The Vasculitis Foundation is pleased to announce it has selected two new research studies on EGPA/Churg Strauss for funding in the amount of $98,000. EGPA/CSS is a serious rare autoimmune condition that affects multiple organs primarily lung, peripheral nerves, heart, gastrointestinal tract and skin. The disease can manifest as a combination of symptoms, ranging from asthma, rash, fever, gastrointestinal bleeding, numbness of hands and feet and joint pains. EGPA/CSS has no cure and the different symptoms are controlled by a combination of powerful anti-inflammatory and immunosuppressant drugs, with severe side effects.
The first study, “Exome Sequencing in EGPA Patients” is a two-year study led by Dr. Davide Martorana, Ph.D., Diagnostic Department, Medical Genetics Unit, University Hospital of Parma, Italy. Specific aims of the study will be to develop newer potential EGPA diagnostic and prognostic biomarkers by exome sequencing and computational modeling, clinical subset stratification of EGPA patients on the basis of the genetic study and to ascertain the diagnostic-prognostic role of the whole findings.
The second study is titled, “Investigating Autoimmune Mechanisms Underlying EGPA”. It is a $50,000, one-year study, led by Parameswaran Nair, M.D., McMaster University in Ontario, Canada.
There is a need to understand the underlying mechanisms of the disease to improve patient management and cure. Autoantibodies against a type of white blood cells called neutrophils (usually associated with infections) which particularly target ‘myeloperoxidase’ (MPO), is seen in 40% of the EGPA patients presenting with severe respiratory (asthma) and heart complications. However, the disease as obvious by its name is characterized by an increase of eosinophils in blood and airways. The reason for this paradigm is not known. Eosinophils are a different kind of white blood cells that exclusively release ‘eosinophil peroxidase’ (EPX), a similar compound to MPO.
In this study, the investigators propose to determine the presence of antibodies against MPO, their relation to severity of the disease and a possible involvement of EPX in the underlying autoimmune mechanism. This novel idea has therapeutic implications in that these auto antibodies may determine the severity of disease and allow us to better manage and treat these patients.
The studies were selected through a special award competition held earlier this year and are being funded by a generous donation from Mr. and Mrs. Ben and Kathy Johnson.
The Vasculitis Foundation is the largest private funder of research on vasculitis and collaborates with researchers around the world to fund the most promising studies. The program is guided by the VF Scientific Advisory Board which reviews, critiques and ranks the applications and provides guidance to the VF Board of Directors on the selection of studies to fund. The Program is funded by donations, honors, memorials and special fundraising events.