scholarly journals Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study

2018 ◽  
Vol 77 (4) ◽  
pp. 563-570 ◽  
Author(s):  
Ariane L Herrick ◽  
Sebastien Peytrignet ◽  
Mark Lunt ◽  
Xiaoyan Pan ◽  
Roger Hesselstrand ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Observational Study ◽  
Prediction Models ◽  
Rna Polymerase Iii ◽  
Logistic Models ◽  
Roc Curves ◽  
Skin Thickness ◽  
Skin Score ◽  
Registration Number ◽  
Randomised Controlled

ObjectivesOur aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs).MethodsThe modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. ‘Progressors’ were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (±3 months). Logistic models were fitted to predict progression and, using receiver operating characteristic (ROC) curves, were compared on the basis of the area under curve (AUC), accuracy and positive predictive value (PPV).Results66 patients (22.5%) progressed, 227 (77.5%) did not (33 could not have their status assessed due to insufficient data). Progressors had shorter disease duration (median 8.1 vs 12.6 months, P=0.001) and lower mRSS (median 19 vs 21 units, P=0.030) than non-progressors. Skin score was highest, and peaked earliest, in the anti-RNA polymerase III (Pol3+) subgroup (n=50). A first predictive model (including mRSS, duration of skin thickening and their interaction) had an accuracy of 60.9%, AUC of 0.666 and PPV of 33.8%. By adding a variable for Pol3 positivity, the model reached an accuracy of 71%, AUC of 0.711 and PPV of 41%.ConclusionsTwo prediction models for progressive skin thickening were derived, for use both in clinical practice and for cohort enrichment in RCTs. These models will inform recruitment into the many clinical trials of dcSSc projected for the coming years.Trial registration numberNCT02339441.

scholarly journals Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate)

2017 ◽  
Vol 77 (2) ◽  
pp. 212-220 ◽  
Author(s):  
Dinesh Khanna ◽  
Christopher P Denton ◽  
Celia J F Lin ◽  
Jacob M van Laar ◽  
Tracy M Frech ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Phase Ii ◽  
Controlled Trial ◽  
Safety Data ◽  
Open Label ◽  
Double Blind ◽  
Skin Score ◽  
Open Label Extension ◽  
Registration Number ◽  
Randomised Controlled

ObjectivesAssess the efficacy and safety of tocilizumab in patients with systemic sclerosis (SSc) in a phase II study.MethodsPatients with SSc were treated for 48 weeks in an open-label extension phase of the faSScinate study with weekly 162 mg subcutaneous tocilizumab. Exploratory end points included modified Rodnan Skin Score (mRSS) and per cent predicted forced vital capacity (%pFVC) through week 96.ResultsOverall, 24/44 (55%) placebo-tocilizumab and 27/43 (63%) continuous-tocilizumab patients completed week 96. Observed mean (SD (95% CI)) change from baseline in mRSS was –3.1 (6.3 (–5.4 to –0.9)) for placebo and –5.6 (9.1 (–8.9 to–2.4)) for tocilizumab at week 48 and –9.4 (5.6 (–8.9 to –2.4)) for placebo-tocilizumab and –9.1 (8.7 (–12.5 to –5.6)) for continuous-tocilizumab at week 96. Of patients who completed week 96, any decline in %pFVC was observed for 10/24 (42% (95% CI 22% to 63%)) placebo-tocilizumab and 12/26 (46% (95% CI 27% to 67%)) continuous-tocilizumab patients in the open-label period; no patients had >10% absolute decline in %pFVC. Serious infection rates/100 patient-years (95% CI) were 10.9 (3.0 to 27.9) with placebo and 34.8 (18.0 to 60.8) with tocilizumab during the double-blind period by week 48 and 19.6 (7.2 to 42.7) with placebo-tocilizumab and 0.0 (0.0 to 12.2) with continuous-tocilizumab during the open-label period.ConclusionsSkin score improvement and FVC stabilisation in the double-blind period were observed in placebo-treated patients who transitioned to tocilizumab and were maintained in the open-label period. Safety data indicated increased serious infections in patients with SSc but no new safety signals with tocilizumab.Trial registration numberNCT01532869; Results.

A Preliminary Study of Acoustic Radiation Force Impulse Quantification for the Assessment of Skin in Diffuse Cutaneous Systemic Sclerosis

2015 ◽  
Vol 42 (3) ◽  
pp. 449-455 ◽  
Author(s):  
Yong Hou ◽  
Qing-li Zhu ◽  
He Liu ◽  
Yu-xin Jiang ◽  
Liang Wang ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Acoustic Radiation ◽  
Radiation Force ◽  
Acoustic Radiation Force ◽  
Acoustic Radiation Force Impulse ◽  
Skin Thickness ◽  
Healthy Controls ◽  
Skin Involvement ◽  
Skin Score ◽  
Valuable Adjunct

Objective.To investigate skin elasticity using acoustic radiation force impulse (ARFI) quantification in systemic sclerosis (SSc), and compare the modified Rodnan skin score (mRSS) with measured shear wave velocity (SWV) and thickness of the skin.Methods.Fifteen patients with diffuse cutaneous SSc (dcSSc) and 15 age-matched and sex-matched healthy controls were evaluated. The SWV and thickness of skin were measured at 17 sites corresponding to those assessed in the mRSS in each participant. The SWV measurements of skin were compared between patients with dcSSc and healthy controls. The correlations between the mRSS and the skin SWV and thickness were explored using Spearman’s correlation.Results.The SWV values were higher in patients with dcSSc compared with healthy controls at right hand dorsum, right forearm, left hand dorsum, left forearm, right foot dorsum, and left foot dorsum (p < 0.05). In patients with dcSSc, the SWV values of uninvolved skin were higher than those of controls (p < 0.001), and the SWV values increased with increasing skin scores except for skin score 3 (p < 0.05). The sum of the SWV values correlated with total clinical skin score (r = 0.841, p < 0.001), and the sum of the skin thickness correlated with total clinical skin score (r = 0.740, p = 0.002).Conclusion.ARFI quantification is feasible and reliable for assessing the skin involvement in dcSSc. ARFI quantification could identify early skin change that may precede palpable skin involvement, and may be a valuable adjunct to skin evaluation in patients with SSc.

scholarly journals Skin score as a predictor of prolonged QTc in systemic sclerosis.

2021 ◽  
Author(s):  
◽  
Nafiseh Abdolahi ◽  
Mehrdad Aghaei ◽  
Ahmad Mohammadi ◽  
Keyword(s):  
Systemic Sclerosis ◽  
Significant Relationship ◽  
Rna Polymerase Iii ◽  
Cardiac Conduction ◽  
Control Group ◽  
Qtc Interval ◽  
Skin Score ◽  
Duration Of Disease ◽  
Pulmonary Arterial ◽  
American Society

Abstract Background Systemic sclerosis is an autoimmune disease characterized by endothelial dysfunction and fibrosis of the skin and internal organs. Cardiac involvement during systemic sclerosis can be primary or secondary to pulmonary arterial hypertension and renal pathology. Among the disorders in systemic sclerosis, prolongation of QTc time is also associated with more anti-RNA polymerase III antibodies, longer duration and severity of disease. Methods This case-control study was performed on 35 patients with systemic scleroderma who filled in the American Society of Rheumatism (ACR / EULAR criteria) and 35 healthy subjects prior to entering the study. Then, the QTc distance was extracted from the electrocardiogram and calculated using the formula. The measured QTc distance in the electrocardiogram, QTc> 440ms in men and QTc> 460ms in women, was defined as QTc long. Then the patients and the control group underwent echocardiography and changes in QTc interval and its relation with echocardiographic findings was evaluated. Results The results of this study indicated a significant relationship between QTc distance in patients with scleroderma compared with healthy controls. There was also a significant relationship between QTc and Skin Score of patients. However, there was no significant correlation between QTc distance and age, gender, duration of disease, Anti-Centromere, Anti-Scl70, and pulmonary artery pressure. Conclusion This study concludes that patients with scleroderma are at high risk for cardiac conduction impairment. The only factor that significantly correlated with QTc was the Skin Score of the patients.

scholarly journals Evaluation of thyroid volume in patients with systemic sclerosis; what did we find?

2021 ◽  
Author(s):  
Suade BADAK ◽  
Bozkurt GÜLEK ◽  
Esra KAYACAN ERDOĞAN ◽  
Hülya BİNOKAY ◽  
Eren ERKEN
Keyword(s):  
Systemic Sclerosis ◽  
Severity Score ◽  
Thyroid Dysfunction ◽  
Disease Duration ◽  
Thyroid Volume ◽  
Significant Negative Correlation ◽  
Skin Thickness ◽  
Cross Sectional ◽  
Skin Score ◽  
Modified Rodnan Skin Score

Introduction: Systemic sclerosis is a multisystemic disease. Thyroid involvement in systemic sclerosis is an issue that can be ignored. Our study aimed to evaluate the decreased thyroid volume in SSc. Also, we aimed to show the relationship between patients’ thyroid volume and severity score, clinical and laboratory parameters. Method: This was a single-center, cross-sectional study. Eighty-eight patients were included in the study. A radiologist evaluated patients’ thyroid volumes by ultrasonography. Demographic and clinical characteristics of the patients were recorded. Skin thickness was evaluated by the modified Rodnan skin score and the disease severity by the Medsger severity score. Findings were analyzed statistically. Results: Thyroid volume was in the atrophic range in 53.4% of the patients. There was a significant negative correlation between thyroid volume and mRSS, MSS, and disease duration. Logistic regression analysis showed that modified Rodnan skin score and disease duration were risk factors for thyroid atrophy. Conclusions: Many studies point out that thyroid autoantibodies are a cause of thyroid dysfunction in patients with SSc. However, in most of these studies, thyroid volume was not evaluated. As a result of our study, we saw that the major cause of thyroid dysfunction in our SSc patients was thyroid atrophy. Also, we observed that thyroid atrophy was more common in patients with ILD. We would like to draw attention to the fact that thyroid dysfunction and volume changes increase with the disease’s duration and severity in systemic sclerosis.

scholarly journals P152 Skin score trajectory associates with survival and pulmonary outcome in diffuse cutaneous systemic sclerosis

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Svetlana I Nihtyanova ◽  
Emma C Derrett-Smith ◽  
Carmen Fonseca ◽  
Voon H Ong ◽  
Christopher P Denton
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Complications ◽  
Rate Of Change ◽  
Skin Thickness ◽  
Group Level ◽  
Skin Score ◽  
Risk Of Death ◽  
Increased Risk ◽  
Diffuse Cutaneous Systemic Sclerosis ◽  
Over Time

Abstract Background Skin thickness improves over time in most diffuse cutaneous systemic sclerosis (dcSSc) patients and the use of group level skin score (mRss) as an endpoint is clinical trials can be challenging. We explore the association between individual mRss trajectories and outcome in early dcSSc patients. Methods Subjects with at least one mRss assessment within the first 5 years from onset were included. Random effects models were fitted to evaluate changes in mRss over time and model-predicted individual patient intercepts and slopes were included in Cox regression to assess associations with outcome. Results Of the 467 patients, 22.7% were male and mean age of disease onset was 45.5 years. Most frequent autoantibodies were anti-Scl70 in 30.2% and anti-RNA polymerase (ARA) in 30.0% of subjects. Average mRss at 12 months from onset was 25 and this declined over time, slowing down with longer disease duration (3.4, 2.7, 1.9 and 1.2 units at years 2, 3, 4 and 5). Higher initial mRss associated with greater subsequent decline (correlation coefficient -0.3). Both higher baseline mRSS (intercept) and slower decline (higher slope) predicted increased risk of death with 8% increase in hazard for every unit higher baseline mRss and 4% increase for every unit higher slope (Table 1). Adjusting for autoantibodies did not change the estimates. ANA+ENA- subjects had the highest risk of death, followed by ATA + (HR 0.91, p = 0.677 v ANA+ENA-), while risk was lowest among ARA+ subjects (HR 0.47, p = 0.002 v ANA+ENA-). Risk of pulmonary complications was associated with rate of change in mRss but not with baseline absolute value. A unit slower yearly decline in mRss increased the hazard of pulmonary fibrosis (PF) by 3.5% and pulmonary hypertension (PH) by 7% (Table 1). The association between mRss and PF disappeared after adjusting for antibody specificities, while the association between skin and PH did not change. Autoantibodies did not show significant association with PH development within this dcSSc cohort. Conclusion Although at a group level there is an improvement in skin over the initial 5 years, for individual patients, poor outcome for skin predicts increased risk of pulmonary complications and higher mortality. Disclosures S.I. Nihtyanova None. E.C. Derrett-Smith None. C. Fonseca None. V.H. Ong None. C.P. Denton None.

scholarly journals Durometry as an alternative tool to the modified Rodnan’s skin score in the assessment of diffuse systemic sclerosis patients: a cross-sectional study

2020 ◽  
Vol 60 (1) ◽  
Author(s):  
Marília de Fátima Cirioli de Oliveira ◽  
Vanessa Cristina Leopoldo ◽  
Karla Ribeiro Costa Pereira ◽  
Daniela Aparecida de Moraes ◽  
Juliana Bernardes Elias Dias ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Intraclass Correlation ◽  
Cross Sectional Study ◽  
Kappa Coefficient ◽  
Skin Thickness ◽  
Sectional Study ◽  
Rater Agreement ◽  
Cross Sectional ◽  
Skin Score ◽  
Diffuse Systemic Sclerosis

Abstract Background The reproducibility and reliability of the modified Rodnan’s Skin Score (mRSS) are debated due to investigator-related subjectivity. Here, we evaluate if durometry correlates with mRSS in patients with diffuse systemic sclerosis (SSc). Methods This cross-sectional study was conducted from December 2018 to June 2019, including 58 diffuse SSc patients. Two certified researchers, blind to each other’s scores, performed the mRSS, followed by durometry at 17 predefined skin sites. For durometry and mRSS, individual scores per skin site were registered. Durometry and mRSS results measured by each researcher, as well as scores from different researchers, were compared. Skin thickness measurements from forearm skin biopsies were available in a subset of the patients, for comparisons. Statistical analyses included Cohen’s Kappa Coefficient, Intraclass Correlation Coefficient, Kendall’s Coefficient and Spearman’s test. Results Mean (standard deviation, SD) patient age was 44.8 (12.9) years, and 88% were female. Inter-rater agreement varied from 0.88 to 0.99 (Intraclass correlation coefficient) for durometry, and 0.54 to 0.79 (Cohen’s Kappa coefficient) for mRSS, according to the specific evaluated sites. When data were compared with skin thickness assessed in forearm biopsies, durometry correlated better with skin thickness than mRSS. Conclusion Durometry may be considered as an alternative method to quantify skin involvement in patients with diffuse SSc. The strong inter-rater agreement suggests that the method may be useful for the assessment of patients by multiple researchers, as in clinical trials.

Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis

2018 ◽  
Vol 46 (1) ◽  
pp. 85-92 ◽  
Author(s):  
Sarah M. Gordon ◽  
Rodger S. Stitt ◽  
Robert Nee ◽  
Wayne T. Bailey ◽  
Dustin J. Little ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Sclerosis ◽  
Clinical Characteristics ◽  
Rna Polymerase Iii ◽  
High Risk Population ◽  
Scleroderma Renal Crisis ◽  
Skin Thickness ◽  
Laboratory Findings ◽  
Close Observation ◽  
Renal Crisis

Objective.Systemic sclerosis (SSc) is a disease of autoimmunity, fibrosis, and vasculopathy. Scleroderma renal crisis (SRC) is one of the most severe complications. Corticosteroid exposure, presence of anti-RNA polymerase III antibodies (ARA), skin thickness, and significant tendon friction rubs are among the known risk factors at SSc diagnosis for developing future SRC. Identification of additional clinical characteristics and laboratory findings could expand and improve the risk profile for future SRC at SSc diagnosis.Methods.In this retrospective cohort study of the entire military electronic medical record between 2005 and 2016, we compared the demographics, clinical characteristics, and laboratory results at SSc diagnosis for 31 cases who developed SRC after SSc diagnosis to 322 SSc without SRC disease controls.Results.After adjustment for potential confounding variables, at SSc diagnosis these conditions were all associated with future SRC: proteinuria (p < 0.001; OR 183, 95% CI 19.1–1750), anemia (p = 0.001; OR 9.9, 95% CI 2.7–36.2), hypertension (p < 0.001; OR 13.1, 95% CI 4.7–36.6), chronic kidney disease (p = 0.008; OR 20.7, 95% CI 2.2–190.7), elevated erythrocyte sedimentation rate (p < 0.001; OR 14.3, 95% CI 4.8–43.0), thrombocytopenia (p = 0.03; OR 7.0, 95% CI 1.2–42.7), hypothyroidism (p = 0.01; OR 2.8, 95% CI 1.2–6.7), Anti-Ro antibody seropositivity (p = 0.003; OR 3.9, 95% CI 1.6–9.8), and ARA (p = 0.02; OR 4.1, 95% CI 1.2–13.8). Three or more of these risk factors present at SSc diagnosis was sensitive (77%) and highly specific (97%) for future SRC. No SSc without SRC disease controls had ≥ 4 risk factors.Conclusion.In this SSc cohort, we present a panel of risk factors for future SRC. These patients may benefit from close observation of blood pressure, proteinuria, and estimated glomerular filtration rate, for earlier SRC identification and intervention. Future prospective therapeutic studies could focus specifically on this high-risk population.

scholarly journals Skin thickness score as a surrogate marker of organ involvements in systemic sclerosis: a retrospective observational study

2019 ◽  
Vol 21 (1) ◽  
Author(s):  
Kazuki M. Matsuda ◽  
Ayumi Yoshizaki ◽  
Ai Kuzumi ◽  
Takemichi Fukasawa ◽  
Satoshi Ebata ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Observational Study ◽  
Surrogate Marker ◽  
Retrospective Observational Study ◽  
Skin Thickness

scholarly journals Thermography Improves Clinical Assessment in Patients with Systemic Sclerosis Treated with Ozone Therapy

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Danuta Nowicka
Keyword(s):  
Systemic Sclerosis ◽  
Skin Temperature ◽  
Immunosuppressive Treatment ◽  
Skin Condition ◽  
Additional Treatment ◽  
Skin Thickness ◽  
Clinical Parameters ◽  
Ozone Therapy ◽  
Skin Score ◽  
Before And After

Objective. Treatment of scleroderma is challenging and limited. The aim of our study was to evaluate the usefulness of thermography in assessment of the clinical condition (joints movability and skin thickness) in clinically advanced patients with systemic sclerosis before and after ozone therapy. Method. The study included 42 patients aged 32 to 73 years with advanced systemic sclerosis hospitalized in the university clinic between 2003 and 2006. Thermography and clinical examinations were conducted at baseline and after two series of bath in water with ozone. Results. The comparison of results showed significant increase in skin temperature by 2.5°C, significant increase in interphalangeal joints movability by 18 degrees, and significant decrease in skin score by 14.7 points. The skin temperature was correlated with skin score (r=-0.59) and joints movability (r=+0.8). Conclusions. Ozone therapy shows positive effect on clinical parameters and skin temperature as measured with thermography. The study indicated possibility of introducing ozonotherapy as an independent therapy in cases with low level of progression or during remission periods and as additional treatment in patients with advanced disease requiring immunosuppressive treatment. Thermography is useful in assessment of skin condition showing strong correlation between skin temperature and clinical parameters.

scholarly journals The Relationship Between Modified Rodnan Skin Score (mRSS) With Pulmonal Hypertension and Lung Fibrosis in Systemic Sclerosis Patients

2021 ◽  
Vol 13 (1) ◽  
pp. 483-485
Author(s):  
Syaifur Rohman ◽  
Rakhma Yanti Hellmi ◽  
Friska Anggraini HS
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Blood Vessels ◽  
Significant Relationship ◽  
Lung Damage ◽  
Skin Thickness ◽  
Skin Score ◽  
Modified Rodnan Skin Score ◽  
The Relationship

Background: Systemic sclerosis (SSc) is a connective tissue disease, which affects the skin, blood vessels, heart, lungs, kidneys, gastrointestinal tract and musculoskeletal system. The manifestations in blood vessels include pulmonary hypertension which can be seen from echocardiography, while the manifestations to the lungs in the form of pulmonary fibrosis are examined by high-resolution computed tomography (HRCT). Modified Rodnan Skin Score (mRSS) a method for measuring skin thickness in SSc patients. Objective: This study aims to determine the relationship between mRSS and pulmonary hypertension and pulmonary fibrosis in SSc patients. Methods and Materials: Research with a cross sectional approach at Dr Kariadi Hospital. There were 23 study subjects with SSc patients having their mRSS measured by a rheumatologist and an HRCT examination by a radiologist to see the percent of lung damage and echocardiographic examination by a cardiologist to measure the Tricuspid Regurgitation Velocity Maximum (TRV Max) as a marker of pulmonary hypertension. Normality test using Saphiro-Wilk. The Spearman rank correlation test was used to analyze the relationship between the mRSS score, pulmonary hypertension and pulmonary fibrosis. Results: The proportion of study subjects with a mean age of 40.39 years, women, and 4.43 years of illness. The mean mRSS score was 17.43. Examination of pulmonary hypertension from echocardiography found most of the light category 95.6%, heavy category 4.4%, while the HRCT examination found pulmonary fibrosis of 86.9%, normal 13.1%. There are significant relationship between mRSS and pulmonary fibrosis (r = 0.485, p = 0.019), and significant relationship between pulmonary hypertension (r = 0.63, p = 0.001) Conclusion: There is a significant relationship between mRSS, pulmonary fibrosis and pulmonary hypertension.

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