Service d'Immunologie Biologique, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France.
Semin Thromb Hemost. 2010 Sep;36(6):633-40. doi: 10.1055/s-0030-1262885. Epub 2010 Sep 23.
Non-Shiga toxin-associated hemolytic uremic syndrome (atypical HUS) is a rare form of thrombotic microangiopathy that associates hemolytic anemia, thrombocytopenia, and acute renal failure. The disease has been demonstrated to be linked with a complement alternative pathway dysregulation due to genetic defects but also to development of autoantibodies to factor H (FH), the main plasmatic alternative pathway regulatory protein. In this review, we summarize the more recent data of this autoimmune form of HUS at the level of epidemiology and its clinical and biological features. We propose the performance of anti-FH autoantibodies screening at the very onset of the disease in all cases of HUS to first make the proper diagnosis as early as possible, and second to support an appropriate therapy including early plasma exchanges and immunosuppressive treatments.
非志贺毒素相关性溶血尿毒症综合征（非典型 HUS）是一种罕见的血栓性微血管病，其特征为溶血性贫血、血小板减少和急性肾衰竭。该疾病已被证明与补体替代途径失调有关，这种失调是由于遗传缺陷所致，但也与针对因子 H（FH）的自身抗体的产生有关，FH 是主要的血浆替代途径调节蛋白。在这篇综述中，我们总结了这种自身免疫性 HUS 在流行病学水平及其临床和生物学特征方面的最新数据。我们建议在所有 HUS 病例中，在疾病发作时即进行抗 FH 自身抗体筛查，以便尽早做出正确诊断，其次是支持适当的治疗，包括早期血浆置换和免疫抑制治疗。
