Session Information
Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud’s – Pathogenesis, Animal Models and Genetics
Session Type: Abstract Submissions (ACR)
Background/Purpose: Systemic sclerosis (SSc) results in significant morbidity and mortality due to organ fibrosis characterized by increased deposition of extracellular matrix (ECM). Periostin is one of the matricellular proteins, a class of ECM-related molecules defined by their ability to modulate cell–matrix interactions. Recent studies revealed that periostin serves as a critical regulator of wound healing, epithelial mesenchymal transition, and fibrosis. However, the role of periostin in the pathogenesis of SSc is unknown. In this study, we determined periostin levels in association with clinical characteristics, including severity of skin sclerosis and disease duration in patients with SSc.
Methods: Expression of periostin was examined in primary fibroblasts and the skin obtained from SSc patients using immunoblotting and immunohistochemistry respectively. Enzyme-linked immunosorbent assay was performed to evaluate serum periostin levels in association with clinical characteristics in 56 patients with SSc (diffuse cutaneous SSc (dSSc); n=16, limited cutaneous SSc (lSSc); n=40) and 66 healthy controls. In addition, the sensitivity and specificity of serum periostin levels for detecting SSc were analyzed using a receiver operating characteristic (ROC) curve.
Results: Periostin was strongly expressed in the affected dermis and primary fibroblasts obtained from SSc patients. Serum levels of periostin in dSSc patients were markedly elevated compared to those in lSSc patients and control subjects. Patients with lSSc had increased periostin levels compared with healthy controls. In addition, significantly higher levels of periostin were observed in dSSc patients with disease duration £5 years compared with those with disease duration >5 years. No significant difference was found in serum periostin levels between SSc patients with interstitial lung disease (ILD) and those without ILD. Furthermore, the modified Rodnan total skin thickness score (MRSS) was positively correlated with periostin levels in SSc patients. Serial analysis revealed the relevance of MRSS and periostin levels in some dSSc patients during their course of the disease. Finally, ROC analysis demonstrated that measurement of serum periostin by ELISA would be useful to distinguish SSc patients from healthy controls.
Conclusion: An elevated periostin level in SSc patients was correlated with severity of skin sclerosis. Periostin may be a potential biomarker reflecting for disease severity in patients with SSc.
Disclosure:
Y. Yamaguchi,
None;
J. Ono,
None;
M. Masuoka,
None;
S. Ohta,
None;
K. Izuhara,
None;
Z. Ikezawa,
None;
M. Aihara,
None;
K. Takahashi,
None.
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