C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction

Ravneet Bajwa, John A. Depalma, Taimoor Khan, Anmol Cheema, Sheila A. Kalathil, Mohammad A. Hossain, Attiya Haroon, Anne Madhurima, Min Zheng, Ali Nayer, Arif Asif

Research output: Contribution to journalArticle

Abstract

The advances in our understanding of the alternative pathway have emphasized that uncontrolled hyperactivity of this pathway causes 2 distinct disorders that adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome (aHUS), renal dysfunction occurs along with thrombocytopenia, anemia, and target organ injury to multiple organs, most commonly the kidney. On the other hand, in the so-termed C3 glomerulopathy, kidney involvement is not associated with thrombocytopenia, anemia, or other system involvement. In this report, we present 2 cases of alternative pathway dysfunction. The 60-year-old female patient had biopsy-proven C3 glomerulopathy, while the 32-year-old female patient was diagnosed with aHUS based on renal dysfunction, thrombocytopenia, anemia, and normal ADAMTS-13 level. The aHUS patient was successfully treated with the monoclonal antibody (eculizumab) for complement blockade. The patient with C3 glomerulopathy did not receive the monoclonal antibody. In this patient, management focused on blood pressure and proteinuria control with an angiotensin-converting enzyme inhibitor. This article focuses on the clinical differences, pathophysiology, and treatment of aHUS and C3 glomerulopathy.

Original languageEnglish (US)
Pages (from-to)25-34
Number of pages10
JournalCase Reports in Nephrology and Dialysis
Volume8
Issue number1
DOIs
StatePublished - Jan 31 2018
Externally publishedYes

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Keywords

  • Atypical hemolytic uremic syndrome
  • C3 glomerulopathy
  • Complement dysfunction

ASJC Scopus subject areas

  • Nephrology

Cite this

Bajwa, R., Depalma, J. A., Khan, T., Cheema, A., Kalathil, S. A., Hossain, M. A., Haroon, A., Madhurima, A., Zheng, M., Nayer, A., & Asif, A. (2018). C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction. Case Reports in Nephrology and Dialysis, 8(1), 25-34. https://doi.org/10.1159/000486848