Arthritis & Rheumatism, Volume 60,
October 2009 Abstract Supplement
The 2009 ACR/ARHP Annual Scientific Meeting
Philadelphia October 16-21, 2009.
Juvenile Systemic Lupus Erythematosus Frequently Develops in a Background of Primary Immunodeficiencies
Jesus1, Adriana A., Liphaus1, Bernadete, Silva1, Clovis A., Andrade2, Luis Eduardo C., Coutinho3, Antonio, Carneiro-Sampaio1, Magda
To evaluate the frequency of complement and antibody primary immunodeficiencies (PIDs) in Juvenile Systemic Lupus Erythematosus (JSLE) patients and to compare lupus patients with and without PID regarding demographic data, clinical features, disease activity and damage, treatment and occurrence of severe sepsis.
Seventy-two JSLE (ACR criteria) patients (1 to 16 yrs at diagnosis) were analyzed for early components of the classical complement pathway (C1q, C1r/C1s, C4, C2, C3) and immunoglobulin levels (IgG, IgA, IgM, IgE, and IgG2 subclass). Anti-C1q antibody was detected by ELISA (Inova Diagnostics - QUANTA Lite Anti-C1q, San Diego, USA). Statistical analysis was carried out according to Fisher's exact test, Mann-Whitney test and Backward Stepwise multivariate analysis.
Nineteen patients (26%) had underlying PIDs. Complement deficiencies were detected in 9 JSLE patients: C2 deficiency in 5 cases, C4 deficiency in 2 (all with persistently very low values in the presence of normal levels of other complement components, and SLEDAI < 4) and complete C1q deficiency in 2. All patients had normal C3 levels. Immunoglobulin deficiencies were found in 10 JSLE patients: IgG2 deficiency (<20mg%) in 4, IgA deficiency (<7mg%) in 3, IgM deficiency (<35mg%) in 3. All patients with immunoglobulin deficiencies were identified after 10-years of age and had normal or high total IgG levels. One IgA deficient patient also presented C4 and C2 deficiencies. The frequency of male gender was significantly higher in JSLE patients with PID compared to those without PID (37% vs. 11%, p=0.032). The 2 cases of infantile SLE (age at onset <2 years) were both males (one with C1q deficiency and other with IgM deficiency). A remarkably higher frequency of severe sepsis was observed in the PIDs group (31% vs. 7.5%; p=0.017). Lupus clinical features (cutaneous, mucosal, neuropsychiatric, cardiopulmonary, renal, hematological and articular manifestations and antiphospholipid syndrome) were comparable in patients with and without PID (p>0.05). SLEDAI score and anti-C1q antibody levels were also alike. On the other hand, the median of the cumulative damage (SLICC/ACR-DI) was significantly higher in PIDs group [1(0 5) vs 0 (0 3); p=0.0075]. Additionally, 44% of JSLE patients (30/68) had high IgE levels (>100UI/ml). Multivariate analysis revealed that male gender (Odds ratio=4.7; CI=1.2 19.2; p=0.034) and SLICC/ACR-DI (Odds ratio=2.5; IC=1.13 4.8; p=0.007) were the independent risk factors for PID.
An exceedingly high frequency of antibody and complement deficiency was observed amongst JSLE patients, suggesting that these immunologic defects may contribute to the disease development. Our results command that these two groups of PIDs should be systematically investigated particularly in males with disease damage.
To cite this abstract, please use the following information:
Jesus, Adriana A., Liphaus, Bernadete, Silva, Clovis A., Andrade, Luis Eduardo C., Coutinho, Antonio, Carneiro-Sampaio, Magda; Juvenile Systemic Lupus Erythematosus Frequently Develops in a Background of Primary Immunodeficiencies [abstract]. Arthritis Rheum 2009;60 Suppl 10 :1319