Background The mainstay of treatment of Systemic JIA (sJIA) are corticosteroids(1,2), with majority being well responsive to steroids. But stopping steroids is really challenging as many relapse on tapering off or withdrawing the drug. To avoid prolonged usage and consequent unwanted side effects of steroids, many disease modifying anti rheumatic drugs (DMARDs) have been tried (1,2).Except the newer biologics, none are found to be of great help.(1,3) In the developing country like India, biologics are not easily available and if available, it is very costly. To overcome this, we have started using Cyclosporine(4), a relatively cheaper drug in the management of refractory sJIA. Here, we present a series of 16 cases of difficult sJIA successfully treated with oral Cyclosporine.
Objectives To evaluate the efficacy of Cyclosporine in Steroid refractory /dependent sJIA.
Methods Clinical records of children fulfilling the diagnostic criteria of sJIA admitted at the Institute of Child Health, Kolkata during the time period of July 2009 to December 2012 were reviewed. The data collected included details of clinical and laboratory features, treatment and outcome. Of the 72 cases of sJIA, 16 were found to be steroid dependent or frequently relapsing. 6 of these presented with steroid unresponsive macrophage Activation Syndrome(MAS) and needed Cyclosporine to control the MAS, remaining 10 was administered cyclosporine subsequently because of unresponsiveness to steroid-methotrexate regimen or persistent disease activity. The mean starting dose of Cyclosporine was 2.55mg/kg/day. After starting Cyclosporine, they were followed up regularly for clinical response and toxicity.
Results 5 patients of MAS received Cyclosporine. 3 responded dramatically and are currently off both steroid and cyclosporine.1 still require cyclosporine but off steroids, another 1 expired 11 patients of frequently relapsing, steroid dependent sJIA received Cyclosporine
i. 8 showed good response to cyclosporine. Steroid could be withdrawn completely in those 8 patients after a mean duration of 3.6 months of starting Cyclosporine and Cyclosporine was tapered off and stopped in 5 of them after a mean duration of 8 months.
ii. The remaining 3 still require steroids in very low doses to maintain remission. They are Currently both in low dose steroids and Cyclosporine.
Conclusions Cyclosporine is very cost effective drugs not only in controlling the hyperinflammatory response in MAS, but also a good add-on drug in maintaining remission in chronic, frequently relapsing steroid dependent sJIA, specially in developing countries like India.
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Disclosure of Interest None Declared