Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Correspondence
  • Published:

Malignant hypertension and atypical hemolytic uremic syndrome: a possible continuum between two entities?

Journal of Human Hypertension volume 36, pages 498–499 (2022)Cite this article

Subjects

Malignant hypertension (MHT) is a known cause of secondary thrombotic microangiopathy (TMA) and can resemble a case of atypical uremic hemolytic syndrome (aHUS) [1, 2]. The differential diagnosis between these entities is essential given the need of a different therapeutic approach: strict blood pressure (BP) control versus treatment with a C5 inhibitor and/or plasmapheresis [2,3,4]. Recently some authors proposed that patients with TMA secondary to MHT with evidence of active complement component may benefit from a C5 inhibitor, such as eculizumab [5].

This case highlights the difficulty in the differential diagnosis between MHT and aHUS, as well as the possible continuum between the two entities. In this case, TMA appears as the common denominator [6]. The diagnosis of MHT was considered in our patient due to the BP values and the fundoscopy findings [1]. In MHT the extreme increase in intravascular pressure leads to fibrinoid necrosis and thrombotic occlusion of the arterioles, promoting platelet consumption and erythrocyte fragmentation, thus being able to trigger a clinic TMA process [7,8,9]. In this patient hematological recovery was quickly accomplished but not accompanied by complete recovery of renal function. C3 consumption was also sustained which led to the performance of a renal biopsy and genetic screening in order to exclude a diagnosis of aHUS.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Fig. 1: H&E staining, 20×.

References

  1. Cremer A, Amraoui F, Lip GYH, Morales E, Rubin S, Segura J, et al. From malignant hypertension to hypertension-MOD: a modern definition for an old but still dangerous emergency. J Hum Hypertens. 2016;30:463–6.

    Article  CAS  Google Scholar 

  2. Karpman D, Loos S, Tati R, Arvidsson I. Haemolytic uraemic syndrome. J Intern Med. 2017;281:123–48.

    Article  Google Scholar 

  3. Bayer G, Von Tokarski F, Thoreau B, Bauvois A, Barbet C, Cloarec S, et al. Etiology and outcomes of thrombotic microangiopathies. Clin J Am Soc Nephrol. 2019;14:557–66.

    Article  Google Scholar 

  4. Timmermans SAMEG, Werion A, Damoiseaux JGMC, Morelle J, Reutelingsperger CP, van Paassen P. Diagnostic and risk factors for complement defects in hypertensive emergency and thrombotic microangiopathy. Hypertension. 2020;75:422–30.

  5. Palma LMP, Sridharan M, Sethi S. Complement in secondary thrombotic microangiopathy. Kidney Int Rep. 2021;6:11–23. [Internet] Available from: https://doi.org/10.1016/j.ekir.2020.10.009.

    Article  PubMed  Google Scholar 

  6. Timmermans SAMEG, Abdul-Hamid MA, Potjewijd J, Theunissen ROMFIH, Damoiseaux JGMC, Reutelingsperger CP, et al. C5b9 formation on endothelial cells reflects complement defects among patients with renal thrombotic microangiopathy and severe hypertension. J Am Soc Nephrol. 2018;29:2234–43.

    Article  CAS  Google Scholar 

  7. Timmermans SAMEG, Abdul-Hamid MA, Vanderlocht J, Damoiseaux JGMC, Reutelingsperger CP, van Paassen P, et al. Patients with hypertension-associated thrombotic microangiopathy may present with complement abnormalities. Kidney Int. 2017;91:1420–5. [Internet] Available from: https://doi.org/10.1016/j.kint.2016.12.009.

    Article  PubMed  Google Scholar 

  8. Cavero T, Arjona E, Soto K, Caravaca-Fontán F, Rabasco C, Bravo L, et al. Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome. Kidney Int. 2019;96:995–1004.

    Article  CAS  Google Scholar 

  9. Demeulenaere M, Devreese K, Vanbelleghem H, De Zaeytijd J, Vande Walle J, Van Biesen W, et al. Thrombomodulin and endothelial dysfunction: a disease-modifier shared between malignant hypertension and atypical hemolytic uremic syndrome. Nephron. 2018;140:63–73.

    Article  CAS  Google Scholar 

  10. Gavriilaki E, Brodsky RA. Complementopathies and precision medicine. J Clin Invest. 2020;130:2152–63.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Nephrology Department, Hospital Professor Doctor Fernando Fonseca, Amadora, Portugal

    Ana Catarina Brás, Afonso Sepulveda Santos, Anna Lima, Miguel Verdelho Costa, Patrícia S. Carrilho & Bruno G. Rodrigues

Authors
  1. Ana Catarina Brás
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Afonso Sepulveda Santos
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Anna Lima
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Miguel Verdelho Costa
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Patrícia S. Carrilho
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Bruno G. Rodrigues
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

ACB and BGR concept and drafted the article; ACB, ASS, and AL revised the paper; MVC, PSC, and BGR made the critical revision of the article; all authors approved the final version of the paper.

Corresponding author

Correspondence to Ana Catarina Brás.

Ethics declarations

Competing interests

The authors declare no competing interests.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Brás, A.C., Santos, A.S., Lima, A. et al. Malignant hypertension and atypical hemolytic uremic syndrome: a possible continuum between two entities?. J Hum Hypertens 36, 498–499 (2022). https://doi.org/10.1038/s41371-021-00651-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/s41371-021-00651-z

Search

Advanced search

Quick links