Unmask aHUS Clinical presentation Complement-amplifying conditions
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Learn how to unmask aHUS

Atypical Hemolytic Uremic Syndrome (aHUS) is a chronic and life-threatening disease1-4

In aHUS, uncontrolled complement activation leads to systemic thrombotic microangiopathy (TMA), causing progressive damage to vital organs and premature death.2,4-7

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Icon Brains

CNS

48% of patients experience neurological symptoms8

  • Confusion9

  • Stroke9

  • Encephalopathy5

  • Seizure8

Icon Lungs

Pulmonary

  • Dyspnea6

  • Pulmonary hemorrhage10

  • Pulmonary edema6

Icon Heart

Cardiovascular

43% of patients experience cardiovascular symptoms8

  • Myocardial infarction6

  • Hypertension11

  • Diffuse vasculopathy2

  • Peripheral gangrene12

Icon Liver

Hepatic

  • Thrombocytopenia6

  • Decreased hemoglobin13

  • Elevated lactate dehydrogenase (LDH)13

  • Decreased haptoglobin13

  • Schistocytes13

Icon Kidney

Renal

More than 50% of patients of patients progress to ESRD13

  • Elevated creatinine14

  • Edema,15 malignant hypertension5

  • Proteinuria8

  • Decreased estimated glomerular filtration rate (eGFR)2

ESRD=End-Stage Renal Disease
Icon Intestines

Gastrointestinal

Up to 30% of patients experience diarrhea16

  • Colitis9

  • Abdominal pain9

  • Pancreatitis17

  • Nausea/Vomiting17

  • Gastroenteritis5

  • Liver necrosis2

79% of all aHUS patients die, require dialysis, or have permanent renal damage within 3 years.5

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Learn about symptoms

TMAs often share the same clinical presentations but differ in the underlying cause18-21

aHUS can look like TTP (due to neurological dysfunction) and STEC-HUS (due to diarrhea)8,16

Watch this short video to see how presentations can overlap

Differential diagnosis for primary TMAs (including ADAMTS13)22

View diagnostic algorithm Download PDF
TTP=Thrombotic thrombocytopenic purpura; STEC-HUS=Shiga-like toxin-producing E. coli; ADAMTS13, A Disintegrin and Metalloproteinase with a ThromboSpondin type 1 motif, member 13;

When standard of care fails to resolve a TMA associated with a complement-amplifying condition, consider aHUS5,13,22-27

69% of aHUS patients presented with their first clinical manifestation while experiencing a complement-amplifying condition5

Watch this short video to learn about the complement-amplifying conditions associated with aHUS

Patient approach to a TMA associated with a complement-amplifying condition22

View diagnostic algorithm Download PDF

References

1. Legendre CM, et al. N Engl J Med. 2013;368(23):2169-2181. 2. Loirat C, et al. Pediatr Nephrol. 2008;23(11):1957-1972. 3. Hirt-Minkowski P, et al. Nephron Clin Pract. 2010;114:219-235. 4. Fang CJ, et al. Br J Haematol. 2008;143(3):336-348. 5. Noris M, Caprioli J, Bresin E, et al. Clin J Am Soc Nephrol. 2010;5:1844-1859. 6. Sallee M, Daniel L, Piercecchi MD, et al. Nephrol Dial Transplant. 2010;25:2028-2032. 7. Loirat C, Garnier A, Sellier-Leclerc AL, et al. Semin Thromb Hemost. 2010;36:673-681. 8. Neuhaus TJ, Calonder S, Leumann EP. Arch Dis Child. 1997;76:518-521. 9. Ohanian M, Cable C, Halka K. Clin Pharmacol. 2011;3:5-12. 10. Sellier-Leclerc AL, Frémeaux-Bacchi V, Dragon-Durey M-A, et al. J Am Soc Nephrol. 2007;18:2392-2400. 11. Kavanagh D, Goodship THJ, Richards A. Br Med Bull. 2006;77-78:5-22. 12. Malina M, Gulati A, Majid M, et al. Pediatr Nephrol. 2011;26:1678. 13. Caprioli J, Noris M, Brioschi S, et al. Blood. 2006;108:1267-1279. 14. Ariceta G, Besbas N, Johnson S, et al. Pediatr Nephrol. 2009;24:687-696. 15. Stahl A-L, Vaziri-Sani F, Heinen S, et al. Blood. 2008;111:5307-5315. 16. Zuber J, Le Quintrec M, Sberro-Soussan R, et al. Nat Rev Nephrol. 2011;7:23-35. 17. Dragon-Durey M-A, Sethi SK, Bagga A, et al. J Am Soc Nephrol. 2010;21:2180-2187. 18. Sadler JE, Moake JL, Miyata T, et al. Hematology Am Soc Hematol Educ Program. 2004;407-423. 19. Zheng XL. Blood. 2010;115:1475-1476. 20. Moake JL. N Engl J Med. 2002;347:589-600. 21. Tsai H-M. J Am Soc Nephrol. 2003;14:1072-1081. 22. Laurence J, et al. Clin Adv Hematol Oncol. 2016;14(11):1-16. 23. Fakhouri F, et al. J Am Soc Nephrol. 2010;21(5):859-867. 24. Song D, et al. Arthritis Res Ther. 2013;15(1):R12. 25. Zhao J, et al. PLoS Genetics. 2011;7(5):e1002079. 26. Totina A, et al. Clin Pediatr (Phila). 2013;52(2):183-186. 27. Kavanagh D, et al. Br Med Bull. 2006;77-78:5-22.
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