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Organ-specific autoantibodies and autoimmune diseases in juvenile systemic lupus erythematosus and juvenile dermatomyositis patients


The purpose of this study was to evaluate organ-specific autoantibodies and autoimmune diseases in JSLE and JDM patients.


Forty-one JSLE and 41 JDM patients were investigated for serum autoantibodies associated with autoimmune hepatitis, primary biliary cirrhosis, type 1 diabetes mellitus (T1DM), autoimmune thyroiditis, autoimmune gastritis and celiac disease. Patients with positive organ-specific antibodies were assessed for the presence of the respective organ-specific autoimmune diseases.


Mean age at diagnosis was significantly higher in JSLE compared to JDM patients (10.3±3.4 vs. 7.3±3.1years, p=0.0001), whereas the mean disease duration was similar in both groups. The frequencies of organ-specific autoantibodies were similar between JSLE and JDM (p>0.05). Of note, a high prevalence of autoantibodies related to DM1 and autoimmune thyroiditis was observed in both groups (20% vs. 15%, p=0.77 and 24% vs. 15%, p=0.41; respectively). Higher frequencies of antinuclear antibody - ANA (93% vs. 59%, p=0.0006), anti-dsDNA (61% vs. 2%, p<0.0001), anti-Ro (35% vs. 0%, p<0.0001), anti-Sm (p=0.01), anti-RNP (p=0.02), anti-La (p=0.03) and IgG aCL (p=0.001) were observed in JSLE compared to JDM patients. Organ-specific autoimmune diseases were evidenced only in JSLE patients (24% vs. 0%, p=0.13). Two JSLE patients had T1DM associated with Hashimoto thyroiditis and another had subclinical thyroiditis. Other JSLE patient had celiac disease diagnosis based on iron deficiency anaemia, presence of anti-endomysial antibody, duodenal biopsy compatible to celiac disease and response to a gluten-free diet.


Organ-specific diseases were observed solely in JSLE patients and required specific therapy. The presence of these antibodies recommends the evaluation of organ-specific diseases and a rigorous follow-up of these patients.


Nadia E. Aikawa: None; Adriana A. Jesus: None; Bernadete L. Liphaus: None; Bernadete L. Liphaus: None; Clovis A. Silva: None; Magda C. Sampaio: None; Adriana M. Sallum: None.

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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Aikawa, N.E., Jesus, A.A., Liphaus, B.L. et al. Organ-specific autoantibodies and autoimmune diseases in juvenile systemic lupus erythematosus and juvenile dermatomyositis patients. Pediatr Rheumatol 10 (Suppl 1), A14 (2012).

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