Clinical and Prognostic Associations Based on Serum Antinuclear Antibodies in Japanese Patients with Systemic Sclerosis

Authors

  • Masataka Kuwana MD,

    Corresponding author
    1. Associate in Medicine, Keio University School of Medicine (current address: Research Investigator in Medicine, University of Pittsburgh School of Medicine)
    2. Departments of Medicine, Keio University School of Medicine, Tokyo, and Nippon Kokan Hospital, Kawasaki, Japan.
    • Kuwana, MD, Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, 985 Scaife Hall, Pittsburgh, PA 15261
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  • Junichi Kaburaki MD,

    1. Assistant Professor of Medicine, Keio University School of Medicine
    2. Departments of Medicine, Keio University School of Medicine, Tokyo, and Nippon Kokan Hospital, Kawasaki, Japan.
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  • Yutaka Okano MD,

    1. Faculty of Medicine, Nippon Kokan Hospital
    2. Departments of Medicine, Keio University School of Medicine, Tokyo, and Nippon Kokan Hospital, Kawasaki, Japan.
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  • Takeshi Tojo MD,

    1. Assistant Professor of Medicine, Keio University School of Medicine
    2. Departments of Medicine, Keio University School of Medicine, Tokyo, and Nippon Kokan Hospital, Kawasaki, Japan.
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  • Mitsuo Homma MD

    1. Professor of Medicine, Keio University School of Medicine.
    2. Departments of Medicine, Keio University School of Medicine, Tokyo, and Nippon Kokan Hospital, Kawasaki, Japan.
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Abstract

Objective. To clarify the clinical features and prognosis of systemic sclerosis (SSc) based on serum antinuclear antibodies (ANA).

Methods. We studied 275 consecutive Japanese patients newly diagnosed as having SSc, who were first evaluated during the period 1971—1990. Eight SSc–related ANA were identified using indirect immunofluorescence, double immunodiffusion, or immunoprecipitation assays. Clinical and prognostic features were retrospectively analyzed in patient groups, categorized by their serum ANA.

Results. Cumulative survival rates at 10 years after diagnosis of SSc were 93% in patients with anticentromere antibodies (ACA), 72% in those with anti—U1 RNP, 66% in those with anti—DNA topoisomerase I (anti—topo I), and 30% in those with anti-RNA polymerases I, II, and III (anti-RNAP). Major organ involvement linked to cause of death included biliary cirrhosis in patients with ACA, isolated pulmonary arterial hypertension and cerebral hemorrhage in those with anti—U1 RNP, pulmonary interstitial fibrosis in those with anti—topo I, and cardiac and renal involvement in those with anti-RNAP.

Conclusion. Determinations of serum ANA in SSc patients are useful in predicting organ involvement and long-term outcome.

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