The phenotype of SLE associated with complete deficiency of complement isotype C4A.
J Clin Immunol
; 18(1): 48-51, 1998 Jan.
Article
en En
| MEDLINE
| ID: mdl-9475353
ABSTRACT
Complete deficiency of the complement C4A isotype is a known genetic risk factor for systemic lupus erythematosus (SLE). The disease phenotype of C4A-deficient patients has never been defined. Among 200 patients with SLE from five centers, 18 (9%) with C4A deficiency were identified. These individuals were compared to those who were C4A replete with regard to a series of clinical and serologic features. The only significant differences between the two groups were in the presence of renal disease (C4A deficient, 11%; C4A replete, 46%; P < 0.006) and a decrease in the serum concentrations of C3 (C4A deficient, 11%; C4A replete, 35%; P < 0.04). There was also a trend for the C4A-deficient individuals to have milder disease. In light of the tendency for C4A-deficient individuals to have lower serum concentrations of C4, it is important that such patients not be subjected to overly aggressive efforts to "normalize" their C4 levels.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Complemento C4a
/
Lupus Eritematoso Sistémico
Tipo de estudio:
Clinical_trials
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Adult
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
J Clin Immunol
Año:
1998
Tipo del documento:
Article
País de afiliación:
Estados Unidos