghu, MD, director of the Interstitial Lung Disease/Sarcoid/Pulmonary Fibrosis Program at the University of Washington Medical Center in Seattle and chair of the collaborative committee that drafted the statement. “The accumulated data and observations made in these studies are substantial and have allowed us to provide new guidelines for the diagnosis and management of IPF to the global pulmonary community-at-large to the patients and to all concerned based on evidence for the very first time.”
The new guidelines represent a collaborative effort between the ATS, the European Respiratory Society (ERS), the Japanese Respiratory Society (JRS) and the Latin American Thoracic Association (ALAT).
The recommendations in the guidelines are ranked from weak to strong to help the clinician understand the strength of the recommendations made in managing patients with IPF.
“For example, in the case of a weak recommendation clinicians are especially required to spend adequate time with patients to discuss patients’ values and preferences. This may lead a significant proportion of patients to choose an alternative approach,” Dr. Raghu, who also serves on the CPF’s scientific advisory board, said.
To assist clinicians in their interactions with IPF patients and their families, the ATS has developed a companion patient information series piece on IPF that is also published in the March 15 journal. This education piece is also posted on the ATS Web site (www.thoracic.org), as well as the Web site of the Coalition for Pulmonary Fibrosis (www.coalitionforpf.org), an ATS Public Advisory Roundtable member organization, whose focus is on research, advocacy, support and education for the pulmonary fibrosis community.
About Pulmonary Fibrosis (PF)
Pulmonary Fibrosis (PF) is a lung disorder characterized by a progressive scarring – known as fibrosis -- and deterioration of the lungs, which slowly robs its victims of their ability to breathe. Approximately 128,000 Americans suffer from PF, and there is currently no known cause or cure. An estimated 48,000 new cases are diagnosed each year. PF is difficult to diagnose and an estimated two-thirds of patients die within five years of diagnosis. Sometimes PF can be linked to a particular cause, such as certain environmental exposures, chemotherapy or radiation therapy, residual infection, or autoimmune diseases such as scleroderma or rheumatoid arthritis. However, in many instances, no known cause can be established. When this is the case, it is called idiopathic pulmonary fibrosis (IPF). |
About the CPF
The CPF is a 501©(3) nonprofit organization, founded in 2001 to accelerate research efforts leading to a cure for pulmonary fibrosis (PF), while educating, supporting, and advocating for the community of patients, families, and medical professionals fighting this disease. The CPF funds promising research into new approaches to treat and cure PF; provides patients and families with comprehensive education materials, resources, and hope; serves as a voice for national advocacy of PF issues; and works to improve awareness of PF in the medical community as well as the general public. The CPF’s nonprofit partners include many of the most respected medical centers and healthcare organizations in the U.S. With more than 23,000 members nationwide, the CPF is the largest nonprofit organization in the U.S. dedicated to advocating for those with PF. For more information please visit www.coalitionforpf.org or call (888) 222-8541. |
2. Here is the article from the New England Journal of Medicine regarding the genetic connection of IPF:
Genetic Discovery Published in New England Journal of Medicine Good News/Bad News for Patients, Families with Pulmonary Fibrosis
Genetic Variant Associated with MUC5B Gene ID’s Major Risk Factor, Points Research in New Direction |
| A new discovery in a deadly lung disease may change the direction of research while uncovering increased risk for many patients and families. The Coalition for Pulmonary Fibrosis (CPF) and the Pulmonary Fibrosis Foundation (PFF) applaud the efforts of scientists that led to the discovery of a genetic variation associated with the MUC5B gene which may increase the risk of developing Pulmonary Fibrosis (PF). The two patient organizations partner with National Jewish Health (NJH), which led the team of researchers in the study, on a genetic counseling line that provides medical information and support to patients and families. |
| Results of the MUC5B study are published in the April 21, 2011 issue of the New England Journal of Medicine. |
| Pulmonary Fibrosis (PF) is a devastating lung disease with no FDA approved treatment and no cure and scientists have worked for many years with more questions than answers in the quest to understand its cause. |
| Researchers found in study patients with the idiopathic form of the disease (idiopathic pulmonary fibrosis or IPF), the MUC5B genetic variant was present in 67 percent of patients, and in 59 percent of patients with a known familial origin to the disease (Familial Pulmonary Fibrosis or FPF) as well as in 19 percent of healthy controls. |
| The study findings regarding MUC5B and its role may alter the course of research into PF by focusing attention to mucus production in the lungs as well as the space around the air sacs in the lungs. |
| Scientists also found that carrying one copy of the gene increases risk for pulmonary fibrosis 6-9 times and carrying two copies of the variation increases risk by 20-22 times. |
“We are excited about these important new findings as they will guide future investigations in new directions and more precisely identify patients at risk for developing this devastating disease” said Gregory Cosgrove, MD, Director of the Interstitial Lung Disease Program at National Jewish Health.
“Members of families like mine with Familial Pulmonary Fibrosis know we’re at risk for the disease. |
| This news demonstrates that our relative risk is dramatically higher,” said Deirdre Roney, a member of the CPF board of directors who has lost eight members of her family to PF. “On the other hand, I am excited at learning the results of this important new finding in the genetics of Pulmonary Fibrosis. When you see a whole generation of your family perish from a disease, as I have, and you see that future facing all of your relatives, you desperately hope that discoveries around genetics will be found. Once found, the next hope is that gene therapy will be the family’s salvation. |
| My family is deeply grateful to the scientists whose commitment has led to the success of the MUC5B research.” |
| The MUC5B gene is an important finding for all patients with PF, not just people at risk for the genetic version of the disease. The CPF (www.coalitionforpf.org) and the PFF (www.pulmonaryfibrosis.org) partner with National Jewish Health to provide a toll-free PF counseling line. To find out more and to ask questions, please contact the National Jewish Health Genetic Counseling Line at 1-800-423-8891, ext. 1097. |
| “This is a significant genetic finding in pulmonary fibrosis, one that may be used in the future as a tool to identify individuals at risk, used for earlier detection and more predictable prognosis. This finding increases the genetic knowledge for pulmonary fibrosis that physicians and researchers can utilize to develop new tests and target for therapies” said Janet Talbert, MS, CGC Director or the Familial Pulmonary Fibrosis Genetic Counseling Program at National Jewish Health. |
No comments:
Post a Comment