Scoring Adult Onset Still's Disease
Editorial Scoring Adult Onset Still’s Disease Adult-onset Still’s disease (AOSD) is a systemic inflamma- patient may be counterproductive as initial treatment; simi- tory disease of uncertain etiology. Patients with AOSD larly, antiinfective treatment of an AOSD patient may delay develop a combination of several disease manifestations. immunosuppression. Thus, differentiating these 2 diseases Some of these disease manifestations are arthritis, fever, is important. leukocytosis, and evanescent rash. But, in parallel, various Besides the above mentioned AOSD classification and systemic manifestations such as splenomegaly and pneu- diagnostic criteria, Pouchot, et al published an activity monitis, among others, may occur. score with a sensitivity of 92% and a specificity of 93% for The diagnosis of AOSD is problematic, because no single discriminating active and non-active AOSD12. And, in this diagnostic test or characteristic histopathology exists. Patients issue of The Journal, Rau, et al13 used the same Pouchot sometimes suffer from delays in diagnosis including protract- score12 in a changed version, adding arthritis and serum fer- ed efforts to exclude occult infection or neoplasm because ritin > 3000 µg/l as new factors instead of abdominal pain AOSD is a rare diagnosis, and differential diagnoses must be and splenomegaly. The other factors of the Pouchot score excluded. The clinician should consider AOSD in the evalua- comprised fever, evanescent rash, pharyngitis, myalgia, tion of undiagnosed fever of unknown origin, particularly if pleuritis, pericarditis, pneumonia, lymphadenopathy, present in association with rheumatic complaints1. hepatomegaly or elevated liver enzymes, and leukocyte More than 99% of patients with AOSD manifest with fever count > 15,000/µl.
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