Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage

Arthritis Rheum. 1998 Mar;41(3):445-52. doi: 10.1002/1529-0131(199803)41:3<445::AID-ART10>3.0.CO;2-3.


Objective: To find serologic markers of disease activity in patients with Churg-Strauss syndrome (CSS) linked to possible pathogenetic mechanisms by studying endothelial cell damage (soluble thrombomodulin [sTM]) in relation to T cell and eosinophil activation markers (soluble interleukin-2 receptor [sIL-2R] and eosinophil cationic protein [ECP]), and the presence of autoantibodies (antineutrophil cytoplasmic antibodies [ANCA] and anti-endothelial cell antibodies [AECA]) during both active and inactive phases of disease.

Methods: Sixteen consecutive patients who fulfilled the 1992 Chapel Hill definition of CSS were studied over a period of 4.5 +/- 3.9 years (mean +/- SD). ECP was detected by Columbo immunocapture (immunoCAP) assay, sIL-2R and sTM by enzyme-linked immunosorbent assay (ELISA), AECA by cell ELISA, and ANCA by indirect immunofluorescence and ELISA.

Results: In patients with active disease, ECP (8.4 +/- 90 units/ml), sIL-2R (3,725 +/- 2,310 units/ml), and sTM levels (5.5 +/- 2.9 units/liter) were significantly elevated compared with those in remission. Levels of sIL-2R showed a close correlation with levels of sTM (r = 0.75, P < 0.05). Interestingly, during remission, sIL-2R levels remained elevated in 4 of 7 patients, although the erythrocyte sedimentation rate, C-reactive protein level, and sTM level returned to the normal range (levels > 1,000 units/ml were associated with relapse). ANCA were found in only 7 patients (4 had classic ANCA, 3 had perinuclear ANCA), and AECA in 11 sera from 8 patients. In contrast to AECA, ANCA were associated with active disease.

Conclusion: In its active state, CSS is associated with markedly increased levels of sIL-2R and ECP, indicating T cell and eosinophil activation. Elevated sTM is a sign of endothelial cell damage that can be closely linked to T cell activation, as indicated by increased sIL-2R levels.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antibodies / analysis
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Blood Proteins / analysis
  • Churg-Strauss Syndrome / blood*
  • Churg-Strauss Syndrome / pathology*
  • Churg-Strauss Syndrome / therapy
  • Endothelium, Vascular / immunology
  • Endothelium, Vascular / pathology*
  • Eosinophil Granule Proteins
  • Female
  • Humans
  • Inflammation Mediators / blood
  • Lymphocyte Activation / physiology*
  • Male
  • Middle Aged
  • Receptors, Interleukin-2 / blood
  • Ribonucleases*
  • Solubility
  • Thrombomodulin / blood


  • Antibodies
  • Antibodies, Antineutrophil Cytoplasmic
  • Blood Proteins
  • Eosinophil Granule Proteins
  • Inflammation Mediators
  • Receptors, Interleukin-2
  • Thrombomodulin
  • Ribonucleases