Systemic vasculitis is a group of diseases with severe and acute inflammation in the small vessels. The cause of the inflammation is unknown and it occurs without known external influence. Autoantibodies are known to play a role, but this could be from direct attack of the target organ to being markers of the specific inflammation. The clinical picture varies, depending on the organ that is damaged, making the diagnosis difficult. Apparently healthy individuals can within a couple of weeks develop a life threatening disease. We have concentrated our research on Goodpasture's disease (associated with anti glomerular basement membrane antibodies, anti-GBM), Granulomatosis with polyangeiitis and Microscopic polyangiitis (both associated with anti neutrophilic cytoplasmatic antibodies, ANCA).
Our vision is to reduce patient suffering and save health care costs by increasing the knowledge of pathogenic mechanisms, which would result in development of more specific diagnostic tools and a curative therapy for patients with systemic vasculitis. This will be done by:
1. study of inherited and acquired abnormalities in the immune system that leads to auto-inflammation and autoantibody formation in the patients;
2. development of new tools that can aid the diagnosis and/or monitor the disease activity and therapeutic efficacy;
3. investigator initiated clinical trials and epidemiological studies to develop and evaluate new therapeutic strategies both in the short and the long term perspective.
We hypothesize that the primary cause of systemic vasculitis is an acquired intrinsic cellular abnormality of the leukocytes, possibly triggered by an infectious agent. This abnormality leads to dysfunctional activation and apoptosis programs of leukocytes. In turn, this causes an increased exposure of antigens to the adaptive immune system in a pro-inflammatory milieu, with autoantibody formation as a consequence. Hence, the autoantibodies are a secondary phenomenon but fuel the inflammation leading to the full and devastating phenotype of the disease.