What is ANCA Associated Vasculitis (AAV)
ANCA Associated Vasculitis is a type of autoimmune inflammation caused by auto-antibodies. Normal antibodies are parts in the blood that are produced by the immune system to fight infectious agents (such as bacteria). Auto-antibodies are abnormal antibodies that attack one's own cells and tissues (autos = self). ANCAs are auto-antibodies that attack the thick liquid residing between the cell membrane holding all the cell's internal sub-structures (cytoplasm), except for the nucleus or internal portion of the cell, of a certain type of white blood cells called neutrophils. ANCA stands for Anti-Neutrophil Cytoplasmic Auto-antibody. When ANCAs attack these neutrophils, they cause the white blood cells to attack the walls of small and medium vessels in different tissues and organs of the body.
ANCA Associated Vasculitis encompasses Granulomatosis with Polyangiitis (GPA, formerly known as Wegener's Granulomatosis), Microscopic Polyangiitis (MPA), Churg-Strauss Syndrome (CSS) and Renal (Kidney) Limited Form.
The ANCA Associated Vasculitis Foundation is dedicated to finding a cure for related forms of ANCA Associated Vasculitis within the next 10 years through an aggressively funded research agenda and to ensuring the development of improved therapies for those living with forms of ANCA Associated Vasculitis today. The ANCA Associated Vasculitis Foundation is committed to providing support, resources, and funding to those living with, treating and researching these forms of ANCA associated Vasculitis.
Throughout the years, Wegener's Vasculitis has been called many things. It was first described by Peter McBride (1854–1946) in a BMJ article titled, "Photographs of a case of rapid destruction of the nose and face." The name of the disease around this time would begin to be called pathergic granulomatososis. Heinz Karl Ernst Klinger, a student of Friedrich Wegener (a German pathologist), would add information on the anatomical pathology in 1931. Friedrich Wegener wrote two reports in 1936 and 1939, and is credited for his descriptions of a rare inflammation in blood vessels that eventually came to be known as Wegener's Granulomatosis.
In 2006, Dr. Alexander Woywodt (Preston) and Dr. Eric Matteson (Mayo Clinic) investigated Dr. Wegener's past and found that Dr. Wegener had ties to the Nazi regime and genocide machinery in Lodz. Their data raised serious concerns about Dr. Wegener's professional conduct. They suggested that his name be disassociated with the disease and proposed the acronym for Anti-Neutrophil-Cytoplasmic-Antibodies—ANCA Associated Vasculitis—since ANCA is associated with other form of Vasculitis and not just Wegener’s. In 2011, the authors again asked to have the disease renamed, this time to Granulomatosis with Polyangiitis (GPA).
The "Why" Behind the Foundation
The ANCA Associated Vasculitis Foundation was inspired by Carl W. Lundin III, a business owner who developed ANCA Associated Vasculitis and who was living with Type 1 Diabetes for more than 30 years prior.
Prior to being diagnosed, Lundin made repeated doctor and hospital emergency room visits, only to be either misdiagnosed or sent away with the prescription that his symptoms would work themselves out in a couple of weeks. In the meantime, Lundin could barely conjure up enough energy to get to a doctor. That was frustration enough. But after being diagnosed with ANCA Associated Vasculitis, the frustration mounted as it became increasingly apparent that good information about the best current treatments and care centers available or simply finding a support network of others battling the same disease just didn't exist.
It is Lundin's hope that the ANCA Associated Vasculitis Foundation will provide the essential information and support he found lacking when he was diagnosed. Lundin believes that with a concentrated effort from the ANCA Vasculitis community, elected officials in Washington, DC, and—most importantly—the general public, researchers can pinpoint the cause of ANCA and discover a cure within our lifetime.
Please contact your representatives and let them know we need more funding made available for research, treatment and support programs for people suffering from ANCA Associated Vasculitis.
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