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Course, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis

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Abstract

Objectives

To assess the course, outcome and complications in a mono-centric cohort of 53 patients with systemic onset juvenile idiopathic arthritis (s-JIA).

Methods

In an observational study, 53 consecutive patients diagnosed with s-JIA on or before October 2009 were enrolled and followed up between October 2009 and September 2012. At each 6–12 weekly visit, clinical examination, laboratory investigations and details of on-going treatment were recorded. Disease course was classified as monocyclic, intermittent and persistent. At last visit, outcome was studied with respect to remission (Wallace criteria) and Steinbrocker functional classification. Juvenile Arthritis Damage Index (JADI) was measured on a subset.

Results

In 53 patients analysed, the mean follow-up period was 5.5 ± 1.85 y, with a cumulative follow-up period of 291.5 patient-years. The mean age at diagnosis was 6.3 ± 3.4 y. Thirty-three patients suffered from disease and/or drug related complications. Infections were observed in 16 (30%) and macrophage activation syndrome in 5 (9.4%). Nine (17%) had a monocyclic course, 31 (58.5%) had an intermittent course and 13 (24.5%), a persistent course. At last visit, 9/9 patients of the monocyclic group, 17/31 in the intermittent group and 3/13 in the persistent group were in remission. At the end of the study, 96.2% of the index patients were Steinbrocker functional class I and II with the monocyclic group having the best functional outcome. JADI was performed on 20/53 patients. Nine had significant articular damage. The range of Juvenile arthritis damage index-articular (JADI-A) was 0–25/72 (median-6) and of Juvenile arthritis damage index-extra articular (JADI-EA) was 0–4/17 (median-1).

Conclusions

The outcome of patients with s-JIA in a resource limited setting where early diagnosis, multidisciplinary care and availability of biologics are hurdles, is further altered by complications related to longstanding disease and over use of steroids.

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Contributions

MD: Conceptualized and designed the study, collected data, carried out initial analysis and drafted the initial manuscript; RC: Advised on the conceptualization of the study and provided a critical review of the manuscript; PRC: Involved in further analysis and writing of the final manuscript; RPK: Conceptualized and designed the study, provided the patients for the study, was also involved in data collection and analysis and guided MD during the course of this study. The final manuscript was approved by all the authors. RPK will act as guarantor for the paper.

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Authors and Affiliations

  1. Department of Pediatrics, Pediatric Rheumatology Clinic, Jaslok Hospital and Research Centre, Mumbai, 400026, India

    Mansi Dewoolkar, Pranav Raman Chickermane & Raju P. Khubchandani

  2. Department of Pediatric Rheumatology, AOU Meyer, Florence, Italy

    Rolando Cimaz

Authors
  1. Mansi Dewoolkar
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  2. Rolando Cimaz
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  3. Pranav Raman Chickermane
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  4. Raju P. Khubchandani
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Correspondence to Raju P. Khubchandani.

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Dewoolkar, M., Cimaz, R., Chickermane, P.R. et al. Course, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis. Indian J Pediatr 84, 294–298 (2017). https://doi.org/10.1007/s12098-017-2293-5

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  • DOI: https://doi.org/10.1007/s12098-017-2293-5

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