Secondary systemic lupus erythematosus: An analysis of 4 cases of uncontrolled hereditary angioedema

Abstract The association of systemic lupus erythematosus and hereditary angioedema (HAE) has formed the basis of numerous case reports and is hypothesised to result from consumption of complement C4 with consequent impaired clearance of apoptotic cells. We describe the development of frank lupus or...

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Veröffentlicht in:	Clinical Immunology 2007-04, Vol.123 (1), p.14-17
Hauptverfasser:	Khan, Sujoy, Tarzi, Michael D, Doré, Philip C, Sewell, W.A.C, Longhurst, Hilary J
Format:	Artikel
Sprache:	eng
Schlagworte:	AAE Acquired angioedema Adolescent Adult Allergic diseases Allergy and Immunology Angioedema - complications Angioedema - immunology Angioedema - physiopathology Apoptosis Biological and medical sciences Complement C4 - deficiency Complement deficiency Female Fundamental and applied biological sciences. Psychology Fundamental immunology HAE Hereditary angioedema Humans Immunopathology Lupus Erythematosus, Systemic - complications Lupus Erythematosus, Systemic - immunology Lupus Erythematosus, Systemic - physiopathology Male Medical sciences Middle Aged Skin allergic diseases. Stinging insect allergies Systemic lupus erythematosus
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Zusammenfassung:	Abstract The association of systemic lupus erythematosus and hereditary angioedema (HAE) has formed the basis of numerous case reports and is hypothesised to result from consumption of complement C4 with consequent impaired clearance of apoptotic cells. We describe the development of frank lupus or lupus-like syndrome in four HAE patients with uncontrolled angioedema and low levels of serum C4. Measures that limit hypocomplementaemia in HAE may reduce the incidence of secondary SLE.
ISSN:	1521-6616 1521-7035 1365-2567
DOI:	10.1016/j.clim.2006.09.015