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

Abstract

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: ClinicalTrials.gov NCT00001406.

Conflict of interest statement

No conflicts of Interest

Published 2012. This article is a U.S. Government work and is in the public domain in the USA.

Figures

Figure 1

Subject screening and evaluation for study. Abbreviations: CSS- Churg Strauss Syndrome, HESwAS-Hypereosinophilic syndrome with asthma and/or sinusitis, HES-Hypereosinophilic syndrome.

Figure 2

End organ manifestations demonstrated prior to evaluation at our institution and over the duration of follow-up. The bars represent the percentage of subjects in each group with involvement of the indicated organ system. Abbreviations: CSS-Churg Strauss Syndrome, GI-Gastrointestinal, HES-Hypereosinophilic syndrome, HESwAS- Hypereosinophilic syndrome with asthma and/or sinusitis.

Figure 3

Atopy and Polyp Status within groups. (A) Phadiotop results expressed as Phadiotop Arbitrary Units/liter (PAU/l) were comparable between groups. Open circles represent individual subjects within groups with geometric mean. Subjects in shaded region are not considered atopic by Phadiotop. (B) Proportion of polyps is higher in both probable CSS and definite CSS (n=13) as compared to the subjects with HES (n=2; * p=0.0004, Fisher’s Exact Test). Abbreviations: CSS- Churg Strauss Syndrome, HESwAS-Hypereosinophilic syndrome with asthma and/or sinusitis, HES- Hypereosinophilic syndrome.

Figure 4

Serum cytokine values. Serum evaluation of (A) IL-8 (B) Eotaxin-1, (C) IL-5, (D) IL-6, (E) IL-10 and (F) sIL-2 levels in groups with probable CSS, definite biopsy proven CSS, HES with asthma and/or sinusitis, HES without asthma or sinusitis and normal subjects. * p

Figure 5

CCL17/TARC levels. (A) TARC/CCL17 levels by group, and (B) Lack of correlation of TARC/CCL17 levels with equivalent prednisone dose for treated subjects, and (C) positive correlation of TARC/CCL17 with absolute eosinophil count. *p