Serum biomarkers are similar in Churg-Strauss syndrome and hypereosinophilic syndrome

Allergy. 2012 Sep;67(9):1149-56. doi: 10.1111/j.1398-9995.2012.02873.x. Epub 2012 Jul 9.


Rationale: Churg-Strauss syndrome (CSS) and hypereosinophilic syndrome (HES) overlap considerably in clinical presentation. A reliable means of distinguishing between these groups of patients is needed, especially in the setting of glucocorticoid therapy.

Methods: A retrospective chart review of 276 adult subjects referred for evaluation of eosinophilia > 1500/μl was performed, and subjects with a documented secondary cause of eosinophilia or a PDGFR -positive myeloproliferative neoplasm were excluded. The remaining subjects were assessed for the presence of American College of Rheumatology (ACR) criteria. Laboratory and clinical parameters were compared between subjects with biopsy-proven vasculitis (CSS; n = 8), ≥4 ACR criteria (probable CSS; n = 21), HES with asthma and/or sinusitis without other CSS-defining criteria (HESwAS; n = 20), HES without asthma or sinusitis (HES; n = 18), and normal controls (n = 8). Serum biomarkers reported to be associated with CSS were measured using standard techniques.

Results: There were no differences between the subjects with definite or probable CSS or HES with respect to age, gender, or maintenance steroid dose. Serum CCL17, IL-8, and eotaxin levels were significantly increased in eosinophilic subjects as compared to normal controls, but were similar between the eosinophilic groups. Serum CCL17 correlated with eosinophil count (P < 0.0001, r = 0.73), but not with prednisone dose.

Conclusions: In patients with a history of asthma and sinusitis, distinguishing between ANCA-negative CSS and PDGFR-negative HES is difficult because of significant overlap in clinical presentation and biomarker profiles.

Trial registration: NCT00001406.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, N.I.H., Intramural

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers / blood*
  • Chemokine CCL11 / blood*
  • Chemokine CCL17 / blood*
  • Churg-Strauss Syndrome / blood*
  • Churg-Strauss Syndrome / metabolism
  • Churg-Strauss Syndrome / pathology
  • Female
  • Humans
  • Hypereosinophilic Syndrome / blood*
  • Hypereosinophilic Syndrome / metabolism
  • Hypereosinophilic Syndrome / pathology
  • Interleukin-8 / blood*
  • Male
  • Middle Aged
  • Retrospective Studies
  • Young Adult


  • Biomarkers
  • CCL11 protein, human
  • CCL17 protein, human
  • Chemokine CCL11
  • Chemokine CCL17
  • Interleukin-8

Associated data