Abstract
Primary manifestation of an autoinflammatory systemic disease was found in a 15-year-old patient, which initially ran a fulminant course. The course was first controlled by therapy with steroids and cyclosporin A. In the course of the disease, the patient developed a therapy refractory polyarthritis, which failed to respond to a combination of disease-modifying antirheumatic drugs (DMARDs) and tumor necrosis factor (TNF) inhibitors. A good disease control could only be achieved with an interleukin 6 (IL-6) blockade and DMARDs.
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