ANCA-associated vasculitis
ANCA-associated vasculitides are rare autoimmune disorders characterized by aggressive inflammation that may affect and damage any organ of the body. Little is known about the pathophysiological mechanisms causing the diseases. Current treatment options are typically effective but associated with significant risks. The objectives of our studies are to improve treatment and care of patients with ANCA-associated vasculitides.
Project description
The ANCA-associated small vessel vasculitides (AAV) comprise the rare diseases granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. These are severe inflammatory disorders characterized by necrotizing inflammation of small blood vessels and the presence of auto-antibodies, ANCAs. Patients with AAV are clinically heterogeneous; a wide range of organs may be affected and the severity may vary from mild symptoms to organ- or life-threatening disease. Current therapeutical strategies are effective in the acute phase but rely on broad immunosuppression, associated with risks for infections and malignancies.
In order to improve treatment of patients, we need to increase our understanding of the basic molecular events leading up to disease. Furthermore, for a more individualized treatment and follow-up of patients, it is essential to advance our clinical phenotyping of disease and to prevent co-morbidities.
Our investigations follow three lines of research:
- Identification and functional studies of genetic predisposition of AAV.
Johanna Dahlqvist has established a Scandinavian consortium for genetic analysis of AAV. - Characterization of cellular and molecular events leading up to and propelling the inflammatory cascade in AAV.
- Studies of clinical disease phenotypes, co-morbidities and treatment response in patients with AAV.
Hanna Lindberg, Ann Knight, Johanna Dahlqvist, Erik Hellbacher at the Vasculitis workshop in Barcelona, 2024.