Hellmich B, Löffler C
Vaskulitiszentrum Süd, Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken - Akademisches Lehrkrankenhaus, Universität Tübingen, Eugenstr. 3, 73230, Kirchheim u. Teck, Deutschland.
Z Rheumatol. 2019 Dec;78(10):955-966. doi: 10.1007/s00393-019-00687-0.
Severe, organ-threatening and life-threatening manifestations of inflammatory rheumatic diseases, such as diffuse alveolar hemorrhage in the context of small vessel vasculitis, sometimes inadequately respond to immunosuppressive treatment. In the case of an immanent or already occurring organ failure, immunosuppressive treatment may need to be supplemented with rapidly effective rescue treatment procedures. Due to the rarity of many rheumatic diseases, the evidence for the use of rescue treatment, such as plasmapheresis, extracorporeal membrane oxygenation (ECMO) and the administration of intravenous immunoglobulins (IVIG), is relatively low for many indications. The use of plasmapheresis is considered useful in acute anti-glomerular basement membrane (GBM) disease (Goodpasture's syndrome) or catastrophic antiphospholipid antibody syndrome (APS). The use of ECMO treatment may be considered for persistent respiratory failure despite mechanical ventilation due to diffuse alveolar hemorrhage or acute respiratory distress syndrome (ARDS). Administration of IVIG is indicated for acute cardiac involvement in Kawasaki's disease and may be considered in catastrophic APS and refractory myositis.
炎症性风湿性疾病的严重、危及器官和危及生命的表现，如小血管血管炎背景下的弥漫性肺泡出血，有时对免疫抑制治疗反应不佳。在即将发生或已经出现器官衰竭的情况下，免疫抑制治疗可能需要辅以迅速有效的抢救治疗措施。由于许多风湿性疾病较为罕见，对于诸如血浆置换、体外膜肺氧合（ECMO）和静脉注射免疫球蛋白（IVIG）等抢救治疗的使用证据，在许多适应症方面相对较少。血浆置换被认为对急性抗肾小球基底膜（GBM）病（肺出血肾炎综合征）或灾难性抗磷脂抗体综合征（APS）有用。对于因弥漫性肺泡出血或急性呼吸窘迫综合征（ARDS）导致机械通气后仍持续存在的呼吸衰竭，可考虑使用ECMO治疗。IVIG适用于川崎病的急性心脏受累，在灾难性APS和难治性肌炎中也可考虑使用。
