Background/Purpose: Periostin is one of the matricellular proteins, a class of ECM-related molecules defined by their ability to modulate cell–matrix interactions. Several lines of evidences suggest that periostin serves as a regulator of wound healing, epithelial mesenchymal transition, and fibrosis. We have previously reported overexpressed periostin levels in patients with systemic sclerosis (SSc) and a correlation between its serum levels and skin thickness score in SSc patients. In this study, we further analyzed circulating periostin levels in associations with clinical phenotypes including organ involvements in larger SSc samples.

Methods: We enrolled 385 patients with SSc and 204 healthy controls, who were recruited form 3 medical centers in Japan. Circulating levels of periostin were determined by enzyme-linked immunosorbent assay, and univariate and multivariate analysis was conducted to investigate their correlations with disease duration, antibody profiles, the modified Rodnan total skin thickness score (mRSS), and SSc-related organ involvements. Furthermore, available serial samples were analyzed to evaluate if a consecutive variation of periostin level reflect a change of mRSS (n = 76).

Results: One hundred and forty-six patients (38%) were classified as having diffuse cutaneous SSc (dcSSc), and 78 (20%), 169 (44%), 29 (8%), 209 (54%), 21 (5%), and 6 (2%) of SSc patients had digital ulcers (DU), interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), gastrointestinal involvement (GI), heart and renal involvements, respectively at initial evaluation. Disease duration from first non-Raynaud symptoms at evaluation was　7.8 ± 7.4 years. Periostin level was significantly elevated in SSc patients than in healthy controls (P < 0.0001). Higher periostin levels were observed in patients with dcSSc, shorter disease duration, higher mRSS, and positive for autoantibodies against to topo-I and RNAPIII. Multivariate analysis revealed that elevated periostin was an independent parameter associated with the presence of DU (P = 0.01), ILD (P = 0.0005), PAH (P = 0.003), GI (P = 0.017), and heart involvement (P = 0.024). In addition, mRSS was strongly associated with periosin level, disease duration, anti-topo-I and anti-RNAPIII antibodies by the multiple regression analysis (R2 = 0.35, P < 0.001). Finally, serial variation of periostin level correlated with a change of mRSS (R2 = 0.28, P < 0.001).

Conclusion: Elevated circulating periostin in SSc patients was strongly associated with mRSS and the presence of SSc-related organ involvements. Periostin may be a potential biomarker reflecting for disease severity in patients with SSc.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/an-increased-circulating-level-of-periostin-in-patients-with-systemic-sclerosis-associations-with-functional-impairment-in-various-affected-organs/