Kidney Week

Abstract: SA-PO0197

Clinical Characteristics, Triggers, and Kidney Outcomes in 21 Patients with Atypical Hemolytic Uremic Syndrome

Session Information

• Tubular, Metabolic, and Systemic Noncystic Genetic Kidney Diseases
  October 24, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Genetic Diseases of the Kidneys

• 1202 Genetic Diseases of the Kidneys: Non-Cystic (Complex and Non-Cystic Monogenic)

Authors

• Aierken, Ailima, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China

Ailima Aierken

• Zhuang, Jing, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China

Jing Zhuang

• Qu, Yue, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China

Yue Qu, PA

• Gao, Xiyuan, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China

Xiyuan Gao, MA

• Gu, Siwen, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China

Siwen Gu, MD

• Jiang, Hong, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China

Hong Jiang, MD

• Tian, Xuefei, Yale School of Medicine, New Haven, Connecticut, United States

Xuefei Tian, MD, PhD, FASN

Background

Atypical hemolytic uremic syndrome (aHUS) is a rare, complement-mediated thrombotic microangiopathy. While terminal complement inhibitors significantly improve survival, the interplay between environmental triggers, genetic susceptibility, and long-term renal outcomes is unclear. We analyzed 21 patients for genotype-phenotype correlations and trigger effects on renal salvage.

Methods

This single-center observational study included 21 patients with severe aHUS. We evaluated demographics, initiating triggers, baseline laboratory parameters, complement gene mutations, and renal pathology. Analyzing the distribution of triggers, genetic profiles, and the differential renal responses to eculizumab therapy.

Results

Cohort (66.7% female; mean age 27±7y) with diverse triggers: systemic lupus erythematosus (n=7), primary origin (n=4), malignant hypertension (n=3), pregnancy (n=3), infection (n=2), and transplantation (n=2). Baseline disease was catastrophic: median hemoglobin was 66.0 g/L, platelets 71.0×10^9/L, and eGFR 13.3 mL/min/1.73m^2, 61.9% requiring emergent dialysis. Pathogenic variants in 70.6% (p=0.658 across triggers). All patients received eculizumab, achieving hematologic remission within an average of two weeks. Renal outcomes varied significantly according to the precipitating trigger (p=0.001). MHT (100%, 3/3) and primary aHUS (75%, 3/4) progressed irreversibly to end-stage kidney disease. 60% (3/5) of dialysis-dependent patients triggered by pregnancy, infection, or transplant successfully discontinued dialysis. Within the SLE subgroup, 2 AKI recovered, 1 reduced frequency.

Conclusion

While eculizumab safely and universally halts hematologic TMA activity, structural renal recovery is heavily dictated by the specific initiating trigger. Early trigger identification and individualized, hyper-acute interventions are paramount to maximizing nephron salvage in aHUS patients.