Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2012 Sep;67(9):1149-56.
doi: 10.1111/j.1398-9995.2012.02873.x. Epub 2012 Jul 9.

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

P Khoury  1 , P Zagallo, C Talar-Williams, C S Santos, E Dinerman, N C Holland, A D Klion
Affiliations

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

P Khoury et al. Allergy. 2012 Sep.

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.

PubMed Disclaimer

Conflict of interest statement

No conflicts of Interest

Figures

Figure 1
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
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
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
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<0.05 by 1 way ANOVA. Horizontal line denotes mean minimal detectable levels. Mean minimal detectable values are as follows: IL-8 (0.3pg/mL), IL-6 (0.4pg/mL), Eotaxin-1 (2.1pg/mL), IL-10 (0.3pg/mL), IL-5 (0.1pg/mL), and sIL-2R (7.5pg/mL). Abbreviations: CSS-Churg Strauss Syndrome, HESwAS-Hypereosinophilic syndrome with asthma and/or sinusitis, HES-Hypereosinophilic syndrome.
Figure 5
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<0.05 by 1 way ANOVA. Horizontal line denotes minimal detectable level of TARC/CCL17 (7.8pg/mL). Abbreviations: CSS- Churg Strauss Syndrome, HESwAS-Hypereosinophilic syndrome with asthma and/or sinusitis, HES- Hypereosinophilic syndrome.
See this image and copyright information in PMC

References

    1. Simon HU, Rothenberg ME, Bochner BS, Weller PF, Wardlaw AJ, Wechsler ME, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol. 2010;126(1):45–49. - PMC - PubMed
    1. Radice A, Sinico RA. Antineutrophil cytoplasmic antibodies (ANCA) Autoimmunity. 2005;38(1):93–103. - PubMed
    1. Pagnoux C, Guillevin L. Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol. 2010;22(1):21–28. - PubMed
    1. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis) Arthritis Rheum. 1990;33(8):1094–1100. - PubMed
    1. Dallos T, Heiland GR, Strehl J, Karonitsch T, Gross WL, Moosig F, et al. CCL17/thymus and activation-related chemokine in Churg-Strauss syndrome. Arthritis Rheum. 2010;62(11):3496–3503. - PubMed

Publication types

Associated data

LinkOut - more resources