CCL17/thymus and activation-related chemokine in Churg-Strauss syndrome.
OBJECTIVE
Churg-Strauss syndrome (CSS) is a Th2-mediated systemic vasculitis characterized by eosinophilic infiltration, blood eosinophilia, and high IgE levels. CCL17/thymus and activation-regulated chemokine (TARC) is a chemokine responsible for the recruitment of Th2 cells. This study was undertaken to explore a possible role of CCL17/TARC in CSS.


METHODS
CCL17/TARC levels in serum from patients with active or inactive CSS, hypereosinophilic syndrome, systemic small-vessel vasculitis other than CSS, other types of eosinophilia, and healthy controls were determined by enzyme-linked immunosorbent assay. Biopsy samples of affected tissue from CSS patients were examined by immunohistochemical staining for Th2 infiltration and CCL17/TARC expression.


RESULTS
Serum CCL17/TARC levels were significantly elevated in CSS patients with active disease (mean ± SEM 1,122.0 ± 422.7 pg/ml) compared with controls (220.6 ± 27.9 pg/ml) and patients with inactive disease (388.9 ± 72.6 pg/ml) (P < 0.001 and P < 0.05, respectively). These levels correlated with the clinical disease course of CSS and with absolute eosinophil counts as well as IgE levels. Infiltrating Th2 cells in active CSS lesions were evidenced by CD294 staining. CCL17/TARC in the affected tissue of CSS patients was readily identified by immunohistochemical analysis. Elevated CCL17/TARC levels were also noted in patients with hypereosinophilic syndrome (794.5 ± 294.8 pg/ml) and other disorders associated with eosinophilia (1,096.0 ± 345.3 pg/ml) (both P < 0.005 versus controls).


CONCLUSIONS
CCL17/TARC may contribute to CSS pathogenesis by recruitment of Th2 cells into affected tissue. Serum CCL17/TARC levels reflect disease activity, and further studies to validate its use as an activity marker in CSS are warranted.
Dallos
Tomáš
T
University of Erlangen-Nuremberg, Erlangen, Germany; Comenius University in Bratislava, Bratislava, Slovakia.
Heiland
Gisela Ruiz
GR
Strehl
Johanna
J
Karonitsch
Thomas
T
Gross
Wolfgang L
WL
Moosig
Frank
F
Holl-Ulrich
Constanze
C
Distler
Jörg H W
JH
Manger
Bernhard
B
Schett
Georg
G
Zwerina
Jochen
J
D054414:Chemokine CCL17 / Q000097:blood* / Q000276:immunology; D015267:Churg-Strauss Syndrome / Q000097:blood* / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D004804:Eosinophils / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D018709:Statistics, Nonparametric; D018418:Th2 Cells / Q000276:immunology*; D013950:Thymus Gland / Q000276:immunology*
D054414:Chemokine CCL17