Secondary systemic lupus erythematosus: an analysis of 4 cases of uncontrolled hereditary angioedema.
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.
Khan
Sujoy
S
Path Links Immunology, Scunthorpe General Hospital, Scunthorpe, North Lincolnshire, UK. Sujoy.khan@nlg.nhs.uk
Tarzi
Michael D
MD
Doré
Philip C
PC
Sewell
W A C
WA
Longhurst
Hilary J
HJ
D000293:Adolescent; D000328:Adult; D000799:Angioedema / Q000150:complications* / Q000276:immunology* / Q000503:physiopathology; D003181:Complement C4 / Q000172:deficiency*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged
D003181:Complement C4