Adult-onset still's disease

Authors

  • John J. Cush MD,

    1. Fellow in Rheumatology, University of Texas Health Science Center at Dallas
    2. Departments of Medicine and Radiology, University of Pittsburgh School of Medicine, Pittsburgh, PA and the Department of Medicine, Coney Island Hospital, Coney Island, NY
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  • Thomas A. Medsger Jr. MD,

    Corresponding author
    1. Professor of Medicine and Chief, Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine
    2. Departments of Medicine and Radiology, University of Pittsburgh School of Medicine, Pittsburgh, PA and the Department of Medicine, Coney Island Hospital, Coney Island, NY
    • Department of Medicine, 985 Scaife Hall, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261
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  • Wallace C. Christy MD,

    1. Clinical Associate Professor of Medicine, University of Pittsburgh School of Medicine
    2. Departments of Medicine and Radiology, University of Pittsburgh School of Medicine, Pittsburgh, PA and the Department of Medicine, Coney Island Hospital, Coney Island, NY
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  • David C. Herbert MD,

    1. Professor of Radiology, University of Pittsburgh
    2. Departments of Medicine and Radiology, University of Pittsburgh School of Medicine, Pittsburgh, PA and the Department of Medicine, Coney Island Hospital, Coney Island, NY
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  • Lawrence A. Cooperstein MD

    1. Assistant Professor of Radiology, University of Pittsburgh
    2. Departments of Medicine and Radiology, University of Pittsburgh School of Medicine, Pittsburgh, PA and the Department of Medicine, Coney Island Hospital, Coney Island, NY
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Abstract

We reviewed the long-term natural history of 21 adult-onset Still's disease patients. Patient subsets were identified according to clinical course patterns. These included monocyclic systemic disease in 4, polycyclic systemic disease in 2, chronic articular monocyclic systemic disease in 10, and chronic articular polycyclic systemic disease in the remaining 5 patients. Functional outcome differed according to course patterns and the extent of articular involvement. Systemic manifestations, per se, did not contribute to poor functional prognosis. Chronic articular disease had the worst outcome: 27% evolved to functional class III status, compared with none in the cyclic systemic groups. Those patients who had a chronic articular pattern or a polyarticular onset and course were at higher risk to develop disabling arthritis. An aggressive approach to therapy, including the early use of remittive agents, should be considered in these patient subsets.

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