Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients

Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients

Author Jesus, A. A. Google Scholar
Liphaus, B. L. Google Scholar
Silva, C. A. Google Scholar
Bando, S. Y. Google Scholar
Andrade, L. E. C. Autor UNIFESP Google Scholar
Coutinho, A. Google Scholar
Carneiro-Sampaio, M. Google Scholar
Institution Universidade de São Paulo (USP)
Universidade Federal de São Paulo (UNIFESP)
Inst Gulbenkian Ciencias
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 (<20 mg/dl) in four, IgAD (<7 mg/dl) in three, and IgMD (<35 mg/dl) in three; one of these patients presented IgA, C2 and C4D. Two patients had low C4 GCN and two had type I C2D. Demographic data, family history of autoimmune disease and PID, JSLE clinical findings, occurrence of infections, disease activity and therapies were similar in patients with and without PID (p > 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. Lupus (2011) 20, 1275-1284.
Keywords antibody deficiency
complement deficiency
C1q deficiency
IgA deficiency
juvenile systemic lupus erythematosus
primary immunodeficiency
Language English
Sponsor Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
Federico Foundation
Grant number FAPESP: 02/05880-4
FAPESP: 08/58238
CNPq: 302469-05-2
CNPq: 34802-0
Date 2011-10-01
Published in Lupus. London: Sage Publications Ltd, v. 20, n. 12, p. 1275-1284, 2011.
ISSN 0961-2033 (Sherpa/Romeo, impact factor)
Publisher Sage Publications Ltd
Extent 1275-1284
Access rights Closed access
Type Article
Web of Science ID WOS:000295689000006

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