Increased production of IL-5 and dominant Th2-type response in airways of Churg-Strauss syndrome patients

Rheumatology (Oxford). 2012 Oct;51(10):1887-93. doi: 10.1093/rheumatology/kes171. Epub 2012 Jul 5.


Objective: Churg-Strauss syndrome (CSS) is a rare systemic vasculitis associated with eosinophilia and asthma. We assessed the local immune response in airways of CSS patients with different activity of the disease.

Methods: Concentration of IL-5, CCL17, CCL22 and CCL26 (ELISA) together with cell expression of T-helper-related genes (real-time PCR array) were measured in bronchoalveolar lavage fluid (BALF) sampled from 11 patients with active CSS, 11 patients with CSS in remission and 9 control subjects with bronchial asthma.

Results: In active CSS, both BALF and blood eosinophil counts were increased (P<0.01). BALF cells in active disease were characterized by an increased expression of Th2 and regulatory-type transcripts: STAT6, STAT3, GATA3, IL4, IL5 and IL10 as compared with asthmatics, and STAT5A, CCR4, FOXP3, IL4, IL5 and IL10 when compared with inactive CSS. There was significant increase in BALF concentration of IL-5 and CCL26 in exacerbation of CSS. CCR4-active chemokines were detected more frequently in active disease. We found a strong positive correlation between clinical parameters of disease activity (BVAS, eosinophilia) and expression of IL4, IL5, IL10 and STAT5A.

Conclusion: These results indicate that as compared with asthma, active-CSS patients have much stronger local Th2 response in the airways. Airway cells may contribute to lung eosinophilia in CSS by producing IL-5 and eosinophil active chemokines.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Bronchoalveolar Lavage Fluid / chemistry*
  • Bronchoalveolar Lavage Fluid / immunology
  • Chemokine CCL17 / analysis
  • Chemokine CCL22 / analysis
  • Chemokine CCL26
  • Chemokines, CC / analysis
  • Churg-Strauss Syndrome / immunology*
  • Churg-Strauss Syndrome / metabolism
  • Eosinophils / immunology
  • Female
  • Gene Expression / immunology*
  • Humans
  • Interleukin-5 / analysis*
  • Male
  • Middle Aged


  • CCL26 protein, human
  • Chemokine CCL17
  • Chemokine CCL22
  • Chemokine CCL26
  • Chemokines, CC
  • Interleukin-5