Coincidence of hereditary angioedema (HAE) with Crohn's disease

Immunol Invest. 1999 Jan;28(1):43-53. doi: 10.3109/08820139909022722.

Abstract

A patient with two diseases, based presumably on different immunopathological mechanisms, hereditary angioedema (HAE) and Crohn's disease, was followed for 8 years. For more than three years of this observation period, detailed laboratory data were also available and could be analyzed. Both diseases had severe courses requiring chronic treatment with danazol and sulfasalazine, respectively. During exacerbation of Crohn's disease, the levels of C4 was found to be significantly lower than during the periods free of symptoms of both diseases. This drop was probably due to an impaired C1-inhibitor activity. HAE attacks and acute exacerbation of Crohn's disease never occurred simultaneously. This finding may be a mere chance but may also indicate that the different immunopathological processes underlying HAE and Crohn's disease influence each other.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Angioedema / complications*
  • Angioedema / immunology
  • Angioedema / pathology
  • Complement C1 Inactivator Proteins / deficiency
  • Complement C4 / analysis
  • Complement Pathway, Classical
  • Crohn Disease / complications*
  • Crohn Disease / immunology
  • Crohn Disease / pathology
  • Humans
  • Longitudinal Studies
  • Male

Substances

  • Complement C1 Inactivator Proteins
  • Complement C4