ABSTRACT
OBJECTIVE: To determine the diagnostic sensitivity and specificity and the clinical usefulness of parallel anticentromere-A and anticentromere-B antibody (anti-CENP-A and anti-CENP-B) testing in patients with systemic sclerosis (SSc). METHODS: Sera from 280 consecutive patients with SSc and 259 controls were tested for the presence of anti-CENP-A and anti-CENP-B antibodies by a monospecific line immunoblot assay (LIA) with recombinant human centromere proteins A and B as well as by indirect immunofluorescence (IIF). Crossreactivity and possible associations with clinical manifestations were studied. RESULTS: Both antibodies revealed a diagnostic sensitivity of 36.8% and a specificity of > 97% for SSc, with a high concordance rate of 94.3% despite different amino acid sequences of the antigens and absence of crossreactivity. There was a significant correlation of the antibody levels measured by LIA. Both antibodies were associated with similar clinical manifestations and identified patients with limited disease and rather mild skin sclerosis. CONCLUSION: Detected by LIA, anti-CENP-A and anti-CENP-B antibodies show high concordance in patients with SSc and share significant associations to clinical manifestations, but are not completely identical. Detection of both antibodies in parallel may slightly increase the diagnostic sensitivity for SSc.
Subject(s)
Antibodies, Antinuclear/immunology , Autoantigens/immunology , Centromere Protein B/immunology , Chromosomal Proteins, Non-Histone/immunology , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/immunology , Autoantigens/genetics , Centromere Protein A , Centromere Protein B/genetics , Chromosomal Proteins, Non-Histone/genetics , Humans , Scleroderma, Systemic/pathology , Scleroderma, Systemic/physiopathology , Sensitivity and Specificity , Severity of Illness IndexABSTRACT
OBJECTIVE: To assess and analyse nutritional status in patients with systemic sclerosis (SSc) and identify possible associations with clinical symptoms and its prognostic value. METHODS: Body mass index (BMI) and parameters of bioelectrical impedance analysis (BIA) were assessed in 124 patients with SSc and 295 healthy donors and matched for sex, age and BMI for comparisons. In patients with SSc, BMI and BIA values were compared with clinical symptoms in a cross-sectional study. In a prospective open analysis, survival and changes in the nutritional status and energy uptake induced by nutritional treatment were evaluated. RESULTS: Patients with SSc had reduced phase angle (PhA) values, body cell mass (BCM), percentages of cells, increased extracellular mass (ECM) and ECM/BCM values compared with healthy donors. Malnutrition was best reflected by the PhA values. Of the patients with SSc, 69 (55.7%) had malnutrition that was associated with severe disease and activity. As assessed by multivariate analysis, low predicted forced vital capacity and high N-terminal(NT)-proBNP values discriminated best between good and bad nutritional status. Among different clinical parameters, low PhA values were the best predictors for SSc-related mortality. BMI values were not related to disease symptoms or mortality. Fifty per cent of patients with SSc had a lower energy uptake related to their energy requirement, 19.8% related to their basal metabolism. Nutritional treatment improved the patients' nutritional status. CONCLUSIONS: In patients with SSc, malnutrition is common and not identified by BMI. BIA parameters reflect disease severity and provide best predictors for patient survival. Therefore, an assessment of nutritional status should be performed in patients with SSc.
Subject(s)
Malnutrition/etiology , Nutritional Status , Scleroderma, Systemic/complications , Adolescent , Adult , Aged , Body Composition , Body Mass Index , Electric Impedance , Epidemiologic Methods , Germany/epidemiology , Humans , Malnutrition/mortality , Malnutrition/physiopathology , Middle Aged , Prognosis , Scleroderma, Systemic/mortality , Scleroderma, Systemic/physiopathology , Young AdultABSTRACT
OBJECTIVE: In this pilot study, the effect of sildenafil on digital ulcer (DU) healing and related clinical symptoms was analysed. METHODS: A total of 19 patients with systemic sclerosis (SSc) were treated with maximally tolerated sildenafil doses up to 6 months. Primary outcome was the healing of DUs. Changes in other clinical symptoms were also evaluated. RESULTS: In all, 49 DUs were present at baseline; this decreased to 17 ulcers (p<0.001) at the end of sildenafil treatment. Furthermore, the visual analogue scale (VAS) score for Raynaud's phenomenon (RP), pain and activity improved (p=0.003, p=0.002 and p=0.05, respectively). A total of 9 patients developed 12 new DUs during sildenafil treatment. CONCLUSIONS: This study indicates an effect of sildenafil on DU healing in patients with SSc and an improvement of RP and associated symptoms that should be validated in controlled studies.
