Document Detail


Immunoregulatory disorders associated with hereditary angioedema. I. Clinical manifestations of autoimmune disease.
MedLine Citation:
PMID:  3084606     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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).
Authors:
C M Brickman; G C Tsokos; J E Balow; T J Lawley; M Santaella; C H Hammer; M M Frank
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of allergy and clinical immunology     Volume:  77     ISSN:  0091-6749     ISO Abbreviation:  J. Allergy Clin. Immunol.     Publication Date:  1986 May 
Date Detail:
Created Date:  1986-06-16     Completed Date:  1986-06-16     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  1275002     Medline TA:  J Allergy Clin Immunol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  749-57     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Angioedema / genetics,  immunology*
Autoimmune Diseases / complications,  immunology
B-Lymphocytes
Female
Genes, MHC Class II
Humans
Kidney Diseases / immunology
Lupus Erythematosus, Systemic / immunology
Male
Middle Aged
Thyroiditis / immunology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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