New Information for the Diagnosis and Treatment of Systemic Vasculitis

Abstract

Systemic vasculitis is a general term for the disease that causes various organ failure with inflammatory vasculitis. At present, systemic vasculitis is classified by the size of the blood vessel as described by the Chapel Hill Consensus Conference (CHCC) criteria. Small size vasculitis is classified as ANCA-associated vasculitis and immune-complex associated vasculitis. Various symptoms occur with systemic vasculitis because various sizes of vessels cause injury. The pathological etiology of systemic vasculitis is giant cell arteritis, necrotizing vasculitis or leukocyteclastic vasculitis. Glucocorticoids were the main treatment for systemic vasculitis, but today, combination therapy with glucocorticoids and some immunosuppressive drugs is the mainstream treatment for systemic vasculitis. Recently, some biological drugs have been used in the treatment of systemic vasculitis. Tocilizumab, an IL-6 receptor antagonist, is used to treat rheumatoid arthritis and Castleman’s disease. In 2017, tocilizumab was used to treat Takayasu arteritis and giant cell arteritis, which it is able to improve with glucocorticoid monotherapy. Infliximab, an anti-TNFα monoclonal antibody, is used as a treatment for rheumatoid arthritis and other autoimmune diseases. In 2015, infliximab was used as a treatment for Kawasaki disease, which it improves, by intravenous immunoglobulin treatment. Rituximab, an anti-CD20 monoclonal antibody, is usually used as a treatment for CD20+ B cell lymphoma. While rituximab is usually used to treat RA and SLE in the United States and Europe, it cannot be used to treat RA and SLE in Japan. In 2017, rituximab was used as a treatment for MPA and GPA when there is improvement caused by glucocorticoids. Mepolizumab, an IL-5 monoclonal antibody, is used as treatment for severe bronchial asthma. Since 2017, mepolizumab has been used as a treatment for EGPA when there is improvement caused by glucocorticoids. It is expected that more new treatments for systemic vasculitis will be developed.