Introduction: Primary systemic necrotizing vasculitides (SNVs) include polyarteritis nodosa, Kawasaki disease, ANCA-associated vasculitides, IgA vasculitis, and cryoglobulinemic vasculitis. All are rare but potentially severe, life-threatening conditions. Evidence-based treatments are well established, but continue to evolve and management requires some expertise. Areas covered: The objectives of this review are to outline results of the main recent therapeutic studies for SNV, which have led to the establishment of current treatment strategies and significant improvement in patients' outcomes, and to describe knowledge gaps that ongoing research hopes to bridge. Expert opinion: Therapy is mainly dictated by diagnosis, disease extent, and severity. In ANCA-associated vasculitis, an initial induction phase consists of tapering glucocorticoids combined with specific immunosuppressants. Maintenance therapy begins after 3 to 6 months, once all evidence of active disease has resolved, and can require years of therapy to prevent relapse. Results from ongoing and future trials for vasculitis will likely impact these treatment approaches. Entirely avoiding GC may become possible, perhaps even the next gold standard, if medications such as avacopan are confirmed to be safe and effective. New combination strategies, more individualized for each patient, may also prove to be more effective, faster.
Keywords: IgA vasculitis; Kawasaki disease; Vasculitis; cryoglobulinemic vasculitis; eosinophilic granulomatosis with polyangiitis; granulomatosis with polyangiitis; microscopic polyangiitis; polyarteritis nodosa.