ANCA-associated vasculitis
ANCA-associated vasculitides (AAV) are a group of relatively rare diseases (Prevalence: 1-5/10.000) characterized by recurrent, chronic small vessel inflammation and deleterious organ damage. A hallmark of AAV is the presence of autoantibodies (called anti-neutrophil cytoplasmic antibodies, ANCA). ANCA are intimately involved in triggering and maintaining the inflammatory disease process. In principle, AAV can affect all organ systems, but kidney and lung disease (upper and lower airways) can quickly become organ- and life-threatening and are clinically most relevant. In recent years, early AAV diagnosis and initial disease control by targeted treatment have improved considerably. In particular, early depletion of B cells has proven very efficacious as first-line intervention, showing the relevance of B cells and auto-antibodies in this disease process. Nonetheless, disease flares are common and feared, as each flare can cause and aggravate irreversible organ damage. Consequently, an important clinical challenge now is the prediction and control of flares and the recognition of disease persistence (residual disease) despite immunosuppressive treatment. We aim to understand these aspects of AAV as such understanding is crucial to reduce the burden of treatment complications and to induce both clinical and immunological cure.
Studies into the pathophysiology of ANCA-associated vasculitis
The potentially life-threatening disease anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is characterized by autoantibodies against proteinase 3 (PR3) and myeloperoxidase (MPO). Despite decades of research, the trigger that initially breaks tolerance and causes ANCA production remains unknown. An important aim of our research is to gain better understanding of the trigger that breaks tolerance as well as the trigger that is responsible for disease flare.
…The potentially life-threatening disease anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is characterized by autoantibodies against proteinase 3 (PR3) and myeloperoxidase (MPO). Despite decades of research, the trigger that initially breaks tolerance and causes ANCA production remains unknown. An important aim of our research is to gain better understanding of the trigger that breaks tolerance as well as the trigger that is responsible for disease flare.
As ANCA-associated vasculitis is a relatively rare disease, the research is embedded in the larger investigations into the autoreactive B cell- and antibody response performed at our department as this allows optimalization of effort and resources through sharing of know-how, infrastructure and technological advances.