Roadmap to vasculitis: a rheumatological treasure hunt: Part IV. Management of vasculitis

At the stop sign we read the “red flags” and made up our mind and followed one of the road signs pointing to secondary, primary or fake vasculitis. Since then we have steadily followed the road map and passed the first (patient history and physical exam), second and third milestones (laboratory, imaging and pathology studies in the primary care and specialized centres) and have finally reached our destination at the fourth milestone (Part IV) on the road map review to vasculitis. In the management of these syndromes, Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI) are not widely used in the routine clinical work, but they are introduced as the idea behind them is really valid. The backbone of the medical therapy is the use of immunosuppressive doses of prednisone (1 mg/kg/day). In some life-threatening and non-responsive vasculitides this is combined with cyclophosphamide 2–4 mg/kg/day or 0.5–1.0 g/m2 i.v. every 2–4 weeks (European Vasculitis group uses 15 mg/kg every 2–3 weeks), often at 3–6 months substituted either with methotrexate or azathioprine. In contrast, i.v. immunoglobulins are to be used in Kawasaki's syndrome; cyclosporine, dapsone or colchicine in Behçet's disease; calcium channel blockers in BACNS; and NSAID in small vessel disease; whereas plasmapheresis or immunoadsorption are added to the therapy in Goodpasture's syndrome. Particular attention is drawn to the treatment of the triggers, use of biologicals and new cytostatic drugs and anti-metabolites, prevention of thromboembolic complications with anti-platelet drugs as well as to odd and orphan entities. A short travelogue ends our odyssey as the last sign on our roadmap.