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


Journal 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).

Full Text

Cited Authors

  • Brickman, CM; Tsokos, GC; Balow, JE; Lawley, TJ; Santaella, M; Hammer, CH; Frank, MM

Published Date

  • May 1, 1986

Published In

Volume / Issue

  • 77 / 5

Start / End Page

  • 749 - 757

PubMed ID

  • 3084606

Pubmed Central ID

  • 3084606

International Standard Serial Number (ISSN)

  • 0091-6749

Digital Object Identifier (DOI)

  • 10.1016/0091-6749(86)90424-0


  • eng

Conference Location

  • United States