New Hope for Patients PDF Print E-mail
Monday, 30 May 2011 20:40
taken from http://www.curecancerproject.orgPulmonary Fibrosis (PF) and Pulmonary Arterial Hypertension (PAH) are incurable rare lung diseases and are the leading cause of mortality in patients with Systemic Sclerosis (SSc). However patients might have a reason to breathe easier since researchers from the University of Pittsburgh now provide a novel insight that explains how Systemic Sclerosis develops.

Systemic Sclerosis (SSc) is a rare autoimmune disease which affects the connective tissue of the skin and internal organs, such as lungs, kidneys or heart. It is often accompanied by pulmonary complications, Pulmonary Fibrosis (PF) and Pulmonary Arterial Hypertension (PAH), for which therapies have only a low benefit and patients often require a lung transplantation. Despite previous research, how the disease develops remains poorly understood.

How SSc develops has eluded researchers, because they have lacked the necessary samples of affected tissue from patients. That is why Dr. Carol A. Feghali-Botswick’s research team from the University of Pittsburgh School of Medicine started collecting lung tissue samples from affected patients.

“The challenge was collecting a critical number of lung tissues from patients with SSc,” said Dr. Carol A. Feghali-Botswick. A certain number of lung tissue samples from patients with Systemic Sclerosis was necessary to obtain good results, but as lung biopsies are not routinely done the availability was fairly limited.

“We started banking these tissues in the mid-1990s and had to wait until we accrued a sufficient number,” explained Dr. Feghali-Botswick. “The University of Pittsburgh Medical Center has a vibrant lung transplant program and transplants more SSc patients than anywhere else in the country or the world, so we were uniquely poised to do this study once we had enough patients donate their lungs.” Finally they managed to analyze lung tissues samples from 33 patients with SSc who underwent lung transplantations. They created a list of characteristics, so called gene expression profiles, which were unique or shared throughout the samples.

With the data in hand, Dr. Feghali-Botswick’s team was able to generate signatures that were specific for SSC as well as signatures that were specific for the two complications: Pulmonary Fibrosis (PF) and Pulmonary Arterial Hypertension (PAH). These signatures provided Dr. Feghali-Botswick’s team with insights to genes that might be involved in causing SSc.

To follow up this successful work the researchers are continuing the next phase of the project. “We are currently looking at some of the genes identified through the analysis to determine if they can serve as markers of lung disease in SSc, and possibly even as predictors of lung involvement.“

Markers, such as biomarkers, are substances used to examine an organ’s function or other aspects of health. It is an important tool in diagnostics that allows for easy disease detection, limits the risks of misdiagnosis and also reduces the costs needed for treatment.

In the ongoing search for potential markers, Dr. Feghali-Botswick told CheckOrphan, “Some patients have serum samples obtained prior to the onset of their lung disease as well as following the diagnosis of lung involvement”. This way the researchers will be able to measure the proteins in the serum and to see if some of the protein levels increase prior to the onset of lung involvement or only following the development of lung disease.

“In the long run, this will allow us to develop a panel of proteins we can use for screening patients at risk of developing lung disease with the goal of initiating treatment very early when it's likely to be more effective,” Dr. Feghali-Botswick said.

“I would like to emphasize that none of this work would have been possible without the generosity of our patients who are always willing to donate blood, skin biopsies and lung tissues following lung transplantation surgery.“

Malisova, B. (2011), "New Hope for Patients With Rare Lung Diseases, Systemic Sclerosis (SSc)"; original article can be accessed here.
 
More articles :

» Geneticists Hunt for Scleroderma Triggers

In all its forms, gives Dartmouth geneticist Michael Whitfield, his graduate students, and his postdoctoral researchers a sense of urgency in their search for the triggers of the chronic condition. In a study that the Journal of Investigative...

» UT Researchers At Forefront Of Efforts To Stem The Tide Of Autoimmune Diseases

Autoimmune and autoinflammatory diseases are on the rise and researchers and physicians at are at the forefront of efforts to stem the tide. These diseases are often hard to diagnose and often affect individuals differently.Autoimmune and...

» Eating Well: Nutritional Needs in Scleroderma

The purpose of this article is to identify nutritional needs in and provide practical food advice, with the two goals of minimizing nutrition-related side effects and to improve your overall nutritional health. Since Scleroderma can be manifested...

» The FDA Approves IV Sildenafil for Hypertension

The FDA has approved an intravenous formulation of the pulmonary arterial hypertension drug sildenafil (Revatio) for patients who are temporarily unable to take the medication in its oral form. The tablet form of sildenafil was approved in 2005 to...

» Pulmonary Arterial Hypertension And Scleroderma

is a disease that affects the skin and internal organs. The literal meaning of the word is "hard skin"; people with Scleroderma experience a hardening and tightening of their skin. The inflammation and scarring associated with Scleroderma can cause...

» New Stem Cell Therapy To Help With Stubborn Wounds

The skin is the largest organ in the body with amazing healing powers, but what if your wounds didn't heal?That's the case for 6 million Americans suffering from chronic wounds that can take months, even years, to heal. Millions have no other choice...

Add comment

Do feel free to leave your comments, as they would add value and knowledge to the community. However, please refrain from making any disparaging, uninformed, or unrelated comments. Thanks :)

Security code
Refresh