Clin Exp Rheumatol
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Letter Case Reports
Interleukin-6 targeting therapy in familial Mediterranean fever.
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Diffuse systemic sclerosis carries a high morbidity and mortality. The Prospective Registry of Early Systemic Sclerosis (PRESS), a multicentre incident cohort study of patients with early diffuse cutaneous systemic sclerosis, has the goal of advancing the understanding of disease pathogenesis and identifying novel biomarkers. ⋯ This review highlights the unmet need for effective treatment in early diffuse SSc as well as its more rigorous study. Nonetheless, the PRESS investigators aim to decrease intra- and inter-institutional variability in prescribing in order to improve the understanding of the clinical course of early diffuse SSc skin disease.
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To investigate the clinical characteristics of SSc patients with DUs in China. ⋯ The occurrence of DUs in Chinese SSc patients is frequent. It is possible that SSc patients with DUs were influenced by the disease earlier in life, which should be detected early for effective intervention.
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Heart and pulmonary involvement is a leading cause of systemic sclerosis (SSc)-related deaths. The six-minute walk test (6MWT) is a simple and reproducible test commonly used to evaluate exercise capacity. We tried to assess a potential relationship between exercise capacity assessed by 6MWT, echocardiographic parameters of right ventricular function and serum levels of endothelin-1 and NTproBNP. ⋯ Decreased exercise capacity significantly correlates with biochemical and echocardiographic parameters of right ventricular dysfunction and neurohormonal activation providing a potential link for neuroendocrine derangement in patients with SSc.
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To compare measurement properties of the EQ-5D and SF-6D utility measures, to assess the association and agreement between these measures and to estimate minimal important differences (MID) in patients with systemic sclerosis (SSc). ⋯ Although there was a marked correlation between the measures, the moderate agreement implies that EQ-5D and SF-6D scores cannot be used interchangeably. The MID estimates we provided can be used to calculate sample sizes for clinical trials involving SSc patients, and in interpreting the relevance and importance of treatment effects.