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Systemic vasculitis is a group of heterogeneous, disabling disorders. Great interest has recently arisen in pathophysiology, clinical phenotypes and therapy of large- and small-vessel vasculitis. The general work hypothesis has been to promote research focused on disease-related pathogenetic pathways, with the ultimate goal of identifying novel diagnostic and prognostic biomarkers, thus leading towards more effective targeted treatments. Following the previous annual reviews of this series, we will hereby provide a critical digest of the recent literature on small- and large-vessel systemic vasculitis, with a specific focus on novel possible disease-related biomarkers and their impact on current and future therapies.

Juvenile systemic lupus erythematosus (JSLE) is a systemic autoimmune chronic disease that can affect any part of the body. It is characterised by formation of antibodies against nuclear antigens. Vasculitis may be found in the course of SLE but rarely corresponds to an anti-neutrophil cytoplasmic antibodies (ANCA) - associated vasculitis (AAV. We report five cases of severe JSLE associated with AAV diagnosed between 1991 to 2011 in three University- based tertiary care centers. All patients (3 girls and 2 boys, aged 12 to 17) presented with severe clinical picture and following features: cytopenia (n=5), autoimmune hepatitis (n=3), lupus nephritis stage V (n=1), pancreatitis (n=1), secondary antiphospholipid syndrome (n=2), impending respiratory failure (n=2) and gastointestinal bleeding (n=1). All patients were proteinase 3 (PR3) ANCA positive, while one of them were myeloperoxidase (MPO) and PR3 ANCAs positive at the same time. Patients were treated with corticosteroids and immunossupresive drugs (cyclophosphamide and azathioprine). Remission of disease was achieved at three patients. Course of disease had been worsening in two patients and we started with Rituximab (anti- CD20). In conclusion, all of our patients presented as the most severe SLE patients that need to be diagnosed as soon as possible and treat very intensively. Since an coexistance of JSLE and AAV occurs very rarely in children, presentation of such patients, their clinical pictures, treatment and course of diseases are experiences that can be of great help.