FAIRVASC – building registry interoperability to inform clinical care

The Skåne Registry

The Skåne Vasculitis Register: In 1997, we established a large population-based cohort of AAV that we have followed over time to include all consecutive cases of AAV in Skåne. Today the cohort consist of a total of 325 incident cases (150 women) of AAV diagnosed during a 20-year period (1997-2016). All patients are from a defined geographical area in the region of Skåne, the southernmost region in Sweden with a population of 1.3 million inhabitants.  Diagnoses of vasculitis were based on clinical manifestations and supported by serology and histopathology findings of vasculitis and/ granuloma. The classification of AAV into the three disease types (GPA, MPA and EGPA) was based on the European Medicines Agency (EMA) algorithm. In addition to basic clinical and demographic data, the register contains information on disease activity and organ involvement at time of diagnosis as well as outcome data including organ damage at 1 year after diagnosis of AAV  In addition, the occurrence of the following comorbidities has been registered: cardiovascular diseases, venous thromboembolic diseases, cancer and severe infections. Furthermore, information on renal and patient’s survival and cause of death are continuously updated.