Veeranki Vamsidhar, Meyyappan Jeyakumar, Srivastava Arpit, Kushwaha Ravi Shanker, Behera Manas, Patel Manas Ranjan, Kaul Anupma, Bhadauria Dharmendra Singh, Yachha Monika, Jain Manoj, Kishun Jai, Prasad Narayan
Department of Nephrology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Indian J Nephrol. 2025 May-Jun;35(3):402-409. doi: 10.25259/IJN_106_2024. Epub 2024 Aug 14.
Atypical hemolytic uremic syndrome (aHUS) is a severe thrombotic microangiopathy predominantly affecting the kidneys, often associated with complement dysregulation. This study is aimed to analyze the clinical characteristics, treatment outcomes, and long-term implications of aHUS in a resource-limited setting.
A retrospective observational study conducted at an institute between January 2016 and December 2022 included all patients with aHUS, excluding secondary causes and renal transplant recipients. Demographic profiles, clinical features, laboratory parameters, treatment modalities (immunosuppression and plasma exchange), and outcomes were collected. Anticomplement Factor H (anti-CFH) antibody, complement levels, and genetic mutation analysis were performed to ascertain etiological factors. The patient and renal outcomes of anti-CFH positive and negative patients on long-term follow-up were compared.
Fifty-seven patients (mean age: 12.5 ± 4.9 years; 63% males) were analyzed. Among them, 33 (57.9%) tested positive for anti-CFH antibodies and eight presented postpartum. Initial remission was achieved in 42 (73.6%) patients, with 13 (22.8%) partial and 29 (50.9%) complete remission. The median follow-up duration was 24 months [interquartile range (IQR) 8.5-84]; 12 (21%) patients died, with two deaths during the index admission, six among nonresponders, and 4 among responders. Dialysis-free renal survival was superior in anti-CFH seropositive patients (81.2%) compared to seronegative counterparts (55.9%), while patient survival was statistically similar between the two groups. Elevated anti-CFH titers (>4000 AU/ml), age ≥16 years, female gender, and seizures predicted nonresponsiveness.
Anti-CFH antibody associated aHUS had better kidney outcomes than the seronegative counterparts. In resource limited settings, a combination of plasma exchange and immunosuppression showed promising results in the short and long term.
非典型溶血性尿毒症综合征（aHUS）是一种主要影响肾脏的严重血栓性微血管病，常与补体调节异常有关。本研究旨在分析资源有限环境下aHUS的临床特征、治疗结果及长期影响。
在一家机构于2016年1月至2022年12月进行的一项回顾性观察研究纳入了所有aHUS患者，排除继发原因及肾移植受者。收集人口统计学资料、临床特征、实验室参数、治疗方式（免疫抑制和血浆置换）及结果。进行抗补体因子H（anti-CFH）抗体、补体水平及基因突变分析以确定病因。比较长期随访中anti-CFH阳性和阴性患者的患者及肾脏结局。
分析了57例患者（平均年龄：12.5±4.9岁；63%为男性）。其中，33例（57.9%）anti-CFH抗体检测呈阳性，8例产后发病。42例（73.6%）患者实现初始缓解，其中13例（22.8%）部分缓解，29例（50.9%）完全缓解。中位随访时间为24个月[四分位间距（IQR）8.5 - 84]；12例（21%）患者死亡，其中2例在首次住院期间死亡，6例在无反应者中死亡，4例在有反应者中死亡。anti-CFH血清阳性患者的无透析肾脏生存率（81.2%）高于血清阴性患者（55.9%），而两组患者生存率在统计学上相似。anti-CFH滴度升高（>4000 AU/ml）、年龄≥16岁、女性及癫痫发作预示无反应。
与血清阴性的aHUS患者相比，anti-CFH抗体相关的aHUS肾脏结局更好。在资源有限的环境中，血浆置换和免疫抑制联合应用在短期和长期均显示出有前景的结果。
