Immunoregulatory disorders associated with hereditary angioedema. I. Clinical manifestations of autoimmune disease.

  • M C
  • Tsokos G
  • Balow J
 et al. 
  • 1


    Mendeley users who have this article in their library.
  • N/A


    Citations of this article.


Occasional reports have appeared linking hereditary angioedema (HAE) with autoimmune diseases. We have systematically evaluated 157 patients for manifestations of autoimmunity. Nineteen of these patients (12%) had clinical immunoregulatory diseases including glomerulonephritis (five patients), Sjögren's syndrome (three), inflammatory bowel disease (three), thyroiditis (two), systemic lupus erythematosus (one), drug-induced lupus (one), rheumatoid arthritis (one), juvenile rheumatoid arthritis with IgA deficiency (one), incipient pernicious anemia (one), and sicca syndrome (one). All eight patients with HAE who developed an autoimmune disease with a known human histocompatibility antigen association developed a disease associated with their histocompatibility antigen haplotype (p = 0.014). Although only four patients developed Sjögren's syndrome or sicca syndrome, an additional nine manifested part of the sicca complex. We also found patients with HAE with features suggestive of an immune-based abnormality. These features included idiopathic pancreatitis (three patients), Raynaud's disease (two), partial lipodystrophy (one), chronic chorioretinitis (one), and alopecia universalis (one).

Author-supplied keywords

  • Adolescent
  • Adult
  • Angioedema
  • Angioedema: genetics
  • Angioedema: immunology
  • Autoimmune Diseases
  • Autoimmune Diseases: complications
  • Autoimmune Diseases: immunology
  • B-Lymphocytes
  • Female
  • Genes
  • Humans
  • Kidney Diseases
  • Kidney Diseases: immunology
  • Lupus Erythematosus
  • MHC Class II
  • Male
  • Middle Aged
  • Systemic
  • Systemic: immunology
  • Thyroiditis
  • Thyroiditis: immunology

Get free article suggestions today

Mendeley saves you time finding and organizing research

Sign up here
Already have an account ?Sign in

Find this document


  • C M

  • G C Tsokos

  • J E Balow

  • T J Lawley

  • M Santaella

  • C H Hammer

Cite this document

Choose a citation style from the tabs below

Save time finding and organizing research with Mendeley

Sign up for free