Zhang Xiaohui, Bi Wenhua, Ma Chengjun, Li Kun, Gao Yanxia
Department of Nephrology, Qilu Hospital of Shandong University (QingDao), Qingdao, China.
J Hypertens. 2025 Aug 7. doi: 10.1097/HJH.0000000000004116.
Complement-mediated thrombotic microangiopathy (TMA) presenting as malignant hypertension (mHTN) is frequently misdiagnosed as mHTN-associated TMA, particularly when their clinical presentations overlap. The diagnostic challenge is further compounded when patients have normal serum levels of complement factor H (CFH) and test negative for anti-CFH. We presented two patients of complement-mediated TMA exhibiting clinical features consistent with mHTN-associated TMA, yet their renal function did not improve following the achievement of target blood pressure (BP) levels. Notably, both patients demonstrated elevated serum soluble C5b-9 levels. Based on these findings, we hypothesized the presence of complement-mediated TMA and initiated treatment with eculizumab. Consequently, one patient was successfully weaned off dialysis, and the other experienced substantial improvement in renal function, highlighting that in patients with mHTN-associated TMA where antihypertensive treatment fails to ameliorate renal function, consideration should be given to the possibility of complement-mediated TMA.
表现为恶性高血压（mHTN）的补体介导的血栓性微血管病（TMA）常被误诊为mHTN相关的TMA，尤其是当它们的临床表现重叠时。当患者血清补体因子H（CFH）水平正常且抗CFH检测呈阴性时，诊断挑战会进一步加剧。我们报告了两名补体介导的TMA患者，其临床特征与mHTN相关的TMA一致，但在达到目标血压（BP）水平后肾功能并未改善。值得注意的是，两名患者的血清可溶性C5b-9水平均升高。基于这些发现，我们推测存在补体介导的TMA，并开始使用依库珠单抗进行治疗。结果，一名患者成功停用透析，另一名患者的肾功能有显著改善，这突出表明，在mHTN相关的TMA患者中，若抗高血压治疗未能改善肾功能，则应考虑补体介导的TMA的可能性。
