Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: CXCL4 is a pleiotropic antiangiogenic and immunomodulatory chemokine, that has been shown to be increased in the sera of patients with systemic sclerosis (SSc) as well as in patients previously referred to as early SSc, at present labeled as undifferentiated connective tissue disease (UCTD) at risk for SSc. We aimed to search for relationships between CXCL4 serum levels and demographic, serological, and clinical features in patients with UCTD at risk for SSc, that is patients with Raynaud’s phenomenon (RP) and SSc marker autoantibodies and/or nailfold capillaroscopy (NVC) scleroderma pattern, who do not satisfy the 2013 ACR/EULAR criteria for SSc classification.
Methods: Serum levels of CXCL4 were measured in 24 autoantibody positive UCTD patients at risk for SSc and 24 osteoarthritis/fibromyalgia controls by a fluorescence-based suspension immunoassay. All patients were investigated for preclinical and clinical organ involvement, SSc marker autoantibodies, and NVC abnormalities. The new 2013 ACR/EULAR classification score for SSc was calculated for each patient at baseline and at the end of follow-up.
Results: Median serum levels of CXCL4 were increased in UCTD patients at risk for SSc (6.79 ng/ml, interquartile range – IQR- 3.4-13.8 vs 1.44 ng/ml, IQR 0.14-3 in controls, p < 0.0001). CXCL-4 resulted to be correlated to ICAM-1 serum levels (r=0.44, p < 0.05); had higher levels in the 6 anti-Scl70 positive patients (median 16.27 ng/ml, IQR 10.25-33.5) than in the 17 ACA positive patients (5, IQR 2.9-8.1 ng/ml, p < 0.01) and exceeded the 95° percentile of control values (12.24 ng/ml) in 5/15 (33.3 %) of patients who progressed to definite SSc vs 1/9 (10%) of those who did not at the end of the follow-up period (median follow-up: 2.75 years, range 0.4-8.4) (p = 0.3). Moreover, CXCL4 positively correlated with serum levels of soluble ICAM-1 (r = 0.44; p < 0.05).
Conclusion: Here we confirm that patients with UCTD at risk for SSc have increased serum levels of CXCL4. Moreover, we point out an association with anti-Scl70 positivity and a correlation with serum ICAM-1, a marker of endothelial activation. The predictive role, if any, of CXCL4 in patients with UCTD at risk for SSc awaits to be assessed in larger series of patients.
To cite this abstract in AMA style:Vettori S, Irace R, Giacco V, Riccardi A, Vicedomini L, Pellecchia L, Valentini G. Serum CXCL4 Increase in Patients with Undifferentiated Connective Tissue Disease at Risk for Systemic Sclerosis Is Associated with Anti-Scl70 Antibodies and ICAM-1, a Marker of Endothelial Activation [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/serum-cxcl4-increase-in-patients-with-undifferentiated-connective-tissue-disease-at-risk-for-systemic-sclerosis-is-associated-with-anti-scl70-antibodies-and-icam-1-a-marker-of-endothelial-activation/. Accessed July 23, 2019.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/serum-cxcl4-increase-in-patients-with-undifferentiated-connective-tissue-disease-at-risk-for-systemic-sclerosis-is-associated-with-anti-scl70-antibodies-and-icam-1-a-marker-of-endothelial-activation/