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Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients
- Source :
- Lupus. 20:1275-1284
- Publication Year :
- 2011
- Publisher :
- SAGE Publications, 2011.
-
Abstract
- 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 ( 0.05). Remarkably, the median of Systemic Lupus International Collaborating Clinics/ACR-damage index (SLICC/ACR-DI) was significantly higher in JSLE patients with PID compared with patients without these abnormalities ( p = 0.0033), likewise the high frequency of SLICC/ACR-DI > 1 ( p = 0.023). Conclusions: A high frequency of PID was observed in JSLE patients, suggesting that these defects may contribute to lupus development. Our findings indicate that these two groups of PID should be investigated in severe pediatric lupus.
- Subjects :
- Male
Adolescent
Gene Dosage
Immunoglobulins
Classical complement pathway
Rheumatology
immune system diseases
medicine
Humans
Lupus Erythematosus, Systemic
Juvenile
Copy-number variation
Child
skin and connective tissue diseases
Autoantibodies
DNA Primers
Base Sequence
biology
business.industry
Complement C1q
Immunologic Deficiency Syndromes
Infant
Complement C4
Complement System Proteins
Complement C2
Complement deficiency
medicine.disease
Immunoglobulin Classes
Antibodies, Antinuclear
Child, Preschool
Immunology
biology.protein
Primary immunodeficiency
Female
Antibody
business
Anti-SSA/Ro autoantibodies
Subjects
Details
- ISSN :
- 14770962 and 09612033
- Volume :
- 20
- Database :
- OpenAIRE
- Journal :
- Lupus
- Accession number :
- edsair.doi.dedup.....fea0a1f88bb552f2192e93aa46341758