Systemic Juvenile Idiopathic Arthritis

The ACR recommendations suggest that there is a role for a trial of NSAIDs for up to two weeks if disease activity is relatively low and poor prognostic features are absent. Patients with high disease activity (such as symptomatic pericarditis) are recommended to receive systemic corticosteroids and if they prove unresponsive, anakinra is recommended. However, considering the available evidence and expert opinion, the recommendations do recognize that anakinra could be used as a first-line treatment for active systemic symptoms of sJIA, particularly if there is persistent fever and if poor prognostic features are present.

In view of the more recent data alluded to above, one might also consider tocilizumab as an alternative to IL-1 inhibitors. The ACR recommendations for children who have active arthritis but no systemic features include NSAIDS, followed by methotrexate. If there is no response to methotrexate, the recommendations suggest that patients should receive either anakinra or a tumor necrosis factor–α inhibitor. The potential role of IL-1 and IL-6 inhibitors for arthritis without systemic features remains to be clarified.

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The patient was treated with anakinra 100mg (2 mg/kg) daily by subcutaneous injection. There was a dramatic improvement in his clinical and laboratory features after less than one week of treatment. Similar improvements might be anticipated with the use of high-dose systemic corticosteroids or tocilizumab.

Although clinical trials have addressed the use of anakinra or tocilizumab in patients who have not responded adequately to systemic corticosteroids, there is increasing use of these agents in patients with active systemic features and arthritis, even prior to receiving corticosteroids. The role of these agents in corticosteroid-naive patients requires further study. The results of a proposed prospective study of the comparative effectiveness of these agents in early sJIA may help to elucidate the most appropriate use of each of these agents. In the interim, systemic corticosteroids still have an important role in treating acute severe manifestations of the disease and severe complications such as symptomatic pericarditis and in treating MAS.

Disclosure

Dr. Schneider is a consultant for Hoffmann-La Roche.

Dr. Schneider is associate chair (education) in the division of rheumatology and associate professor in the department of paediatrics and a rheumatologist in the division of rheumatology at the University of Toronto and The Hospital for Sick Children in Ontario, Canada. Dr. Laxer is a rheumatologist in the division of rheumatology at The Hospital for Sick Children and professor of paediatrics and medicine at the University of Toronto.

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