New Treatment for Adult-Onset Still’s Disease Studied

Still’s Disease is a form of arthritis that is characterized by high spiking fevers and transient salmon-colored rash. Still’s disease was first described in children, but it is now known to occur, much less commonly, in adults (in whom it is referred to as adult-onset Still’s disease). There is commonly swelling of the lymph glands, enlargement of the spleen and liver, and sore throat. Some patients develop inflammation of the lungs (pleuritis) or around the heart (pericarditis) with occasional fluid accumulation around the lungs (pleural effusion) or heart (pericardial effusion). Although the arthritis may initially be overlooked because of the impressive nature of the systemic symptoms, everyone with Still’s Disease eventually develops joint pain and swelling. This usually involves many joints (polyarticular arthritis). Any joint can be affected, although there are preferential patterns of joint involvement in Still’s Disease.

There is no known cause or cure for Still’s Disease but there is research into treatment options. One such study was reported in the January, 2011 issue of Arthritis Care & Research.

This was a small cohort study which included only 14 patients with Still’s Disease. The study participants had failed all available therapies, which in some cases included three TNF inhibitors, abatacept, and rituximab. Eight of the patient’s had suffered irreversible joint damage as a result of their arthritis. Recurrent systemic symptoms were present in seven of the patients.

The 14 study participants were treated with the anti-interleukin (IL)-6 receptor antibody tocilizumab (Actermra). The dosages varied in the study, but the most common was the recommended dose for rheumatoid arthritis – 8mg/kg every 4 weeks.

The average disease activity score in 28 joints (DAS28) decreased from 5.61 at the beginning of the study to 3.21 after three months and decreased further, to 2.91 after six months.

Based on the European League Against Rheumatism (EULAR), a good response to treatment requires a DAS28 score or 3.2 or lower. 64% of the patients achieved this level. In addition, EULAR describes remission as a DAS28 score below 2.6, which was achieved by 57% of the patients.

Concurrent with the treatment with tocilizumab the patient’s average dosage of prednisone fell from 23.3 mg/day and the beginning of the study to 13 mg/day after three months and to 10.3 mg/day after six months.

A 60% improvement was seen in number of tender and swollen joints and in patient global health rating at six months and systemic symptoms resolved in 86% by three months.

The study was conducted by Xavier Puéchal, MD, PhD, of Le Mans General Hospital in Le Mans, France, and colleagues.

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