8/23/18 Goldstein Morning Report

PGY3 Kyndal McKnight presented an interesting case of a woman with fever, rash, joint pain. The patient also complained of myalgias and odynophagia. On exam she had salmon colored macules over her bilateral lower extremities, neck and abdomen. Labs were significant for leukocytosis, thrombocytosis, mildly elevated transaminases, elevated ESR/CRP and ferritin of 3662 with a transferrin saturation of 7%. Work-up for rheumatologic diseases including lupus, rheumatoid arthritis, inflammatory myopathies, Sjogren’s and vasculitides were all negative. A skin biopsy was performed to rule out dermatomyositis, which showed findings consistent with Still’s disease.

Adult Onset Still’s Disease
– The etiology of adult onset Still’s disease is unknown and although genetic factors and infectious triggers have been suggested but not proven.
– As there are no specific laboratory tests, the diagnosis of Still’s disease depends on a constellation of clinical findings, and there is often a delay in diagnosis.
– While Still’s disease can often be confused with other rheumatic diseases, pharyngitis does not generally occur in other rheumatologic processes.
– The Yamaguchi criteria are the most validated diagnostic criteria for adult onset Stills’ disease, of which the patient met 7 of 11 criteria. The presence of 5/9 criteria has >90% sensitivity and specificity for the diagnosis of Stills.
– Still’s disease can often be confused with acute rheumatic fever, for which the Jones criteria is used for diagnosis.
– Glucocorticoids and NSAIDs can be used for the treatment of mild disease, while glucocorticoids combined with biologic agents may be necessary for more severe disease.

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