ABSTRACT
OBJECTIVES: To test the acceptability, feasibility, reliability and validity of the Italian translated version of the UCLA Scleroderma Clinical Trial Consortium GIT (UCLA-SCTC GIT) 2.0. Gastrointestinal tract (GIT) involvement is frequent in systemic sclerosis (SSc). The UCLA-SCTC GIT 2.0 is a validated instrument for measuring the presence and impact of GIT symptoms in SSc patients. METHODS: Acceptability and feasibility of the questionnaire were evaluated based on the input from the patients. Internal consistency was evaluated by Cronbach's alpha. External consistency was measured by comparing with the Short Form (SF)-36 and EQ-5D by Spearman's rho, meaningful if ≥0.30. RESULTS: Sixty-two consecutive SSc patients (mean age 60.6) were recruited, 88.5% were female. The UCLA-SCTC GIT 2.0 was well accepted. Percentage of missing data in UCLA-SCTC GIT total score was 2 %. Internal consistency was acceptable (alpha≥0.70) for all domains. Cronbach's alpha was ≥0.70 for all domains. UCLA-SCTC GIT 2.0 discriminated between patients with or without gastroesophageal reflux disease whether diagnosed clinically or by objective testing (p<0.01 for both). UCLA-SCTC GIT emotional well-being was correlated with the conceptually equivalent SF-36 mental health domains (correlation coefficient>0.35) and with the EQ-5D usual activities domain (0.38), thus reflecting the impact on everyday activities. The distention/bloating domain strongly correlated with the EQ-5D anxiety/depression domain (0.51) and reflux domain with role emotional of SF-36 (0.44). CONCLUSIONS: This is the first validation study of the Italian version of UCLA-SCTC GIT 2.0. Our data support its feasibility, reliability, and validity in Italian SSc patients.
Subject(s)
Gastrointestinal Diseases/diagnosis , Health Status , Scleroderma, Systemic/complications , Surveys and Questionnaires , Activities of Daily Living , Aged , Cost of Illness , Emotions , Feasibility Studies , Female , Gastrointestinal Diseases/etiology , Gastrointestinal Diseases/physiopathology , Gastrointestinal Diseases/psychology , Humans , Italy , Male , Mental Health , Middle Aged , Predictive Value of Tests , Reproducibility of Results , Scleroderma, Systemic/diagnosis , Severity of Illness Index , TranslatingABSTRACT
Systemic sclerosis (SSc) is a connective tissue disorder characterized by tissue fibrosis affecting the skin and internal organs, fibroproliferative vasculopathy, and autoimmune activation. SSc still heralds a poor prognosis with significant morbidity and mortality. Early detection of organ involvement is critical as currently available treatments are most effective when started early. Many candidate biomarkers have been investigated in the past two decades. However, despite the enormous efforts, no accurate tool to predict the pattern of organ involvement and to assess disease activity has been yet identified. The N-terminal fragment of probrain natriuretic peptide (N-TproBNP) is a neurohormone released by ventricular myocytes in response to pressure overload. N-TproBNP is highly relevant for diagnosis, prognosis, and prediction of pulmonary arterial hypertension in SSc. Moreover, several studies support its potential benefit for cardiac assessment of scleroderma patients. Conversely, the role of N-TproBNP as surrogate marker of pulmonary fibrosis and skin involvement is much less clear. We provide an extensive review of the studies that have previously investigated the role of N-TproBNP as candidate biomarker in scleroderma manifestations, presenting also the findings of a recent study we conducted in a cohort of 87 SSc patients.
