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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%
Age wise breakup of clinical and immunological feature at disease onset
Comparison of ACR 1997 criteria vs SLICC 2012 criteria for disease classification
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.