Akbariansaravi Abrisham, Dekhne Anushka, Dhamelia Archi, Mekhail Mario
Internal Medicine, American University of Antigua, Antigua, ATG.
Internal Medicine, MGM (Mahatma Gandhi Mission) Medical College, Navi Mumbai, IND.
Cureus. 2024 Oct 7;16(10):e71019. doi: 10.7759/cureus.71019. eCollection 2024 Oct.
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by hemolytic anemia, renal failure, and thrombocytopenia. While the typical form of HUS is often associated with Shiga toxin-producing (STEC) infections, atypical hemolytic uremic syndrome (aHUS) is caused by uncontrolled complement system activation, leading to endothelial damage, microthrombi formation, and other complications. Although aHUS is commonly linked to genetic mutations and infections, emerging evidence suggests that certain substances, particularly illicit drugs like heroin, cocaine, and ecstasy, can also trigger this condition, adding complexity to its diagnosis and management. This narrative review examines the mechanisms by which substance use can lead to aHUS, discusses its clinical presentation, and highlights the diagnostic challenges in distinguishing it from other thrombotic microangiopathies, such as thrombotic thrombocytopenic purpura (TTP) and STEC-HUS. A thorough literature search identified relevant case reports, case series, and observational studies, underscoring the need for genetic testing and complement assays to confirm aHUS in substance users. The review also explores the role of complement inhibitors, such as eculizumab and ravulizumab, which target the underlying pathophysiology and have shown promise in improving patient outcomes. However, the management of substance-induced aHUS remains challenging due to limited data, varying clinical presentations, and the need to optimize treatment protocols. Early recognition and tailored therapy are crucial for effective management. Further research is needed to refine diagnostic criteria, develop new therapeutic approaches, and improve care for patients with this under-recognized condition.
溶血尿毒综合征（HUS）是一种血栓性微血管病，其特征为溶血性贫血、肾衰竭和血小板减少。虽然典型的HUS形式通常与产志贺毒素大肠杆菌（STEC）感染有关，但非典型溶血尿毒综合征（aHUS）是由补体系统的失控激活引起的，导致内皮损伤、微血栓形成及其他并发症。尽管aHUS通常与基因突变和感染有关，但新出现的证据表明，某些物质，特别是海洛因、可卡因和摇头丸等非法药物，也可引发这种疾病，使其诊断和管理变得更加复杂。这篇叙述性综述探讨了物质使用导致aHUS的机制，讨论了其临床表现，并强调了将其与其他血栓性微血管病（如血栓性血小板减少性紫癜（TTP）和STEC-HUS）区分开来的诊断挑战。全面的文献检索确定了相关的病例报告、病例系列和观察性研究，强调了进行基因检测和补体检测以在物质使用者中确诊aHUS的必要性。该综述还探讨了补体抑制剂（如依库珠单抗和ravulizumab）的作用，这些抑制剂针对潜在的病理生理学，在改善患者预后方面已显示出前景。然而，由于数据有限、临床表现各异以及需要优化治疗方案，物质诱导的aHUS的管理仍然具有挑战性。早期识别和量身定制的治疗对于有效管理至关重要。需要进一步研究以完善诊断标准、开发新的治疗方法并改善对这种未得到充分认识的疾病患者的护理。
