Atypical hemolytic-uremic syndrome (aHUS) driven by anti-factor H (FH) antibodies is a distinct sub-type that constitutes a major proportion of pediatric aHUS cases, predominantly affecting children in the 5–15 year age range. Prompt recognition and antibody-targeted management are central to outcome.

Clinical Scenario

Atypical HUS with confirmed anti-factor H (FH) antibodies in a child aged 5–15 years. Anti-FH antibody-associated illness represents approximately 50% of pediatric aHUS cases in this age group, making the presence of these antibodies a defining feature of the clinical sub-type addressed by this protocol.

Treatment Approach

Management centres on the prompt initiation of plasma exchange together with immunosuppressive therapy. The complete treatment algorithm — including agent selection, sequencing, and monitoring milestones — is set out in the full protocol.

Treatment Goals

The primary targets are a substantial reduction in anti-factor H antibody titers and the achievement of hematological remission, assessed against defined thresholds for platelet count, schistocyte burden, and LDH. The full protocol specifies how each goal is measured and when response is confirmed.

References

DOI: 10.1007/s00467-019-04233-7

Anti-FH antibody-associated illness constitutes ~ 50% of pediatric patients with aHUS in India, chiefly affecting children between the age of 5–15 years.
We recommend a combination of prompt PEX (with fresh frozen plasma as replacement fluid) and immunosuppressive therapy for patients with anti-FH antibodies.
We suggest daily PEX until hematological remission and then taper over 3–5 weeks.
Since antibodies are chiefly IgG, 5–7 PEX achieve 80% reduction in antibody titers.
Hematological remission: Platelets > 100,000/μl, schistocytes < 2%, LDH less than upper limit of normal on 2 consecutive days.

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Treatment of Hemolytic-uremic Syndrome with Anti-factor H Antibodies in Children Aged 5–15

Clinical Scenario

Treatment Approach

Treatment Goals

References