Abstract: PUB221
Eculizumab for Treatment of Recurrent Pregnancy-Triggered Atypical Hemolytic-Uremic Syndrome with a Mutation in Complement 3: A Case Report
Session Information
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Miyabe, Yoei, Tokyo Joshi Ika Daigaku, Shinjuku-ku, Tokyo, Japan
- Nakai, Anna, Tokyo Joshi Ika Daigaku, Shinjuku-ku, Tokyo, Japan
- Watanabe, Kentaro, Tokyo Joshi Ika Daigaku, Shinjuku-ku, Tokyo, Japan
- Karasawa, Kazunori, Tokyo Joshi Ika Daigaku, Shinjuku-ku, Tokyo, Japan
- Kato, Noritoshi, Nagoya Daigaku Daigakuin Igakukei Kenkyuka Igakubu, Nagoya, Aichi, Japan
- Maruyama, Shoichi, Nagoya Daigaku Daigakuin Igakukei Kenkyuka Igakubu, Nagoya, Aichi, Japan
- Moriyama, Takahito, Tokyo Joshi Ika Daigaku, Shinjuku-ku, Tokyo, Japan
- Nitta, Kosaku, Tokyo Joshi Ika Daigaku, Shinjuku-ku, Tokyo, Japan
Group or Team Name
- Tokyo Women's Medical University Department of Medicine Division of Nephrology, Nagoya University Graduate School of Medicine Department of Nephrology
Introduction
In atypical hemolytic-uremic syndrome (aHUS), thrombotic microangiopathy (TMA) often develops due to mutations in complement-related genes or autoantibodies to complement regulators. Anti-C5 monoclonal antibodies (eculizumab and ravulizumab) are expected to improve prognosis. However, the significance of genetic testing is unknown. We report a case of pregnancy-triggered aHUS that was successful with plasmapheresis and eculizumab administration.
Case Description
A 37-year-old Japanese female who had a twin pregnancy underwent a scheduled cesarean section at 37 weeks gestation. On the second day after her delivery, she developed thrombocytopenia, hemolytic anemia, and renal dysfunction. TMA was suspected, and plasma exchange (PE) was started on the 3rd day after her delivery, and steroid pulse was started on the 4th day, and then prednisolone 60 mg/day was administered. Since Shiga toxin-producing Escherichia coli in her stool was negative and both ADAMTS13 activity and inhibitor were normal, the patient was clinically diagnosed with aHUS. Her clinical findings tended to improve, so PE was interrupted after eight times; however, aHUS relapsed due to a urinary tract infection 15 days after her delivery. We decided that eculizumab was necessary because of the relapse of aHUS due to the infection even under high doses of steroids. Eculizumab was administered 22 days after her delivery, and her clinical findings improved. After that, the pathogenic variant C3 p.Ile1157Thr was identified by genetic testing. Since aHUS due to C3 gene mutation has been reported to have a high recurrence rate, eculizumab administration was continued every two weeks.
Discussion
When thrombotic thrombocytopenic purpura (TTP) and aHUS are suspected in patients with TMA, PE should be started as soon as possible, and eculizumab administration should be considered when aHUS is diagnosed by ADAMTS13 testing. In the case of aHUS, genetic testing is essential because it enables the definitive diagnosis and the prediction of prognosis.