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142 Clinical profile and long term outcome of childhood systemic lupus erythematosus
  1. S Sawhney,
  2. A Shivpuri and
  3. M Agarwal
  1. Sir Ganga Ram Hospital, Paediatric Rheumatology, NEW DELHI, India

Abstract

Background and aims Systematic study of all diseases is essential to understand the spectrum of the disease presentation, the severity of the disease and the outcome. There is paucity of data from India on details of paediatric SLE.

This study aims to define:

  • Describe the clinical and immunological profile of SLE within six months of disease onset in three age categories

  • To compare the performance of ACR 1997 criteria vs SLICC 2012 criteria to classify disease in first 6 months of onset

  • To define the mean value of SLEDAI at presentation and over a 5 year follow up

Methods Children attending the paediatric rheumatology clinic from January 2009 to September 2016 were included and details recorded.

Results Of 169 children, 139 (82%) females. Median age at disease onset:11.4 years(3.4–18),median age at diagnosis:12 (3.5–19).

20% had history of autoimmune disease in first degree relative.

Therapy at disease onset(first 6 months):

Hydroxychloroquine: 100%,

Glucocorticoids: 98%,

Mycophenolate: 33%,

Methotrexate: 27%,

Azathioprine: 16%,

Cyclophosphamide: 9%,

Rituximab: 2%.

At last follow up: Glucocortocoids: 37%

Abstract 142 Table 1
Abstract 142 Table 1

Age wise breakup of clinical and immunological feature at disease onset

Abstract 142 Table 2
Abstract 142 Table 2

Comparison of ACR 1997 criteria vs SLICC 2012 criteria for disease classification

Abstract 142 Table 3
Abstract 142 Table 3

SLEDAI

Median follow up 48 months(1–195 months), Mortality:4%,lost to follow up: 19%, active disease at last follow up:25%

Conclusions Patients seen at our centre had a significant disease burden with a median SLEDAI score of >20 at presentation.

Upto 1/2 of the study population did not have a malar rash. 38% had renal disease. Fever was seen in 82% and often was the cause for seeking medical opinion. This is a small data set from a tertiary level centre and not representative of the community disease.

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