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"Normal" acute phase response in systemic sclerosis.

M Chellingsworth, D G Scott, P A Crockson

    British Medical Journal (Clinical Research Ed.)
    |October 13, 1984
    PubMed
    Summary
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    Systemic sclerosis patients can mount a normal acute phase response to infection, contrary to previous beliefs. This indicates that the primary disease may mask this crucial immune reaction in some individuals.

    Area of Science:

    • Rheumatology
    • Immunology
    • Infectious Disease

    Background:

    • Systemic sclerosis is an autoimmune disease characterized by fibrosis and vascular abnormalities.
    • The acute phase response, a key immune reaction to infection, involves elevated serum proteins like C-reactive protein.
    • Previous studies suggested a defective acute phase response in scleroderma patients.

    Observation:

    • A patient with classic active progressive systemic sclerosis presented with a normal C-reactive protein (CRP) level.
    • During a Staphylococcus aureus infection, the patient's CRP concentration significantly increased to 250 mg/L.
    • This rise in CRP was unexpected given the presumed defective acute phase response in scleroderma.

    Findings:

    • The study demonstrates that the acute phase response is not uniformly defective in systemic sclerosis.

    Related Experiment Videos

  • Elevated CRP levels during infection indicate a functional acute phase response in this patient.
  • The findings challenge the long-held assumption of a blunted immune response in scleroderma.
  • Implications:

    • The acute phase response can occur in systemic sclerosis, suggesting the primary disease may mask its clinical manifestation.
    • This finding necessitates a re-evaluation of immune responses in systemic sclerosis patients.
    • Understanding the variability of the acute phase response is crucial for managing infections in scleroderma.