Acquired C1 esterase inhibitor deficiency and angioedema: a review.


Journal Article

A case of acquired C1INH deficiency with angioedema is described. Fifteen cases are thus far recorded. The clinical syndrome of angioedema in these patients closely resembles hereditary angioedema (HAE). Most cases are associated with a paraprotein, cryoglobulin, or autoantibody, which presumably initiates C1 activation and C1 Inhibitor consumption. C1INH, C4 and C2 levels are low in acquired C1INH deficiency, as in HAE. A distinguishing feature is that C1 titers are very low in the acquired disease and only minimally depressed, if at all, in HAE. Most cases have appeared in patients with an underlying lymphoproliferative or autoimmune disease. Therapy is directed at the underlying disorder, but androgen therapy may be helpful in preventing attacks. Future potential therapeutic approaches are discussed.

Full Text

Cited Authors

  • Gelfand, JA; Boss, GR; Conley, CL; Reinhart, R; Frank, MM

Published Date

  • July 1, 1979

Published In

Volume / Issue

  • 58 / 4

Start / End Page

  • 321 - 328

PubMed ID

  • 449665

Pubmed Central ID

  • 449665

International Standard Serial Number (ISSN)

  • 0025-7974

Digital Object Identifier (DOI)

  • 10.1097/00005792-197907000-00004


  • eng

Conference Location

  • United States