@article{oai:nagasaki-u.repo.nii.ac.jp:00016617, author = {Yoshizaki, Ayumi and Komura, Kazuhiro and Iwata, Yohei and Ogawa, Fumihide and Hara, Toshihide and Muroi, Eiji and Takenaka, Motoi and Shimizu, Kazuhiro and Hasegawa, Minoru and Fujimoto, Manabu and Sato, Shinichi}, issue = {2}, journal = {Journal of clinical immunology}, month = {Mar}, note = {INTRODUCTION: The high mobility group box 1 protein (HMGB-1)/advanced glycation end products (RAGE) system is recently shown to play an important part in immune/inflammatory disorders. However, the association of this system in systemic sclerosis (SSc) remains unknown. MATERIALS AND METHODS: To determine clinical association of serum levels of HMGB-1 and soluble RAGE (sRAGE) in patients with SSc, sera from 70 patients with SSc and 25 healthy controls were examined by enzyme-linked immunosorbent assay. Sera from tight-skin mice and bleomycin-induced scleroderma mice, animal models for SSc, were also examined. Skin HMGB-1 and RAGE expression was assessed by immunohistochemistry. RESULTS AND DISCUSSION: Serum HMGB-1 and sRAGE levels in SSc were higher than those in controls. Similarly, HMGB-1 and sRAGE levels in animal SSc models were higher than those in control mice. SSc patients with elevated HMGB-1 and sRAGE levels had more frequent involvement of several organs and immunological abnormalities compared to those with normal levels. Furthermore, HMGB-1 and sRAGE levels correlated positively with modified Rodnan total skin thickness score and negatively with pulmonary function test. CONCLUSIONS: HMGB-1 and sRAGE expression in the sclerotic skin was more intense than normal skin. These results suggest that elevated serum HMGB-1 and sRAGE levels are associated with the disease severity and immunological abnormalities in SSc., Journal of clinical immunology, 29(2), pp.180-189; 2009}, pages = {180--189}, title = {Clinical significance of serum HMGB-1 and sRAGE levels in systemic sclerosis: association with disease severity.}, volume = {29}, year = {2009} }