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Abstract
Our previous study demonstrated increased expression of Heat shock protein (Hsp) 90 in the skin of patients with systemic sclerosis (SSc). We aimed to evaluate plasma Hsp90 in SSc and characterize its association with SSc-related features. Ninety-two SSc patients and 92 age-/sex-matched healthy controls were recruited for the cross-sectional analysis. The longitudinal analysis comprised 30 patients with SSc associated interstitial lung disease (ILD) routinely treated with cyclophosphamide. Hsp90 was increased in SSc compared to healthy controls. Hsp90 correlated positively with C-reactive protein and negatively with pulmonary function tests: forced vital capacity and diffusing capacity for carbon monoxide (DLCO). In patients with diffuse cutaneous (dc) SSc, Hsp90 positively correlated with the modified Rodnan skin score. In SSc-ILD patients treated with cyclophosphamide, no differences in Hsp90 were found between baseline and after 1, 6, or 12 months of therapy. However, baseline Hsp90 predicts the 12-month change in DLCO. This study shows that Hsp90 plasma levels are increased in SSc patients compared to age-/sex-matched healthy controls. Elevated Hsp90 in SSc is associated with increased inflammatory activity, worse lung functions, and in dcSSc, with the extent of skin involvement. Baseline plasma Hsp90 predicts the 12-month change in DLCO in SSc-ILD patients treated with cyclophosphamide.
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1 Institute of Rheumatology, Prague, Czech Republic (GRID:grid.418965.7) (ISNI:0000 0000 8694 9225); Charles University, Department of Rheumatology, First Faculty of Medicine, Prague, Czech Republic (GRID:grid.4491.8) (ISNI:0000 0004 1937 116X)
2 Institute of Rheumatology, Prague, Czech Republic (GRID:grid.418965.7) (ISNI:0000 0000 8694 9225); Charles University, Department of Physiotherapy, Faculty of Physical Education and Sport, Prague, Czech Republic (GRID:grid.4491.8) (ISNI:0000 0004 1937 116X)
3 Charles University, Department of Physiotherapy, Faculty of Physical Education and Sport, Prague, Czech Republic (GRID:grid.4491.8) (ISNI:0000 0004 1937 116X)
4 Charles University, Department of Methodology, Faculty of Physical Education and Sport, Prague, Czech Republic (GRID:grid.4491.8) (ISNI:0000 0004 1937 116X)
5 University of Erlangen-Nuremberg, Department of Internal Medicine III and Institute for Clinical Immunology, Erlangen, Germany (GRID:grid.5330.5) (ISNI:0000 0001 2107 3311)