Up-regulation of endothelial cell adhesion molecules characterizes disease activity in systemic lupus erythematosus. The Shwartzman phenomenon revisited

Arthritis Rheum. 1994 Mar;37(3):376-83. doi: 10.1002/art.1780370311.


Objective: To test the hypothesis that during exacerbations of systemic lupus erythematosus (SLE), endothelial cells are activated to increase their expression of adhesion molecules.

Methods: Endothelial cell expression of E-selectin, vascular cell adhesion molecule 1 (VCAM-1), and intercellular adhesion molecule 1 (ICAM-1) was quantitated immunohistochemically in 20 biopsy specimens from nonlesional, non-sun-exposed skin from 16 SLE patients. Disease activity was evaluated with the SLE Disease Activity Index (SLEDAI) and with measurements of complement components C3a desArg, C3, and C4.

Results: The mean expression of all 3 adhesion molecules was significantly elevated in patients with SLE versus healthy controls, as well as in patients with active versus inactive SLE. The mean C3a desArg level was significantly higher in patients with active SLE compared with those with inactive SLE. The SLEDAI scores correlated directly with C3a desArg levels and inversely with C3 and with C4 levels. Evaluation of serial biopsy specimens demonstrated loss of endothelial cell adhesion molecules and reduction of C3a levels with clinical improvement.

Conclusion: Our findings demonstrate up-regulation of the surface expression of 3 distinct adhesion molecules, E-selectin, VCAM-1, and ICAM-1, in patients with SLE. The abnormal expression of these endothelial cell adhesion molecules is most marked in patients with active disease characterized by significant elevations of the complement split product C3a desArg. We suggest that in certain SLE patients, excessive complement activation in association with primed endothelial cells induces leukocyte-endothelial cell adhesion and leuko-occlusive vasculopathy.

MeSH terms

  • Cell Adhesion Molecules / metabolism*
  • Complement C3 / metabolism
  • Complement C3a / analogs & derivatives
  • Complement C3a / metabolism
  • Complement C4 / metabolism
  • E-Selectin
  • Endothelium, Vascular / metabolism*
  • Humans
  • Immunohistochemistry
  • Intercellular Adhesion Molecule-1
  • Lupus Erythematosus, Systemic / metabolism*
  • Lupus Erythematosus, Systemic / pathology
  • Shwartzman Phenomenon / metabolism*
  • Skin / metabolism
  • Skin / pathology
  • Up-Regulation*
  • Vascular Cell Adhesion Molecule-1


  • Cell Adhesion Molecules
  • Complement C3
  • Complement C4
  • E-Selectin
  • Vascular Cell Adhesion Molecule-1
  • complement C3a, des-Arg-(77)-
  • Intercellular Adhesion Molecule-1
  • Complement C3a