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Cardiovascular complications in atypical haemolytic uraemic syndrome

Nature Reviews Nephrology volume 10, pages 174–180 (2014)Cite this article

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Abstract

Haemolytic uraemic syndrome (HUS) is characterized by nonimmune haemolytic anaemia, thrombocytopenia and renal impairment—most incidents in childhood are caused by shiga toxin-producing bacteria. Atypical HUS (aHUS) accounts for 10% of cases and has a poor prognosis. About 60% of patients with aHUS have dysregulation of the alternative complement pathway (complement-mediated aHUS). The kidney is the main target organ, but other organs might also be affected. Cardiac complications occur in 3–10% of patients with complement-mediated aHUS, as a consequence of microangiopathic injury in the coronary microvasculature, and can cause sudden death. Emerging evidence also suggests that either thrombosis or stenosis of the medium and large arteries might complicate disease course, and such disorders occur even after renal function is lost. In this Perspectives article we discuss the impact of cardiovascular involvement in complement-mediated aHUS, the role of acute and chronic complement hyperactivation in such events and the implications for treatment.

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Figure 1: Pathways of complement activation and regulation.
Figure 2: Potential pathophysiological mechanisms of cardiovascular complications in aHUS.

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Acknowledgements

The authors are partially supported by grants from Fondazione ART per la Ricerca sui Trapianti ONLUS, (Milan, Italy) and the European Community (FP7 Grant 2012-305,608 EURenOmics).

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  1. IRCCS—Istituto di Ricerche Farmacologiche Mario Negri, Clinical Research Centre for Rare Diseases “Aldo e Cele Daccò”, via Camozzi 3, Ranica, 24020, Bergamo, Italy

    Marina Noris

  2. Unit of Nephrology and Dialysis, Azienda Ospedaliera Papa Giovanni XXIII, Piazza OMS1, Bergamo, 24100, Italy

    Giuseppe Remuzzi

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M. Noris researched the data and wrote the article. G. Remuzzi reviewed and edited the manuscript before submission. Both authors made substantial contribution to discussion of the article content.

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Correspondence to Giuseppe Remuzzi.

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Noris, M., Remuzzi, G. Cardiovascular complications in atypical haemolytic uraemic syndrome. Nat Rev Nephrol 10, 174–180 (2014). https://doi.org/10.1038/nrneph.2013.280

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