Mediterr J Rheumatol 2015; 26(2): 18-42
Evidence based treatment of systemic vasculitides
Authors Information

Clinical Immunology-Rheumatology Unit, 2nd Department of Internal Medicine and Laboratory, Athens Medical School, Hippocration General Hospital, Athens

Systemic vasculitides comprise a wide spectrum of diseases with high mortality and morbidity when not treated appropriately. In this paper, a systematic review of the suggested treatment regimens was performed under the scope of recent data regarding the use of biologic agents for the treatment of systemic vasculitides. For Takayasu arteritis and giant-cell arteritis, classic corticosteroid therapy as well as the use of conventional synthetic disease-modifying antirheumatic drugs (DMARDs) and the role of biologic therapies for resistant or relapsing cases is discussed. In antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitides, treatment depends on disease severity and here we discuss the indications of a recently approved biologic agent that depletes B lymphocytes (rituximab). Data on adult Immunoglobulin A (IgA) vasculitis are scarce and are mainly derived from studies in children. Regarding Behcet’s disease, treatment regimens are suggested based on organ involvement and new data are presented for newer treatment options in resistant or relapsing cases. A special mention is made for the need of collaboration between the rheumatologist and hepatologist in secondary virus-associated (HBV, HCV) forms of cryoglobulinemic vasculitis and polyarteritis nodosa. Finally, we discuss the indications of non drug treatments, such as plasma exchange, for life threatening cases of ANCA associated or cryoglobulinemic vasculitis.