Woman, 33, with lupus developed aHUS after pregnancy in rare case
'Delayed recognition' of condition prevented use of correct treatment: Report
A woman with lupus in her early 30s, who developed atypical hemolytic uremic syndrome (aHUS) after pregnancy, was not suspected of having the rare condition until five days after her baby’s birth, which resulted in her not receiving the correct treatment, according to a case report from researchers in Japan.
“In this case, delayed recognition of aHUS precluded the use of [appropriate] therapies,” the researchers wrote.
According to the team, “this case highlights the importance for clinicians to consider the possibility of aHUS in postpartum patients with severe renal [kidney] dysfunction and [other] symptoms, even if the patient has an underlying [lupus], as early diagnosis and treatment of aHUS is necessary to improve maternal outcomes.”
The woman was ultimately given prednisolone, a corticosteroid, which helped ease her symptoms, but she remained hospitalized for about a month after undergoing an emergency cesarean section.
The study, “The Critical Importance of Diagnosing Atypical Hemolytic Uremic Syndrome in Postpartum Renal Dysfunction in a Patient With Systemic Lupus Erythematosus: A Case Report and Comprehensive Review,” was published in the journal Cureus.
Woman diagnosed with lupus in 20s, had first pregnancy in 30s
aHUS is a rare disease characterized by anemia driven by the destruction of red blood cells, low platelet counts, and organ damage. In some cases, the condition can arise after pregnancy and childbirth — and is then known as postpartum aHUS.
Postpartum aHUS requires prompt diagnosis to manage appropriately, case studies have shown. The disease also can appear in people with underlying autoimmune diseases, such as lupus.
Here, scientists from Shiga University of Medical Science in Otsu described a challenging case of a 33-year-old woman with lupus who developed aHUS after giving birth.
The woman had been diagnosed with lupus in her early 20s and had been managing the disease using standard treatments that included the antimalarial agent hydroxychloroquine and the immunosuppressant tacrolimus.
At the age of 33, she became pregnant for the first time.
Toward the start of the third trimester of pregnancy, at about 28 weeks, her blood pressure started to increase. A few days later, she lost consciousness at home and was brought to the hospital, where tests revealed abnormal kidney function. The symptoms were indicative of preeclampsia — a pregnancy condition marked by high blood pressure and organ damage — and eclampsia, when seizures develop following preeclampsia.
Due to her severe condition, doctors performed an emergency surgery to deliver the infant, who was brought to a neonatal intensive care unit, or NICU, for further care.
Shortly after the baby was born, the woman’s urine output decreased to the point that she was no longer urinating. She then started having difficulty breathing.
Two days after the baby was delivered, she was moved to an intensive care unit and put on mechanical ventilation to help her breathe. She was also placed on continuous hemodiafiltration, a form of kidney replacement therapy, and given infusions of red blood cells and platelets.
Doctors first thought case was episode of lupus worsening
Doctors initially suspected the woman might be experiencing an episode of lupus worsening, but lab tests ruled that out. Tests for other autoimmune diseases and for the bacteria that causes typical hemolytic uremic syndrome also came back negative.
Five days after giving birth, lab tests revealed signs of red blood cell destruction. This prompted doctors to stop tacrolimus and switch her to prednisolone instead. The patient’s condition gradually improved from there, and she was eventually able to be taken off ventilation and hemodiafiltration.
The woman was discharged from the hospital about a month after giving birth. At the time of discharge, she was still taking prednisolone and hydroxychloroquine, as well as medications to lower blood pressure.
Although rare, aHUS should be considered in SLE [lupus] pregnancies presenting with severe renal [kidney] dysfunction. … Early recognition and appropriate treatment of aHUS are essential to improving the long-term prognosis of patients.
Kidney dysfunction can be a sign of preeclampsia, but kidney dysfunction related to preeclampsia almost always resolves after birth. That, together with lab tests showing red blood cell destruction and low platelet counts, as well as the patient’s breathing difficulties, led doctors to suspect that she might have been experiencing postpartum aHUS.
“This case represents a rare instance of pregnancy complicated by [lupus] that progressed to preeclampsia, eclampsia, and subsequently aHUS,” the researchers wrote.
People with aHUS often have underlying genetic mutations that set the stage for the disease to develop. Genetic testing did not identify any of these mutations in this woman. Nonetheless, based on her clinical experience, clinicians diagnosed her with postpartum aHUS.
The scientists speculated that, if the diagnosis of aHUS had been made earlier, the patient might have received more effective treatment allowing her to have a shorter hospital stay.
“Early recognition and appropriate treatment of aHUS are essential to improving the long-term prognosis of patients,” the scientists wrote. “Although rare, aHUS should be considered in SLE pregnancies presenting with severe renal dysfunction.”
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