Kidney and pregnancy outcomes in pregnancy-associated atypical hemolytic uremic syndrome: A systematic review and meta-analysis.

BACKGROUND

Pregnancy-associated atypical hemolytic uremic syndrome (p-aHUS) is a rare, life-threatening condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, elevated liver enzymes, and acute kidney injury. Prompt diagnosis and therapy are crucial due to the high risk of progression to chronic kidney disease (CKD), end-stage kidney disease (ESKD), and dialysis dependency, as well as significant maternal and fetal morbidity and mortality.

METHODS

A comprehensive literature search was conducted across EMBASE, MEDLINE, and the Cochrane CENTRAL from January 2000 to March 2024. Studies reporting on pregnancy and kidney outcomes in women diagnosed with p-aHUS were included.

RESULTS

Ten studies involving 386 pregnancies in 380 patients met the inclusion criteria for the final analysis. Renal outcomes varied, with mean creatinine levels ranging from 0.72 to 8.734 mg/dL. Dialysis was required in 66.6% of patients, and 25% developed ESKD. Maternal complications included preeclampsia (36.4%) and hemolysis, elevated liver enzymes, and low platelets syndrome (29.7%), with a 5% maternal mortality rate. Fetal complications included intrauterine fetal demise (n = 25), intrauterine growth restriction, low birth weight, and prematurity. Treatment with eculizumab significantly reduced the risk of CKD and ESKD, with a pooled risk ratio of 0.20 (95% confidence interval: 0.09-0.44) and low heterogeneity (I² = 0%, P = .43).

CONCLUSION

This analysis highlights the severe kidney and pregnancy outcomes associated with p-aHUS. Eculizumab treatment is significantly beneficial in reducing the risk of CKD and ESKD.