Vasculitis Research Initiative

What is Vasculitis?
Vasculitis is a painful, debilitating and devastating vascular disease characterized by:
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  • Immune cells directed against one's own vascular structures
  • Inflammation and injury to medium and small blood vessels
  • Death of the endothelium (cells lining of the vascular system). 
  • Multiple organ involvement- kidney, lung, skin, joints and GI tract
  • High rates of organ failure and mortality.
  • The causes of vasculitis are poorly understood.
  • Better therapeutic treatments are urgently needed. 
Some conditions in which auto-antibodies are directed against neutrophil cytoplasmic antigens  (ANCA) are medically known as microscopic polyangiitis, granulomatosis with polyangiitis (GPA) (Wegener's granulomatosis) and Churg Strauss vasculitis.  More research for drug discovery and animal models are desperately needed to gain a better understanding of the causes of these debilitating diseases and to develop improved therapeutic approaches. Symptoms of ANCA-associated vasculitis discussed by Johns Hopkins
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ANCA auto-antibodies attack the blood cells (Neutrophils) that normally help protect our body from bacterial infections. ANCA antibodies bind to specific neutrophil proteins (myeloperoxidase (MPO), Proteinase 3 (PR3) and Lysosome-Associated Membrane Protein-2 (LAMP-2) on the surface of activated neutrophils. The neutrophils respond to this binding by releasing cytotoxic proteins that injure and often kill the endothelial cells lining small blood vessels. Injured vessels promote more inflammation setting up a complex vicious cycle of inflammation and vessel damage eventually resulting in organ failure. Probetex specializes in vascular (capillary) diseases of the kidney. We are asking for your help in our quest to develop sheep antiserum against neutrophil antigens MPO, PR3, and LAMP-2. These antisera will provide new animal models to aid researchers in their investigations of ANCA vasculitis and provide a platform to develop new therapeutic interventions.
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How is Probetex Helping?
Experimental models have provided a wealth of information on the pathogenesis of small vessel disease. However, it is widely viewed that additional economical animal models of ANCA that mimic the human condition are needed to aid in the development of treatments to combat these devastating diseases. We are seeking funds from a variety of sources to develop new models that can be used by investigators world-wide to study mechanisms and treatments for ANCA vasculitis. Proposal description..... (more)


Famous celebrities that had/have vasculitis:
Janet Leigh: Actress
Jean Harlow: Actress
Harold Ramis: Comedian/Actor/Director
Irina Slutskaya: Olympic skater
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