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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Veröffentlicht in:	Lupus 2011-10, Vol.20 (12), p.1275-1284
Hauptverfasser:	Jesus, AA, Liphaus, BL, Silva, CA, Bando, SY, Andrade, LEC, Coutinho, A, Carneiro-Sampaio, M
Format:	Artikel
Sprache:	eng
Schlagworte:	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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Zusammenfassung:	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