Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients

Objective: To evaluate the frequency of primary immunodeficiencies (PID) in juvenile systemic lupus erythematosus (JSLE) patients. Methods: Some 72 JSLE patients were analyzed for levels of immunoglobulin classes and IgG subclasses and early components of the classical complement pathway. Determinat...

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Bibliographic Details
Published in:Lupus 2011-10, Vol.20 (12), p.1275-1284
Main Authors: Jesus, AA, Liphaus, BL, Silva, CA, Bando, SY, Andrade, LEC, Coutinho, A, Carneiro-Sampaio, M
Format: Article
Language:English
Subjects:
Adolescent
Antibodies
Antibodies, Antinuclear - blood
Autoantibodies - blood
Autoimmune diseases
Base Sequence
Binding sites
Child
Child, Preschool
Complement C1q - antagonists & inhibitors
Complement C1q - deficiency
Complement C1q - immunology
Complement C2 - deficiency
Complement C2 - genetics
Complement C4 - deficiency
Complement C4 - genetics
Complement System Proteins - deficiency
Complement System Proteins - genetics
Disease
DNA Primers - genetics
Family medical history
Female
Gene Dosage
Humans
Immune system
Immunoglobulins
Immunoglobulins - blood
Immunoglobulins - classification
Immunologic Deficiency Syndromes - complications
Immunologic Deficiency Syndromes - genetics
Immunologic Deficiency Syndromes - immunology
Infant
Infections
Lupus
Lupus Erythematosus, Systemic - complications
Lupus Erythematosus, Systemic - genetics
Lupus Erythematosus, Systemic - immunology
Male
Medical records
Mutation
Pediatrics
Sepsis
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Description
Summary:Objective: To evaluate the frequency of primary immunodeficiencies (PID) in juvenile systemic lupus erythematosus (JSLE) patients. Methods: Some 72 JSLE patients were analyzed for levels of immunoglobulin classes and IgG subclasses and early components of the classical complement pathway. Determination of C4 gene copy number (GCN) and detection of type I C2 deficiency (D) were also performed. Results: PID was identified in 16 patients (22%): C2D in three, C4D in three, C1qD in two, IgG2D (
ISSN:0961-2033
1477-0962
DOI:10.1177/0961203311411598