Atypical hemolytic uremic syndrome and complement blockade

established and emerging uses of complement inhibition

Ramy M. Hanna, Marina Barsoum, Andrae Vandross, Ira Kurtz, Richard Burwick

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

PURPOSE OF REVIEW: Atypical hemolytic uremic syndrome (aHUS) is a diagnosis that has captured the interest of specialists across multiple fields. The hallmark features of aHUS are microangiopathic hemolysis and thrombocytopenia, which creates a diagnostic dilemma because of the occurrence of these findings in a wide variety of clinical disorders. RECENT FINDINGS: In most of the instances, aHUS is a diagnosis of exclusion after ruling out causes such as Shigella toxin, acquired or genetic a disintegrin and metalloproteinase thrombospondin motif 13 deficiency (thrombotic thrombocytopenic purpura), and vitamin B12 deficiency. In the purest sense, aHUS is a genetic condition that is activated (or unmasked) by an environmental exposure. However, it is now evident that complement activation is a feature of many diseases. Variants in complement regulatory genes predispose to microangiopathic hemolysis in many rheumatologic, oncologic, and drug-induced vascular, obstetric, peritransplant, and infectious syndromes. SUMMARY: Many 'hemolysis syndromes' overlap clinically with aHUS, and we review the literature on the treatment of these conditions with complement inhibition. New reports on the treatment of C3 glomerulopathy, Shiga toxin-related classic hemolytic uremic syndrome, and medication-related thrombotic microangiopathy will be reviewed as well.

Original languageEnglish (US)
Pages (from-to)278-287
Number of pages10
JournalCurrent opinion in nephrology and hypertension
Volume28
Issue number3
DOIs
StatePublished - May 1 2019
Externally publishedYes

Fingerprint

Hemolysis
Shiga Toxin
Thrombotic Microangiopathies
Thrombospondins
Disintegrins
Vitamin B 12 Deficiency
Thrombotic Thrombocytopenic Purpura
Hemolytic-Uremic Syndrome
Complement Activation
Environmental Exposure
Metalloproteases
Regulator Genes
Thrombocytopenia
Obstetrics
Blood Vessels
Atypical Hemolytic Uremic Syndrome
Therapeutics
Pharmaceutical Preparations

ASJC Scopus subject areas

  • Internal Medicine
  • Nephrology

Cite this

Atypical hemolytic uremic syndrome and complement blockade : established and emerging uses of complement inhibition. / Hanna, Ramy M.; Barsoum, Marina; Vandross, Andrae; Kurtz, Ira; Burwick, Richard.

In: Current opinion in nephrology and hypertension, Vol. 28, No. 3, 01.05.2019, p. 278-287.

Research output: Contribution to journalArticle

Hanna, Ramy M. ; Barsoum, Marina ; Vandross, Andrae ; Kurtz, Ira ; Burwick, Richard. / Atypical hemolytic uremic syndrome and complement blockade : established and emerging uses of complement inhibition. In: Current opinion in nephrology and hypertension. 2019 ; Vol. 28, No. 3. pp. 278-287.
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