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Complement activation in systemic sclerosis.

G Wild1, J Watkins, A M Ward

  • 1Department of Immunology, Royal Hallamshire Hospital, Sheffield, UK.

Journal of Clinical & Laboratory Immunology
|January 1, 1990
PubMed
Summary
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Systemic sclerosis patients commonly show complement activation, primarily via the classical pathway. Nafamstat mesilate enabled accurate measurement of C3a and C4a, revealing frequent elevations despite normal C3 and C4 levels.

Area of Science:

  • Immunology
  • Rheumatology
  • Biochemistry

Background:

  • Systemic sclerosis is an autoimmune disease characterized by fibrosis.
  • Complement system activation is implicated in autoimmune and inflammatory conditions.
  • Accurate in vivo measurement of complement activation in systemic sclerosis has been challenging.

Purpose of the Study:

  • To reliably estimate in vivo complement activation in patients with systemic sclerosis.
  • To investigate the prevalence and pathway of complement activation in systemic sclerosis.

Main Methods:

  • Utilized nafamstat mesilate, a synthetic protease inhibitor, for accurate complement activation measurements.
  • Measured serum levels of C3a and C4a anaphylatoxins in patients with systemic sclerosis.

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Main Results:

  • Elevated C3a anaphylatoxins were detected in 2 out of 30 patients.
  • Elevated C4a anaphylatoxins were detected in 24 out of 30 patients.
  • Complement activation, predominantly via the classical pathway, was common, even with normal C3 and C4 levels.

Conclusions:

  • Complement activation is a frequent finding in systemic sclerosis.
  • The classical pathway is the predominant route of complement activation in this disease.
  • Nafamstat mesilate is a valuable tool for assessing complement activation in vivo.