ANCA or anti-neutrophil cytoplasmic autoantibody vasculitis is a group of autoimmune diseases characterized by vascular inflammation. Antibodies typically target foreign agents in the body, such as viruses, bacteria or cancerous cells. Autoantibodies, like ANCAs, target the body’s healthy cells by mistake. In patients with ANCA vasculitis, autoantibodies attack the cells lining the blood vessel. This vascular damage can occur in many different parts of the body, potentially resulting in serious kidney failure, skin lesions and respiratory distress, among a number of other inflammatory symptoms.

Immune system and ANCA vasculitis

The immune system mediates ANCA vasculitis, and many elements in the immune system may play a role in this disease. Most ANCA vasculitis patients either have high amounts of specific perinuclear (P-ANCA) antibodies or have detectable levels of cytoplasmic (C-ANCA) antibodies. P-ANCA and C-ANCA generally target myeloperoxidase (MPO) and proteinase 3 (PR3). These enzymes activate neutrophils — a specific type of white blood cell — to begin destroying blood vessels. Typically, symptoms do not vary much in patients with P- or C-ANCA. However, the disorder may be diagnosed as different types of ANCA vasculitis.

Underlying causes of ANCA vasculitis

Although scientists understand the systemic modes of operation in which ANCA vasculitis damages the body, the factors causing immune system malfunction are largely unknown. Some research models indicate that environmental factors like infectious agents and drugs that trigger the release of pro-inflammatory cytokines — cell-signaling molecules that trigger inflammation — may boost the availability of PR3 and MCO to interact with ANCAs. This research is preliminary and has not been fully vetted in animal models yet.

Genetics appears to play a small role, but the mechanisms of how genetic risk factors may be linked to ANCA vasculitis are not well understood. It has also been suggested that ANCA vasculitis is a combination of genetic predisposition and infection, but no data currently links the disease to common genetic characteristics or previous infections.

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