Absence of Arthritis as a Sign of Sine Syndrome in Still’s Disease in Adulthood

The authors of this chapter discuss the atypical course of Still’s disease in adulthood. It is a sine syndrome (skin changes, itchy, linear urticarial rash or other skin manifestations, periorbital edema and erythema of eyelids; organ manifestations were not present in the patient) with arthritis – in the form of monoarthritis of the right knee – that occurred after 23 years of the disease. The most pronounced feature was five attacks of fever associated with chills and high inflammatory activity. Therapy with corticosteroids, cyclosporine and methotrexate suppressed and reduced relapses of the disease. An attempt to discontinue basal therapy led to the last relapse of the disease. Differential diagnostic procedures for nosographic demarcation of Still’s disease in adulthood associated with sine syndrome are discussed, as well as the options for future treatment with biologics.