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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.
Highlights
Our previous study demonstrated increased expression of Heat shock protein (Hsp) 90 in the skin of patients with systemic sclerosis (SSc)
We have previously shown that the Hsp[90] expression is increased in SSc skin and dermal fibroblasts and is critical for transforming growth factor (TGF)-β signalling
We report a modest increase in plasma levels of Hsp[90] in SSc patients compared to healthy controls, which was statistically significant
Summary
Our previous study demonstrated increased expression of Heat shock protein (Hsp) 90 in the skin of patients with systemic sclerosis (SSc). Baseline plasma Hsp[90] predicts the 12-month change in DLCO in SSc-ILD patients treated with cyclophosphamide. Systemic sclerosis (scleroderma, SSc) is an autoimmune connective tissue disease characterized by vasculopathy and fibrotic changes in multiple organs, the skin and the lungs It is a rare chronic rheumatic disorder of a complex a etiopathogenesis[1]. Scleroderma is traditionally seen as a prototypical multi-system fibrotic disorder mediated by transforming growth factor (TGF)-β and fibroblasts, which produce excessive extracellular matrix proteins such as collagen Another hypothesis attributes this disease to the malfunction of the connective tissue repair mechanism in response to injury[1,2]. These results have translational implications because several Hsp[90] inhibitors have already been used in clinical trials for other indications, mostly tumorous c onditions[16,25]
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