Skip to content

Advertisement

  • Poster presentation
  • Open Access

Course, outcome and complications in a single centre cohort of 53 indian children with systemic onset juvenile idiopathic arthritis with a minimum follow up of 3 years

  • 1,
  • 2 and
  • 1
Pediatric Rheumatology201412 (Suppl 1) :P65

https://doi.org/10.1186/1546-0096-12-S1-P65

  • Published:

Keywords

  • Juvenile Idiopathic Arthritis
  • Thalidomide
  • Hepatic Encephalopathy
  • Leflunomide
  • Triamcinolone Acetonide

Introduction

Systemic-onset Juvenile Idiopathic Arthritis (SOJIA) is not rare in India, where biotherapies are unaffordable.Data on its course, outcome and complications are scarce.

Objectives

We describe this data in a monocentric cohort of 53 patients, followed up for at least 3 years.

Methods

A pre-biologic era Italian study where one of the authors (RC) had participated, formed a template and comparator. After ethics committee approvals and consents, a cohort of 53 consecutive patients diagnosed with SOJIA before 10-2009 using the ILAR criteria were followed up until 09-2012. At each visit, general (including growth parameters) and articular examination, laboratory parameters (CBC, ESR , liver enymes) and ongoing treatment were entered in a customized database.Course was classified as monocyclic (single episode) polycyclic (multiple episodes with remissions in between) and persistent (continuous articular/systemic disease activity). At last visit, outcome was studied with respect to remission (Wallace criteria) and Steinbrocker functional classification. Juvenile Arthritis Damage Index (JADI) measured on 20/53 patients.

Results

In the 53 patients studied (35-M,18-F), 21 constituted an inceptional cohort and 32 were referred to our center with prior diagnosis / treatment. Mean age at diagnosis was 6.3 years (range 4m-14y), mean follow up period was 5.5 years (range 3-10 years) and mean time from onset of symptoms to diagnosis was 8.5 months (range 2 wks-7 years). Forty four patients received NSAIDs, 52 oral corticosteroids and 34 required pulses of methylprednisolone with intra-articular triamcinolone acetonide being used in 14. Methotrexate was used in 50 patients, other DMARDs in 25(hydroxychloroquine, leflunomide, cyclosporine, cyclophosphamide, thalidomide) and 5 received biologics briefly (etanercept-2, tocilizumab-3). Nine had a monocyclic, 31 intermittent and 13 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. Patients diagnosed within 6 months from disease onset were more likely to have a monocyclic / intermittent than a persistent course, compared to those diagnosed later. 33/53 suffered from complications of the disease and /or drug. MAS was observed in 5 and death occurred in 1, due to hepatic encephalopathy complicating viral hepatitis A.Three required orthopedic surgeries for residual deformities. All children in the monocyclic group belonged to Steinbrocker class 1 at last visit. Of 31 in the intermittent group, 27 belonged to class 1 and 4 to class 2. In 13 of the persistent group, 7 belonged to class 1, 4 to class 2 and 2 to class 3. JADI was performed on 20/53 patients.9 had significant articular damage. The range of JADI-Articular was 0-25/72 (median-6) and the range of JADI- Extra-articular was 0-4/17 (median-1).

Conclusion

Drug regimens comprising NSAIDs, steroids (oral/intra-articular),methotrexate and other DMARDs still form the mainstay of SOJIA treatment in India in contrast with the developed world where biologics have come to the forefront. As a result 33/53(65%) patients showed drug and/or disease-related complications. Delay in diagnosis is a major problem and is associated with a persistent course. Children with monocyclic and intermittent course have the best functional outcome. JADI is an easily applicable bedside tool to evaluate both articular and extra articular damage of SOJIA.

Disclosure of interest

None declared.

Authors’ Affiliations

(1)
Pediatrics, Jaslok Hospital & Research Center, Mumbai, India
(2)
Pediatrics, AOU Meyer, Florence, Italy

Copyright

© Dewoolkar et al; licensee BioMed Central Ltd. 2014

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 (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Advertisement