Pregnancy-associated hemolytic uremic syndrome.

Area of Science:

• Nephrology
• Hematology
• Obstetrics

Background:

• Pregnancy-associated hemolytic uremic syndrome (P-aHUS) is a critical obstetric emergency.
• It carries significant risks of mortality and morbidity, with over 50% progressing to end-stage renal disease if untreated.
• Most cases manifest postpartum, often mimicking other thrombotic microangiopathies.

Purpose of the Study:

• To review the pathophysiology, diagnosis, and management of P-aHUS.
• To highlight the importance of early recognition and intervention.

Main Methods:

• Review of literature on P-aHUS.
• Discussion of diagnostic criteria including genetic analysis and antibody testing.
• Emphasis on current treatment modalities.

Main Results:

• P-aHUS involves complement alternative pathway dysregulation, damaging microvascular endothelium.
• Differential diagnosis includes severe pre-eclampsia, HELLP syndrome, TTP, and AFLP.
• Genetic testing and anti-CFH antibodies aid diagnosis, though 40% lack known mutations.

Conclusions:

• Supportive care and plasmapheresis are foundational treatments for P-aHUS.
• Eculizumab is a safe and effective therapy for inducing and maintaining remission in P-aHUS.
• Early diagnosis and initiation of eculizumab are recommended upon establishing the diagnosis.