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Delay in the Diagnosis of Adult-Onset Still’s Disease

Adult-onset Still’s disease (AOSD) is a systemic autoinflammatory disease characterized by symptoms including spiking fever, arthralgia, myalgia, maculopapular rash, and pharyngitis. The lack of diagnostic biomarker, non-specific clinical presentation, and the rarity of AOSD often result in a signif...

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Detalles Bibliográficos
Autores principales: Pak, Stella, Pham, Cindy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5501706/
https://www.ncbi.nlm.nih.gov/pubmed/28690954
http://dx.doi.org/10.7759/cureus.1321
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author Pak, Stella
Pham, Cindy
author_facet Pak, Stella
Pham, Cindy
author_sort Pak, Stella
collection PubMed
description Adult-onset Still’s disease (AOSD) is a systemic autoinflammatory disease characterized by symptoms including spiking fever, arthralgia, myalgia, maculopapular rash, and pharyngitis. The lack of diagnostic biomarker, non-specific clinical presentation, and the rarity of AOSD often result in a significant delay in diagnosis and treatment. While the average time of initial presentation to diagnosis is four months, we present a case of AOSD diagnosis three years after initial onset of classical symptoms. By reporting the case of delayed diagnosis for AOSD, we hope to raise awareness in our medical community about the diagnostic difficulty in AOSD. The present case describes an otherwise healthy male who presented with typical symptoms of AOSD, but the diagnosis of AOSD was missed during his first presentation. In the second flaring episode, the diagnosis of AOSD was established. He had an excellent therapeutic response to anakinra and prednisone during the acute flaring episode. He is currently in complete remission on methotrexate as maintenance therapy.
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spelling pubmed-55017062017-07-09 Delay in the Diagnosis of Adult-Onset Still’s Disease Pak, Stella Pham, Cindy Cureus Rheumatology Adult-onset Still’s disease (AOSD) is a systemic autoinflammatory disease characterized by symptoms including spiking fever, arthralgia, myalgia, maculopapular rash, and pharyngitis. The lack of diagnostic biomarker, non-specific clinical presentation, and the rarity of AOSD often result in a significant delay in diagnosis and treatment. While the average time of initial presentation to diagnosis is four months, we present a case of AOSD diagnosis three years after initial onset of classical symptoms. By reporting the case of delayed diagnosis for AOSD, we hope to raise awareness in our medical community about the diagnostic difficulty in AOSD. The present case describes an otherwise healthy male who presented with typical symptoms of AOSD, but the diagnosis of AOSD was missed during his first presentation. In the second flaring episode, the diagnosis of AOSD was established. He had an excellent therapeutic response to anakinra and prednisone during the acute flaring episode. He is currently in complete remission on methotrexate as maintenance therapy. Cureus 2017-06-07 /pmc/articles/PMC5501706/ /pubmed/28690954 http://dx.doi.org/10.7759/cureus.1321 Text en Copyright © 2017, Pak et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Rheumatology
Pak, Stella
Pham, Cindy
Delay in the Diagnosis of Adult-Onset Still’s Disease
title Delay in the Diagnosis of Adult-Onset Still’s Disease
title_full Delay in the Diagnosis of Adult-Onset Still’s Disease
title_fullStr Delay in the Diagnosis of Adult-Onset Still’s Disease
title_full_unstemmed Delay in the Diagnosis of Adult-Onset Still’s Disease
title_short Delay in the Diagnosis of Adult-Onset Still’s Disease
title_sort delay in the diagnosis of adult-onset still’s disease
topic Rheumatology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5501706/
https://www.ncbi.nlm.nih.gov/pubmed/28690954
http://dx.doi.org/10.7759/cureus.1321
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