Acquired C1 esterase inhibitor deficiency

Svetomir N. Markovic, David J. Inwards, Evangelos A. Frigas, Robert P. Phyliky

Research output: Contribution to journalReview articlepeer-review

110 Scopus citations


Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. In this paper, we summarize experience with 22 cases of acquired C1 esterase inhibitor deficiency in the context of a review of the published literature on diagnosis and treatment of this condition.

Original languageEnglish (US)
Pages (from-to)144-150
Number of pages7
JournalAnnals of internal medicine
Issue number2
StatePublished - Jan 18 2000

ASJC Scopus subject areas

  • Internal Medicine


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