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Relationship between YKL-40 and pulmonary arterial hypertension in systemic sclerosis

Objectives: Systemic sclerosis (SSc) is an intractable connective tissue disease that causes skin and organ fibrosis. Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) affect its prognosis. YKL-40 protein impacts inflammation and tissue remodeling. Therefore, we evaluated the...

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Published in:Modern rheumatology 2019-05, Vol.29 (3), p.476-483
Main Authors: Furukawa, Tetsuya, Matsui, Kiyoshi, Kitano, Masayasu, Yokoyama, Yuichi, Sekiguchi, Masahiro, Azuma, Naoto, Imai, Yasutomo, Hirota, Seiichi, Yamanishi, Kiyofumi, Sano, Hajime
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Language:English
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Summary:Objectives: Systemic sclerosis (SSc) is an intractable connective tissue disease that causes skin and organ fibrosis. Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) affect its prognosis. YKL-40 protein impacts inflammation and tissue remodeling. Therefore, we evaluated the utility of YKL-40 blood levels in identifying patients with SSc complicated by PAH, as confirmed by immunohistochemistry (IHC) examination. Methods: We retrospectively analyzed 78 patients with SSc and performed IHC on 7 normal and 7 SSc skin samples in the Japanese population. Age-adjusted YKL-40 serum levels were analyzed. Results: YKL-40 age percentile was significantly elevated in SSc patients. There was no difference between patients with SSc with and without ILD and PAH. YKL-40 age percentile was greater in patients with PAH complication. YKL-40 immunostaining was negative in normal skin and prominent in the subcutaneous vascular wall of all SSc samples. Receiver operating characteristic (ROC) curve analysis indicated that YKL-40 age percentile correctly differentiated between patients with and without PAH with a sensitivity of 80% and a specificity of 94.1%. Conclusion: A higher YKL-40 level with PAH may be reflective of angiogenesis due to capillary injury in SSc. YKL-40 may offer a useful and easily applicable diagnostic biomarker of SSc complicated with PAH.
ISSN:1439-7595
1439-7609
DOI:10.1080/14397595.2018.1480256