Departments of Paediatrics and 1 Infectious Diseases, Leiden University Medical Centre, The Netherlands
Correspondence to: R. G. M. Bredius, Department of Paediatrics, Leiden University Medical Centre, Mailbox 9600, 2300 RC Leiden, The Netherlands. E-mail: r.g.m.bredius{at}lumc.nl
SIR, Systemic juvenile idiopathic arthritis (sJIA), Still's disease, is a disease of unknown aetiology that is characterized by uncontrolled inflammation and results in high spiking fever, rash, serositis and polyarthritis. Multiple immunomodulatory strategies are used for disease control. Here we report for the first time the successful treatment of therapy-refractory sJIA with atorvastatin, which was chosen because of its anti-inflammatory properties and its cholesterol-reducing actions [1; for review see reference 2].
A boy aged 2 yr was diagnosed as having sJIA. After a varicella infection he had full remission for 4 yr. At the age of 6 his disease relapsed following a mild respiratory infection (Fig. 1). He did not respond to steroids, subcutaneous methotrexate or cyclosporin, but developed osteoporosis and radiological joint destruction. At the age of 9 yr autologous stem cell transplantation was performed using high-dose cyclophosphamide, antithymocyte globulin and low-dose total body irradiation. Although initially he showed remission of his arthritis, he relapsed after 7 weeks. The boy became steroid-dependent again, and despite intra-articular steroid injections his polyarthritis remained present. Anti-TNF- treatment with infliximab and etanercept was stopped because of lack of effect. Thalidomide was introduced as an immune-modulating agent, but was discontinued after only 2 weeks because of acute renal failure. Finally, the patient was treated with atorvastatin (starting dose 10 mg per day), which was gradually increased to 30 mg per day (1.5 mg/kg/day). Before the start of atorvastatin treatment, the cholesterol level was always within the normal range (3.06.0 mmol/l), whereas during treatment it decreased below normal (2.42.8 mmol/l). At a dose of 30 mg, the arthritis, functional status and all other clinical and laboratory manifestations improved dramatically (Fig. 1). Most importantly, before the start of treatment he was wheelchair-dependent but now he is a normal and fully active child. In addition, the steroids could be tapered and were stopped completely. He had no documented adverse events.
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The patient was started on atorvastatin after we had obtained written informed consent from both parents and the patient. In addition, written permission was given for publication of this case. The medical ethics committee of the Leiden University Medical Centre allowed the treatment of this patient with atorvastatin.
The authors have declared no conflict of interest.
Supplementary data are available at
Rheumatology Online.
References