Background Adult Onset Still's disease (AOSD) is an inflammatory disorder with an expansive and heterogeneous presentation that can often be mistaken for infectious or malignant etiology. The diagnosis is largely based on clinical manifestations with corroborating laboratory features, such as elevated acute inflammatory markers, ferritin, and leukocytosis. The long-term management of AOSD can be difficult due to the limited amount of reliable biomarkers used to predict and manage disease activity.
Objectives We present two cases with the diagnosis of AOSD and the use of Vectra DA to track clinical progression and response to therapy wit a goal to assess clinical utility of MBDA pannel Vectra DA in the management of Adult Onset Still's Disease
Results Case One: 68-year-old male with history of hypothyroidism was evaluated for fever of unknown origin associated with generalized malaise, fatigue, and a maculopapular rash in bilateral upper extremities for 3 weeks. The patient was pan-CT scanned with results only remarkable for bladder thickening. He denied any familial history of rheumatological disorders, and was found to have negative ANA and RF. Further infectious and malignant work-up inpatient was negative. Patient was found to have ferritin level of 15,599 ng/mL, ESR 82 mm/hr, and CRP 18 mg/dL; and was diagnosed with Adult-Onset Still's Disease (AOSD). Vectra DA score was 77 at time of diagnosis. He was started on pulsed steroid and then discharged home to complete a tapered steroid regimen with the addition of Rilonacept with a good response; repeat Vectra DA score at three-month follow-up was 15. Delta of Vectra correlated with normalized CRP <0.9 and ESR 6.
Case Two: 25 year-old female with history of JIA that achieved remission for the past 14 years presented with recurrent fevers, sore throat, generalized malaise, arthralgias, and a new-onset maculopapular rash of the inner forearms and thighs bilaterally for one-month duration. Her blood work revealed WBC 20.8 k/uL but further infectious work-up was negative. She was found to have negative RF, ANA but elevated ferritin level of 321 mg/mL, ESR 75 mm/hr, CRP 138 mg/dL. Patient was diagnosed with AOSD, and started on pulsed oral steroid with methotrexate but failed treatment. Her Vectra DA score was 72 at the peak of her symptoms. Eventually received pulsed IV corticosteroids and started on Rilonasept with symptomatic improvement. A repeat Vectra DA score at three-month follow-up was 17. Delta of Vectra correlated with normalized CRP <0.1 and ESR 2.
Conclusions Vectra DA is a 12-biomarker disease activity pannel commonly employed in the management of rheumatoid arthritis (RA). Vectra DA is not routinely employed in the management of AOSD and, here, we have illustrated two examples demonstrating the clinical utility of Vectra DA as an assessment tool in the diagnosis and management of AOSD. As such, this should prompt to reconsider the value of Vectra DA as a diagnostic tool in AOSD and other inflammatory disorders where there are limited biomarkers utilized to reliably track disease progression.
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Segurado OG, Sasso EH. Vectra DA for the objective measurement of disease activity in patients with rheumatoid arthritis. Clin Exp Rheumatol. 2014 Sep-Oct;32:S-29–34.
Disclosure of Interest None declared