抗糸球体基底膜(GBM)抗体疾患に対するアフェレシス療法(<特集>血管炎症候群とアフェレシス) Apheresis Therapy for Anti-glomerular Basement Membrane Antibody Disease
Anti-glomerular basement membrane (GBM) antibody disease is a relatively rare autoimmune disorder in which glomerulus and/or pulmonary capillary are injured by an anti-GBM antibody that binds to be basement membrane. In Japan, it occurs in around 6% of rapidly progressive glomerulonephritis cases. Since the disease activity of anti-GBM antibody nephritis is very high, the following strategy is necessary as soon as possible to control the disease activity ; strong anti-inflammation by corticosteroid, removal of anti-GBM antibody by plasmapheresis, and suppression of antibody production by immunosuppressive therapy. Though it is recommended to perform plasmapheresis with 5% albumin as the replacement fluid until the antibody titer is reduced to a normal value, no definitive protocol has yet been established. Because plasmapheresis is not effective for renal prognosis in patients with severe renal dysfunction, quick diagnosis and treatment is needed to improve their prognosis.
日本アフェレシス学会雑誌 34(2), 126-130, 2015