Examples of using Diffuse ssc in English and their translations into Portuguese
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The diagnosis of diffuse SSc was established on clinical basis.
Of the 87 patients assessed,19 21.8% had diffuse SSc.
In the present study,as most patients presented late diffuse SSc, the"active" and the"late" capillaroscopic patterns were predominant.
Osteoporosis total femur,femoral neck and/or lumbar spine was found in 37% of the diffuse SSc patients.
Thirty-eight female patients with diffuse SSc attending the Scleroderma outpatient clinic of our institution were eligible to participate in this study.
Honeycombing is most commonly seen in usual interstitial pneumonitis UIP which can be found in diffuse SSc.
The decision to focus the analysis on diffuse SSc and female gender was important to select a more homogeneous group of patients in a heterogeneous disease as SSc. .
Low vitamin D serum levels had a negative impact on physical andemotional domains of quality of life in diffuse SSc patients.
This is a cross-sectional study analyzing 38 diffuse SSc patients who attended the Scleroderma Outpatient Clinic of the University of São Paulo from May 2012 to May 2013.
We confirmed previous studies showing a high frequency of low levels of 25OHD and higher risk for autoimmune diseases,including diffuse SSc.
Inclusion criteria were female gender; age between 18 and50 years; diffuse SSc according to LeRoy et al. criteria; capacity to understand the study and sign the informed consent.
Interestingly, this is the first study to reveal the importance of vitamin D for several physical andemotional domains of quality of life in diffuse SSc.
In this regard,future studies analyzing hand grip in diffuse SSc will be interesting, since vitamin D status was reported to predict hand-grip strength in young adult women.
This is the first study identifying that vitamin D is significantly correlated with QoL andsevere capillaroscopic findings in diffuse SSc.
Hence, we conclude that CPM reduced the severe cutaneous thickening in patients with diffuse SSc and could in the future have beneficial effects in morbidity and mortality of the disease.
Our study proves for the first time that cyclophosphamide has a beneficial effect specifically in the skin of patients with severe diffuse SSc.
Morita and colleagues reported that patients with diffuse SSc had the highest rates of disability in HAQ, higher than those of patients with RA, SLE and other collagen vascular diseases.
On the other hand, Sampaio-Barros et al. found a significant correlation between serum levels of 25-hydroxyvitamin D 25OHD andquality of life in patients with diffuse SSc.
Therefore, unlike the previously published studies,our group analyzed the skin of patients with severe diffuse SSc elevated MRSS in an objective and constant manner during a minimum period of 12 months, without the interference of factors such as severe pulmonary or cardiac disease.
The aim of this study was therefore, to analyze the correlation of vitamin D with quality of life, clinical parameters andnailfold capillaroscopy NC in patients with diffuse SSc.
In the current edition of BJR,Macedo et al. have examined the clinical course of nine patients with diffuse SSc and total skin score TSS> 30 without severe visceral involvement who made use of intravenous cyclophosphamide for 18 months and were followed at the Universidade de São Paulo USP.
In this study, similarly to the findings of other studies, the ACA antibody was associated with indicators of better prognosis,differently from the ATA-1 antibody, associated with diffuse SSc and higher severity.
All patients fulfilled the ACR classification criteria for diffuse SSc with ages between 18 and 70 years and without history of severe or symptomatic pulmonary involvement, discarded by high resolution thorax tomography HRT and pulmonary function test CVF> 80.
Although there was no association with SSc- specific organ manifestations, there was a statistical association with SSc-specific autoantibody,the anti-Scl70 a known biomarker of diffuse SSc, which presented lower levels of 25OHD.
The present study provides novel evidence demonstrating that low levels of 25OHD can contribute to a decreased QoL in patients with diffuse SSc through its association with the underlying vascular involvement worst capillaroscopic findings and the autoantibody production anti-Scl70.
Ten years ago, we also published on BJR the experience at the Universidade Estadual de Campinas UNICAMP with intravenous cyclophosphamide in the treatment of 45 patients with SSc. Thirteen of them had rapidly progressive diffuse SSc and also presented statistical improvement at the TSS.
Considering that limited and diffuse SSc subtypes can present distinct profiles of organ involvement, they could affect QoL in a distinct way;in fact, diffuse SSc patients present higher global and local disability, and lower QoL than limited SSc patients.
This study reports two cases of Erasmus syndrome in patients with history of occupational exposure to silica, who, ten years after being diagnosed with pneumoconiosis,developed SSc. The first patient had skin manifestations characteristic of diffuse SSc, while the second had limited SSc CREST syndrome.
It is also known that IL-13 induces the expression of type I collagen gene and that its levels are elevated in patients with SSc, but its possible role in the induction of fibrosis is not well established. Very recently, a study found increased levels of IL-6 in serum andtissues of patients with diffuse SSc, which was associated with more severe skin involvement and increased mortality after three years, suggesting that IL-6 antagonism would be a promising therapeutic target in this situation.
The results of these two controlled studies have enabled that the cyclophosphamide could be considered and indicated as treatment of SSc interstitial lung disease according to EUSTAR recommendations, which still require further work to consider cyclophosphamide astreatment for aggressive cutaneous features, which are predominant in early diffuse SSc.