Abstract: SA-PO038

Spanish Experience of Pregnancy-Associated Atypical Hemolytic Uremic Syndrome

Session Information

Category: Acute Kidney Injury

  • 003 AKI: Clinical and Translational

Authors

  • Huerta, Ana, Puerta de Hierro Hospital-Nephrology Department., Madrid, Spain
  • Rodriguez de Cordoba, Santiago, Consejo Superior de Investigaciones Cientificas, Madrid, Spain
  • Arjona, Emilia, Centro de Investigaciones Biologicas- CSIC, Madrid, Spain
  • Praga, Manuel, Hospital 12 de Octubre, Pozuelo De Alarcon, Spain
  • Portoles, Jose M., Hospital Universitario Puerta de Hierro , Majadahonda, Spain

Group or Team Name

  • Spanish Group for Study of Complement and Renal Disease
Background

Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) refers to the thrombotic microangiopathy that result from uncontrolled complement activation during pregnancy or the postpartum period. P-aHUS is a devastating disease for which we still have a limited clinical understanding and treatment experience. Here we report a retrospective study to analyze the clinical and prognostic data under different treatments of the cohort of P-aHUS patients from the Spanish aHUS Registry.

Methods

In the Spanish aHUS Registry we identified 242 adult female patients, of whom 22 fulfilled P-aHUS criteria. We performed a functional and genetic study of the complement system in all of them. We retrospectively collected the clinical, analytical, treatment and evolution characteristics of these patients. We present the data as median and interquartile range.

Results

The age of presentation was 34 years. It was the first pregnancy in 16 patients (73%). 73% of the cases occurred postpartum. Of these, 81% required cesarean section. 41% required acute hemodialysis. 19 patients associated hypertension, 7 neurological disorders, 4 gastrointestinal problems and 3 cardiac symtoms. A renal biopsy was performed in 11 patients: 10 presented MAT lesions and 3 an associated glomerulonephritis. Abnormalities in the complement system related genes were detected in only 41% of the cohort. 16 patients underwent plasma replacement, achieving haematologic response in 5 and renal response in 2. 3 patients received infusion treatment of fresh frozen plasma, with haematological response in 3 but renal response in 2. 10 of the 22 patients (45%) received treatment with Eculizumab, achieving haematological and renal response in 100% of them. 3 of the patients treated with Eculizumab required acute hemodialysis, but none of them required renal replacement therapy during follow-up.

Conclusion

P-aHUS seems to have similar characteristics than other types of aHUS. It seems to be an association between cesarean delivery and the development of P-aHUS. Treatment with Eculizumab was effective in 100% of our cohort. More studies are needed to confirm our findings.