Treatment of hemolytic uremic syndrome related to Bordetella pertussis infection —is plasma exchange or eculizumab use necessary?

• Main Authors: Ken Saida, Masao Ogura, Yuji Kano, Shingo Ishimori, Takahisa Yoshikawa, Hiroko Nagata, Mai Sato, Koichi Kamei, Kenji Ishikura
• Format: Article
• Language: English
• Published: BMC 2018-12-01
• Series: BMC Nephrology
• Subjects: Atypical hemolytic uremic syndrome; Bordetella pertussis; Eculizumab; Plasma exchange
• Online Access: http://link.springer.com/article/10.1186/s12882-018-1168-y

Abstract Background Bordetella pertussis infection is a known trigger of atypical hemolytic uremic syndrome (HUS). For patients suspected of having atypical HUS, prompt plasma exchange/infusion (PE/PI) or eculizumab (ECZ) treatment is recommended. Case presentation We report a 1-month-old female infant who was admitted with a severe cough and a B. pertussis-positive sputum culture. She was born at 38 weeks gestation and did not have a family history of renal diseases. Hemophagocytic syndrome was suspected and she was transferred to our hospital 17 days after her initial admission. One day later, she developed acute kidney injury and was diagnosed with HUS triggered by B. pertussis infection. Her plasma complement levels were low and her kidney function continued to worsen over the next few days. However, prior to starting ECZ treatment, her kidney function improved spontaneously; she did not receive PE/PI or ECZ. She was discharged 46 days after her initial hospitalization, without complications. A genetic workup revealed no mutations in CFH, CFI, CFB, C3, MCP, THBD, or DGKE. Conclusions This case demonstrates that B. pertussis infection-related HUS may resolve spontaneously. The decision to treat during the acute phase is challenging because B. pertussis often affects infants suspected of having atypical HUS. However, ECZ may not be the first treatment option for patients with B. pertussis infection-related HUS unless they show an indicated genetic abnormality; if ECZ is used, early discontinuation should be considered.