scholarly journals Reliability and Validity of the Tender and Swollen Joint Counts and the Modified Rodnan Skin Score in Early Diffuse Cutaneous Systemic Sclerosis: Analysis from the Prospective Registry of Early Systemic Sclerosis Cohort

2017 ◽  
Vol 44 (6) ◽  
pp. 791-794 ◽  
Author(s):  
Jessica K. Gordon ◽  
Gandikota Girish ◽  
Veronica J. Berrocal ◽  
Meng Zhang ◽  
Christopher Hatzis ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Content Validity ◽  
Reliability And Validity ◽  
Intraobserver Reliability ◽  
Musculoskeletal Ultrasound ◽  
Skin Score ◽  
Modified Rodnan Skin Score ◽  
Diffuse Cutaneous Systemic Sclerosis ◽  
Early Systemic Sclerosis

Objective.To determine the inter/intraobserver reliability of the tender and swollen joint counts (TJC, SJC) and the modified Rodnan Skin Score (mRSS) in diffuse cutaneous systemic sclerosis (dcSSc) and to assess content validity of the TJC/SJC.Methods.Ten rheumatologists completed the SJC, TJC, and mRSS on 7 patients. Musculoskeletal ultrasound (MSUS) was performed.Results.Interobserver and intraobserver reliability for the TJC was 0.97 and 0.99, for the SJC was 0.24 and 0.71, and for the mRSS was 0.81 and 0.94, respectively. MSUS abnormalities did not correspond with SJC/TJC.Conclusion.We demonstrate excellent inter- and intraobserver reliability for the mRSS and TJC in dcSSc. However, the SJC and TJC did not correspond to MSUS.

Immunosuppression use in early systemic sclerosis may be increasing over time

2021 ◽  
pp. 239719832110009
Author(s):  
Ryan Park ◽  
Tatiana Nevskaya ◽  
Murray Baron ◽  
Janet E Pope
Keyword(s):  
Mycophenolate Mofetil ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Immunosuppressive Therapy ◽  
Skin Score ◽  
The Past ◽  
Modified Rodnan Skin Score ◽  
Diffuse Cutaneous Systemic Sclerosis ◽  
Early Systemic Sclerosis

Background: Immunosuppression remains the main treatment for progressing skin involvement, interstitial lung disease and inflammatory joint or muscle disease in systemic sclerosis. This study investigated the pattern and trends in immunosuppressive agents used in early systemic sclerosis (diagnosed before and after 2007) to determine whether the changes in the preferred type, timing and combination of immunosuppression took place over the past decade. Methods: In total, 397 Canadian Scleroderma Research Group database patients (183 diffuse cutaneous systemic sclerosis and 214 limited cutaneous systemic sclerosis) who had baseline and follow-up visits within 3 years (mean: 1.8 ± 0.8) after disease onset were included: 82% females, age at diagnosis 53 ± 13 years. Bivariate, chi-square, analysis of variance and adjusted regression analyses were used. Results: In total, 115 diffuse cutaneous systemic sclerosis patients (63%) and 62 limited cutaneous systemic sclerosis (29%) received immunosuppressive drugs, most commonly methotrexate, followed by mycophenolate mofetil and cyclophosphamide. In diffuse cutaneous systemic sclerosis, immunosuppressants were prescribed after 2007 more often (74% vs 50%, p = 0.001), especially methotrexate ( p = 0.02) and mycophenolate mofetil ( p = 0.04), and earlier (peak at 2 years after onset). Immunosuppressive therapy was associated with male gender, interstitial lung disease, anti-Scl70 positivity, ACA negativity and inflammatory joint disease in limited cutaneous systemic sclerosis and with ACA negativity and a higher modified Rodnan skin score in diffuse cutaneous systemic sclerosis. Multivariate regression analysis showed that the use of immunosuppressants after 2007 was predicted only by ACA negativity in limited cutaneous systemic sclerosis and by younger age in diffuse cutaneous systemic sclerosis. Conclusion: Over the past decade, there has been a trend to prescribe immunosuppressants more often and earlier in diffuse cutaneous systemic sclerosis patients, regardless of modified Rodnan skin score. Methotrexate is being more frequently used, and mycophenolate mofetil has gained favour over cyclophosphamide. Autoantibody status was the most consistent predictor of immunosuppressive therapy.

scholarly journals P151 Self-assessment of scleroderma skin thickness: development and evaluation of the PASTUL questionnaire

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Julia Spierings ◽  
Voon H Ong ◽  
Christopher P Denton
Keyword(s):  
Correlation Coefficient ◽  
Upper Limb ◽  
Content Validity ◽  
Health Assessment ◽  
Skin Thickness ◽  
Self Assessment ◽  
Skin Score ◽  
Modified Rodnan Skin Score ◽  
Physical Limitations ◽  
Test Retest Reliability

Abstract Background/Aims  Evaluation of skin is central to both clinical practice and trials in systemic sclerosis (SSc). This is generally done with the modified Rodnan Skin Score (mRSS). Remote consultations are now widely implemented in response to the COVID-19 pandemic, which has inevitably limited evaluation of skin. To monitor skin during this pandemic and to further explore ways to assess skin, we developed the PASTUL (Patient self-Assessment of Skin Thickness in Upper Limb) questionnaire. The aim of this study was to evaluate feasibility and validity of the PASTUL in SSc. Methods  The PASTUL questionnaire specifies a simple grading of skin as normal, mild, moderate, or severely thickened at eight sites of upper limb corresponding to mRSS. Assessed grades were converted to an integer scale [0, 1, 2, 3]. Detailed instructions for patients were provided. Scleroderma Skin PRO (SSPRO) and Scleroderma Health Assessment Disability Index (SHAQ-DI) were also completed. The mRSS was done in a selection of patients. Construct validity was evaluated by examining the correlation between PASTUL, mRSS, SSPRO and SHAQ-DI using Pearson’s correlation coefficient. Content validity was evaluated by scoring relevance, clarity and practical difficulty. Test-retest reliability was estimated using intraclass correlation coefficient (ICC). Results  In total, 107 patients were invited of which 83 (77.6%) completed the questionnaires. The mRSS was undertaken in 61 patients. The PASTUL was completed by patients (83.1%) or by a partner/friend (16.9%). Mean PASTUL score was 11 (SD 6), mean HAQ-DI 1.47 (SD 0.76) and mean SSPRO 49.8 (SD 26.6). PASTUL and SSPRO physical limitations correlated strongly (0.62, p < 0.001). Correlations between PASTUL and total SSPRO and mRSS upper limbs were moderate to weak (0.59, 0.50 and 0.32 respectively). Correlation between PASTUL and mRSS was stronger in lcSSc compared to dcSSc patients (0.61 vs 0.29) and when assessed by a partner/friend compared to patients themselves (0.98 vs 0.45). The PASTUL demonstrated excellent test-retest reliability (ICC of 0.92) and good content validity. P151 Table 1:Correlation of PASTUL score with other outcome measuresOutcome measurePearson's correlation coefficientP-valuemRSS0.48<0.001mRSS upper limbs0.50<0.001SHAQ-DI0.320.004VAS pain0.180.107VAS GI0.130.239VAS breathing0.130.236VAS RP0.090.406VAS DU0.090.466VAS limitations0.250.026SSPRO0.59<0.001SSPRO subdomain PE0.56<0.001SSPRO subdomain PL0.62<0.001SSPRO subdomain EE0.49<0.001SSPRO subdomain SE0.370.001DU, digital ulcers; EE, emotional effects; GI, gastrointestinal; HAQ-DI, Health Assessment Questionnaire Disability Index; mRSS, modified Rodnan skin score; PE, physical effects; PL, physical limitations; RP, Raynaud's phenomenon; SE, social effects Conclusion  Moderate and significant correlations of PASTUL scores with total SSPRO, physical limitation scores and mRSS support the usefulness of PASTUL as an outcome measure and indicates it’s potential for use in virtual clinical settings Disclosure  J. Spierings: None. V.H. Ong: None. C.P. Denton: None.

scholarly journals Correlation between Serum Krebs von den Lungen-6 Levels with Forced Vital Capacity and Modified Rodnan Skin Score of Patients with Restrictive Lung Disease in Diffuse-Type Systemic Sclerosis

2019 ◽  
Vol 11 (2) ◽  
Author(s):  
Herlina Yani ◽  
Sumartini Dewi ◽  
Andri Reza Rahmadi
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Skin Fibrosis ◽  
Diffuse Type ◽  
Skin Score ◽  
Restrictive Lung Disease ◽  
Modified Rodnan Skin Score

Background Pulmonary fibrosis / intersitial lung disease (ILD) in systemic sclerosis (SSc) is a complicated restrictive pulmonary disease and the leading cause of disease-related mortality. Progressive skin fibrosis in diffuse-type SSc (dSSc) is associated with decreased forced vital capacity (FVC). Modified Rodnan Skin Score (mRSS) examination is used as a parameter to assess skin fibrosis, while high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) are used to assess pulmonary fibrosis. The HRCT test remains as the gold standard in diagnosing ILD. However, it costs a lot and is not available in all healthcare facilities. Krebs Von den Lungen-6 (KL-6) is a biomarker to evaluate pulmonary fibrosis. The aim of this study was to analyze the correlation of serum KL-6 levels with FVC and mRSS value of patients with restrictive lung disease in dSSc. Method This was a cross-sectional study that used primary data from dSSc patients who visited rheumatology outpatient clinic in Hasan Sadikin Hospital Bandung, Indonesia, during the period of June-July 2019. History taking, physical examination, mRSS, spirometry, and serum KL-6 levels were performed. Data were analyzed using the Rank Spearman correlation test.  Result There were 27 subjects with the mean age of 42 ± 12 years. Based on FVC (%) restrictive lung disease criteria, the majority of subjects (74.1%) had severe restrictive lung disease and the rest of all subjects (25.9%) were non severe restrictive lung disease. Serum KL-6 levels ranged from 0.545 to 8.138 ng/ml. The results showed that there was no correlation between serum KL-6 levels and FVC values (r = -0.118, p = 0.279) and mRSS (r = 0.101, p = 0.312 ). Conclusion There is no correlation between serum KL-6 levels with FVC and mRSS value of patient with restritive lung disease in diffuse type systemic sclerosis. Keywords : diffuse type systemic sclerosis, Forced Vital Capacity, KL-6, mRSS, restrictive lung disease.      

Assessment of rheumatic disease

2013 ◽  
pp. 212-220
Author(s):  
Piet van Riel
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Sclerosis ◽  
Ankylosing Spondylitis ◽  
Rheumatic Diseases ◽  
Clinical Examination ◽  
Spinal Mobility ◽  
Diagnostic Process ◽  
Skin Involvement ◽  
Skin Score ◽  
Modified Rodnan Skin Score

The clinical examination of the musculoskeletal system is the cornerstone in the diagnostic process of rheumatic diseases. Next to this the clinical examination is important in evaluating the course of the different rheumatic diseases and the response to interventions. For instance in rheumatoid arthritis the joint scores—number of painful and swollen joints—are important in the evaluation of the disease activity of the patient. In systemic sclerosis the severity of skin involvement is measured with a skin score such as the modified Rodnan skin score, and in ankylosing spondylitis the spinal mobility is measured using different clinical scores. In general all these examinations should be carried out as far as possible in a standardized, systematic way.

Association of metabolites reflecting type III and VI collagen formation with modified Rodnan skin score in systemic sclerosis – a cross-sectional study

Biomarkers ◽  
2019 ◽  
Vol 24 (4) ◽  
pp. 373-378 ◽  
Author(s):  
Pernille Juhl ◽  
Line Vinderslev Iversen ◽  
Tonny Karlsmark ◽  
Morten Asser Karsdal ◽  
Anne-Christine Bay-Jensen ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional ◽  
Skin Score ◽  
Collagen Formation ◽  
Type Iii ◽  
Modified Rodnan Skin Score

scholarly journals Machine learning integration of scleroderma histology and gene expression identifies fibroblast polarisation as a hallmark of clinical severity and improvement

2020 ◽  
pp. annrheumdis-2020-217840 ◽  
Author(s):  
Kimberly Showalter ◽  
Robert Spiera ◽  
Cynthia Magro ◽  
Phaedra Agius ◽  
Viktor Martyanov ◽  
...  
Keyword(s):  
Gene Expression ◽  
Machine Learning ◽  
Systemic Sclerosis ◽  
Clinical Improvement ◽  
Smooth Muscle Actin ◽  
Clinical Severity ◽  
Support Vector ◽  
Alpha Smooth Muscle Actin ◽  
Modified Rodnan Skin Score ◽  
Diffuse Cutaneous Systemic Sclerosis

ObjectiveWe sought to determine histologic and gene expression features of clinical improvement in early diffuse cutaneous systemic sclerosis (dcSSc; scleroderma).MethodsFifty-eight forearm biopsies were evaluated from 26 individuals with dcSSc in two clinical trials. Histologic/immunophenotypic assessments of global severity, alpha-smooth muscle actin (aSMA), CD34, collagen, inflammatory infiltrate, follicles and thickness were compared with gene expression and clinical data. Support vector machine learning was performed using scleroderma gene expression subset (normal-like, fibroproliferative, inflammatory) as classifiers and histology scores as inputs. Comparison of w-vector mean absolute weights was used to identify histologic features most predictive of gene expression subset. We then tested for differential gene expression according to histologic severity and compared those with clinical improvement (according to the Combined Response Index in Systemic Sclerosis).ResultsaSMA was highest and CD34 lowest in samples with highest local Modified Rodnan Skin Score. CD34 and aSMA changed significantly from baseline to 52 weeks in clinical improvers. CD34 and aSMA were the strongest predictors of gene expression subset, with highest CD34 staining in the normal-like subset (p<0.001) and highest aSMA staining in the inflammatory subset (p=0.016). Analysis of gene expression according to CD34 and aSMA binarised scores identified a 47-gene fibroblast polarisation signature that decreases over time only in improvers (vs non-improvers). Pathway analysis of these genes identified gene expression signatures of inflammatory fibroblasts.ConclusionCD34 and aSMA stains describe distinct fibroblast polarisation states, are associated with gene expression subsets and clinical assessments, and may be useful biomarkers of clinical severity and improvement in dcSSc.

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