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Blockade of the Terminal Complement Cascade Using Ravulizumab in a Pediatric Patient With Anti-complement Factor H
Xiaoyan Wu1, Amanda Szarzanowicz2, Adinoyi Garba3
1Nephrology, University at Buffalo, Buffalo, USA.
Cureus
|December 16, 2021
Summary
A rare pediatric disease, atypical hemolytic uremic syndrome (aHUS), was successfully treated in an 8-year-old girl using eculizumab and then ravulizumab, leading to full recovery. This case highlights the importance of early diagnosis and complement cascade inhibition for favorable outcomes.
Area of Science:
- Nephrology
- Immunology
- Pediatrics
Background:
- Atypical hemolytic uremic syndrome (aHUS) is a rare pediatric condition, with 6-10% of cases linked to complement factor H autoantibodies.
- Ravulizumab offers long-term management by blocking the terminal complement cascade.
Observation:
- An 8-year-old female presented with hemolytic anemia, thrombocytopenia, and acute kidney injury, initially suggestive of aHUS.
- Genetic testing revealed specific haplotypes, and further tests confirmed decreased factor H and elevated factor H autoantibodies, indicating anti-factor H antibody-associated aHUS.
Findings:
- Prompt treatment with eculizumab led to complete renal recovery within one month and resolution of hematological abnormalities within two months.
- Eculizumab was safely switched to ravulizumab after five months, and complement levels normalized within 12 months, although autoantibodies persisted.
Implications:
- Timely recognition and treatment of anti-factor H antibody-associated aHUS in children are crucial for favorable outcomes.
- Early therapy targeting the complement cascade, such as with eculizumab, shows promise for pediatric aHUS cases.
- Switching from eculizumab to ravulizumab is a viable option for sustained treatment in aHUS management.
Keywords:
acute kidney injuryatypical hemolytic uremic syndromecomplement c3eculizumabfactor hfactor h autoantibodyhemolytic anemialactate dehydrogenase (ldh)proteinuriaravulizumabMore Related Videos
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