Clin Exp Rheumatol
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A single-centre retrospective longitudinal study to investigate the predictive value of KL-6 serum levels for the outcome of interstitial lung fibrosis in a large systemic sclerosis (SSc) patient cohort. ⋯ KL-6 can be used as a lung fibrosis severity marker, but its role as a marker for disease activity is questionable. Furthermore, following cyclophosphamide treatment serum KL-6 levels may decrease independently of the lung function parameters.
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To translate and adapt the University of California, Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument 2.0 (UCLA SCTC GIT 2.0) into Dutch and validate it among Dutch systemic sclerosis (SSc) patients. ⋯ The Dutch GIT 2.0 questionnaire showed good internal consistency, construct validity and test-retest reliability.
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In systemic sclerosis (SSc), the frequent involvement of hand and face leads to their disability. We aimed to assess influence of hand and face disability on global disability and Health related Quality of life (HRQoL). ⋯ dSSc patients present higher global and local disability, and lower HRQoL in SPI than lSSc patients. Local disabilities, assessed by CHDFS and MHISS, are independently related to global disability and HRQoL.
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We sought to examine the relationship between measures of ILD severity and PH in patients with SSc. ⋯ No association between severity of ILD and cardiac haemodynamic profiles were identified in this cohort. We believe this underscores the complex nature of PH and ILD in individuals with SSc. We do suspect that some individuals with SSc-ILD will also have concomitant pulmonary vascular disease but simple assessments to grade severity of ILD - by PFT or HRCT estimates of ILD extent - are likely not enough to reliably distinguish between PAH versus PH-ILD. Further research into how to distinguish and manage these subsets is warranted.