Case of pregnancy-related aHUS highlights urgency in diagnosis, treatment

Woman's kidney function remained lower than normal after aHUS treatment

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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Illustration of a pregnant woman.

The case of a woman who developed atypical hemolytic uremic syndrome (aHUS) following pregnancy highlights the importance of an early diagnosis and prompt treatment of the disease.

As many as 20% of aHUS cases occur in people who are pregnant or have just given birth, referred to as pregnancy-related aHUS or p-aHUS. The condition is associated with substantial risks for both the pregnant individual and the developing baby, so timely treatment is key, researchers say.

“Avoiding delay is imperative to help optimize the patient’s long-term outcomes,” researchers wrote in an article, “Complement-Mediated Thrombotic Microangiopathy in Pregnancy: An Educational Case Report,” published in the Canadian Journal of Kidney Health and Disease.

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A pregnant woman holds a teddy bear with one hand while cradling her belly with the other.

Treating pregnancy-related aHUS early important for better outcomes: Study

Pregnancy-related aHUS can resemble other complications like preeclampsia

To facilitate treatment, it’s necessary to accurately diagnose p-aHUS, but this can be difficult since p-aHUS can resemble other pregnancy complications like preeclampsia or HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome.

Here, researchers described the case of a 28-year-old who was pregnant for the first time with twins. She delivered the babies without complications, but a few hours after delivery, she started vomiting and had high blood pressure and abdominal pain. Lab tests revealed abnormally low levels of platelets and hemoglobin, as well as signs of liver and kidney damage.

The woman was admitted to intensive care, where she received a battery of diagnostic tests as well as supportive therapies, such as dialysis for her failing kidneys. Test results showed evidence of abnormal activation of the complement cascade, a group of immune proteins whose abnormal activity drives aHUS, and the woman was subsequently diagnosed with p-aHUS.

Once the diagnosis was made, and after receiving an anti-meningococcal vaccination and starting on a preventive antibiotic to reduce the risk of infection, the woman was treated with Soliris (eculizumab), an approved treatment for aHUS sold by AstraZeneca.

After she started the treatment, her lab tests improved markedly, and she was able to leave the hospital about two weeks later. A few months thereafter, she was able to stop dialysis, though researchers noted her kidney function remained lower than normal.

Clinicians plan to treat the woman with Soliris for a year, with cautious hopes to then stop the treatment and monitor for any recurrence of disease.

The researchers concluded this report highlights how a successful outcome for the management of p-aHUS “relies on multiple aspects, including a prompt diagnosis leading to an early start of the anti-complement therapy.”