Kidney disease caused by dysregulation of the complement alternative pathway: An etiologic approach

An S. De Vriese, Sanjeev Sethi, Jens Van Praet, Karl A. Nath, Fernando C. Fervenza

Research output: Contribution to journalReview articlepeer-review

51 Scopus citations


Kidney diseases caused by genetic or acquired dysregulation of the complement alternative pathway (AP) are traditionally classified on the basis of clinical presentation (atypical hemolytic uremic syndrome as thrombotic microangiopathy), biopsy appearance (dense deposit disease andC3GN), or clinical course (atypical postinfectiousGN). Each is characterized by an inappropriate activation of the AP, eventuating in renal damage. The clinical diversity of these disorders highlights important differences in the triggers, the sites and intensity of involvement, and the outcome of the AP dysregulation. Nevertheless, we contend that these diseases should be grouped as disorders of the AP and classified on an etiologic basis. In this review, we define different pathophysiologic categories of AP dysfunction. The precise identification of the underlying abnormality is the key to predict the response to immune suppression, plasma infusion, and complement-inhibitory drugs and the outcome after transplantation. In a patient with presumed dysregulation of the AP, the collaboration of the clinician, the renal pathologist, andthe biochemical andgenetic laboratory is verymuch encouraged, because this enables the elucidation of both the underlying pathogenesis and the optimal therapeutic approach.

Original languageEnglish (US)
Pages (from-to)2917-2929
Number of pages13
JournalJournal of the American Society of Nephrology
Issue number12
StatePublished - Dec 2015

ASJC Scopus subject areas

  • General Medicine


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