Low levels of C3, CH50, AH50, and complement factor B (CFB) along with elevated levels of C5a, C5b-9, Bb, anti-CFH (anti-complement factor H) autoantibodies, and d-dimer would identify atypical hemolytic uremic syndrome (aHUS), if a comprehensive complement profile were constructed using data generated herein.

• This is a systematic review on the aHUS biomarkers: C3, C5a, C5b-9, factor B, complement factor B, H, and I, CH50, AH50, d-dimer, as well as anti-CFH antibodies.

• This analysis involved 60 studies consisting of 837 aHUS patients, with at least one biomarker reported.

• Relative to the reference range, the biomarkers C3, CH50, AH50 and CFB were identified to be lower among aHUS persons.

• In those with aHUS, the biomarkers including C4, C4d, CFH and complement factor I were within the reference range.

• Patients with aHUS exhibited higher levels of biomarkers C5a, C5b-9, Bb and d-dimer than the reference range.