patients with early-onset systemic juvenile idiopathic arthritis show more inflammation and worse...

ORAL PRESENTATION Open Access Patients with early-onset systemic juvenile idiopathic arthritis show more inflammation and worse outcome R Russo * , M Katsicas From 18th Pediatric Rheumatology European Society (PReS) Congress Bruges, Belgium. 14-18 September 2011 Background Systemic Juvenile Idiopathic Arthritis (SJIA) is heteroge- neous in severity, course and outcome. Predictive factors for a poor outcome include persistent systemic features (fever, thrombocytosis) and younger age at onset. Aim To describe and analyze the disease features in patients with SJIA with very early onset and to compare them with those of patients with later onset. Methods Retrospective analysis of clinical data. Early-onset (EO) was defined as the start of SJIA prior to age 18 months. Variables included: demographic and clinical features at onset and outcome variables during disease course (pat- tern of course, presence of clinical joint damage [using the Juvenile Arthritis Damage Index or JADI], radiographic joint damage [erosions], destructive hip disease, disability [CHAQ > 0.5], growth retardation, develop- ment of macrophage activation syndrome [MAS], need for biologic agents, and death). Patients with EO were compared with patients with disease onset at age ≥ 18 months. Chi square and Mann-Whitney tests were used for comparisons. Results 192 patients (115 girls, 23 EO) followed between 1995 and 2010 were included. Age at onset was 12 (2-17) months in patients with EO, 72 (18-191) months in patients with non-EO. Delay in diagnosis (2 months) and duration of follow-up (10 vs 8 years) were similar in both groups. Eight patients (1 EO, 7 non-EO) died during the observation period. Patients with EO showed more frequently serositis (p=0.0003), hepatomegaly (p=0.01), splenomegaly (p=0.03), lower number of active joints (p=0.01), Hgb (p=0.02), and higher plt (p=0.04) at onset; more frequently MAS (p=0.0001), therapy with biologics (p=0.01), destructive hip disease (p=0.04), radiographic damage (p=0.01), growth retardation (p=0.05), disability (p=0.01), and higher JADI score (p=0.003). Conclusions Patients with SJIA starting before age 18 months show more systemic inflammatory features, and a poorer outcome than children with later disease onset. They may represent a distinct subset. This observation should prompt rheumatologists to initiate early aggressive therapy and close follow-up in this age group. Published: 14 September 2011 doi:10.1186/1546-0096-9-S1-O18 Cite this article as: Russo and Katsicas: Patients with early-onset systemic juvenile idiopathic arthritis show more inflammation and worse outcome. Pediatric Rheumatology 2011 9(Suppl 1):O18. * Correspondence: [email protected] Service of Immunology & Rheumatology, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina Russo and Katsicas Pediatric Rheumatology 2011, 9(Suppl 1):O18 http://www.ped-rheum.com/content/9/S1/O18 © 2011 Russo and Katsicas; licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Page 1: Patients with early-onset systemic juvenile idiopathic arthritis show more inflammation and worse outcome

ORAL PRESENTATION Open Access

Patients with early-onset systemic juvenileidiopathic arthritis show more inflammation andworse outcomeR Russo*, M Katsicas

From 18th Pediatric Rheumatology European Society (PReS) CongressBruges, Belgium. 14-18 September 2011

BackgroundSystemic Juvenile Idiopathic Arthritis (SJIA) is heteroge-neous in severity, course and outcome. Predictive factorsfor a poor outcome include persistent systemic features(fever, thrombocytosis) and younger age at onset.

AimTo describe and analyze the disease features in patientswith SJIA with very early onset and to compare themwith those of patients with later onset.

MethodsRetrospective analysis of clinical data. Early-onset (EO)was defined as the start of SJIA prior to age 18 months.Variables included: demographic and clinical features atonset and outcome variables during disease course (pat-tern of course, presence of clinical joint damage [usingthe Juvenile Arthritis Damage Index or JADI], radio-graphic joint damage [erosions], destructive hip disease,disability [CHAQ > 0.5], growth retardation, develop-ment of macrophage activation syndrome [MAS], needfor biologic agents, and death). Patients with EO werecompared with patients with disease onset at age ≥ 18months. Chi square and Mann-Whitney tests were usedfor comparisons.

Results192 patients (115 girls, 23 EO) followed between 1995and 2010 were included. Age at onset was 12 (2-17)months in patients with EO, 72 (18-191) months inpatients with non-EO. Delay in diagnosis (2 months)and duration of follow-up (10 vs 8 years) were similar in

both groups. Eight patients (1 EO, 7 non-EO) died dur-ing the observation period. Patients with EO showedmore frequently serositis (p=0.0003), hepatomegaly(p=0.01), splenomegaly (p=0.03), lower number of activejoints (p=0.01), Hgb (p=0.02), and higher plt (p=0.04) atonset; more frequently MAS (p=0.0001), therapy withbiologics (p=0.01), destructive hip disease (p=0.04),radiographic damage (p=0.01), growth retardation(p=0.05), disability (p=0.01), and higher JADI score(p=0.003).

ConclusionsPatients with SJIA starting before age 18 months showmore systemic inflammatory features, and a poorer out-come than children with later disease onset. They mayrepresent a distinct subset. This observation shouldprompt rheumatologists to initiate early aggressive ther-apy and close follow-up in this age group.

Published: 14 September 2011

doi:10.1186/1546-0096-9-S1-O18Cite this article as: Russo and Katsicas: Patients with early-onsetsystemic juvenile idiopathic arthritis show more inflammation andworse outcome. Pediatric Rheumatology 2011 9(Suppl 1):O18.

* Correspondence: [email protected] of Immunology & Rheumatology, Hospital de Pediatría Prof. Dr. JuanP. Garrahan, Buenos Aires, Argentina

Russo and Katsicas Pediatric Rheumatology 2011, 9(Suppl 1):O18http://www.ped-rheum.com/content/9/S1/O18

© 2011 Russo and Katsicas; licensee BioMed Central Ltd. This is an open access article distributed under the terms of the CreativeCommons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, andreproduction in any medium, provided the original work is properly cited.

mailto:[email protected]

http://creativecommons.org/licenses/by/2.0

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