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

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.

2019 ◽  
Vol 11 (2) ◽  
Author(s):  
Herlina Yani ◽  
Sumartini Dewi ◽  
Andri Reza Rahmadi

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.      


Author(s):  
Suade BADAK ◽  
Bozkurt GÜLEK ◽  
Esra KAYACAN ERDOĞAN ◽  
Hülya BİNOKAY ◽  
Eren ERKEN

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.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Julia Spierings ◽  
Voon H Ong ◽  
Christopher P Denton

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.


2015 ◽  
Vol 42 (3) ◽  
pp. 449-455 ◽  
Author(s):  
Yong Hou ◽  
Qing-li Zhu ◽  
He Liu ◽  
Yu-xin Jiang ◽  
Liang Wang ◽  
...  

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.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 408.1-408
Author(s):  
A. Lo Gullo ◽  
D. Sinicropi ◽  
M. Cinquegrani ◽  
F. Savarino ◽  
C. Giuffrida ◽  
...  

Background:Systemic sclerosis (SSc) is characterized by early vascular involvement and by varying degrees of fibrosis in skin, lungs, and other tissues. Vascular manifestations include Raynaud’s phenomenon, digital ulcers, and pulmonary hypertension (PAH). The prevalence of PAH is 7.85–13% in SSc and it is the second most common cause of mortality in SSc. Circulating CD34+ cell number is associated to cardiovascular health status in several chronic conditions, including inflammatory disease. CD34+ cell number was found inconstantly reduced in SSc. Endocan is a proteoglycan expressed by endothelial cells likely interacting with white blood cells, recently suggested as a marker of vascular stress.Objectives:to evaluate CD34+ cell number and endocan as a markers od cardiovascular involvement in SSc.Methods:Standard transthoracic echocardiography, Rodnan skin score were performed. Vitamin D levels, CD34+ cell number, markers of inflammation, red blood cells distribution width (RDW) and Endocan plasma levels were also determined.Results:37 female SSc patients (11 diffuse/26 limited) and 35 matched healthy controls (HC) were enrolled. CD34+ cell count was lower as compared to controls (2.3±0.73 vs 2.9±0.6; p<0.001), CRP and ESR were significantly higher in SSc patients than controls. Vitamin D3 was lower in SSc patients with respect to controls (18±4.5 vs 27.26±10.98; p<0.001), Rodnan skin score was 28.89±10.24. Endocan was two times greater than controls and serum endocan levels were higher in patients with diffuse Ssc as compared with limited ones. We found no correlation between Endocan and: Rodnan skin score, ESR, fibrinogen; we found a trend of correlation between Endocan and Vitamin D levels (r= -0.315), RDW (r= 0.310), CRP (r= 0.310), but statistical significance was not reached likely due to the small sample size. We found an association of PAPs with Endocan levels (rho= 0.442, p<0.01) and CD34+ count (-0.349, p<0.05. A significant correlation was also found between Endocan and CD34+ cell number (rho= -0.605, p<0.01).Conclusion:In our study population, we found a significant correlation between CD34+ cell number and Endocan plasma levels and PAPs; Endocan and CD34+ progenitor cells might be suggested as potential marker of pulmonary arterial hypertension in SSc patients.References:[1]Lo Gullo et al. Atherosclerosis 2018Disclosure of Interests:Alberto Lo Gullo Speakers bureau: bayer, Davide Sinicropi: None declared, Mara Cinquegrani: None declared, Francesca Savarino: None declared, Clemente Giuffrida: None declared, Giovanni Squadrito: None declared, Giuseppe Mandraffino: None declared


Author(s):  
Piet van Riel

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.


Biomarkers ◽  
2019 ◽  
Vol 24 (4) ◽  
pp. 373-378 ◽  
Author(s):  
Pernille Juhl ◽  
Line Vinderslev Iversen ◽  
Tonny Karlsmark ◽  
Morten Asser Karsdal ◽  
Anne-Christine Bay-Jensen ◽  
...  

2020 ◽  
pp. jrheum.200234
Author(s):  
Victoria A. Flower ◽  
Shaney L. Barratt ◽  
Darren J. Hart ◽  
Amanda B. Mackenzie ◽  
Jacqueline A. Shipley ◽  
...  

Objective The modified Rodnan skin score (mRSS) remains the preferred method for skin assessment in systemic sclerosis (SSc). There are concerns regarding high inter-observer variability of mRSS and negative clinical trials utilising mRSS as the primary endpoint. High frequency ultrasound (HFUS) allows objective assessment of cutaneous fibrosis in SSc. We investigated the relationship between HFUS with both mRSS and dermal collagen. Methods Skin thickness (ST), echogenicity and novel Shear wave elastography (SWE) were assessed in 53 SSc patients and 15 healthy controls (HC) at the finger, hand, forearm and abdomen. The relationship between HFUS parameters with mRSS (n=53) and dermal collagen (10 SSc patients and 10 HC) was investigated. Intra-observer repeatability of HFUS was calculated using intra-class correlation coefficients (ICCs). Results HFUS assessment of ST (hand/forearm) and SWE (finger/hand) correlated with local mRSS at some sites. Subclinical abnormalities in ST, echogenicity and SWE were present in clinically uninvolved SSc skin. Additionally, changes in echogenicity and SWE were sometimes apparent despite objectively normal ST on HFUS. ST, SWE and local mRSS correlated strongly with collagen quantification (rho 0.697, 0.709, 0.649 respectively). Intra-observer repeatability was high for all HFUS parameters (ICCs for ST 0.946-0.978, echogenicity 0.648- 0.865 and SWE 0.953-0.973). Conclusion Our data demonstrates excellent reproducibility and reassuring convergent validity with dermal collagen content. Detection of subclinical abnormalities is an additional benefit of HFUS. The observed correlations with collagen quantification support further investigation of HFUS as an alternative to mRSS in clinical trial settings.


2019 ◽  
Vol 4 (2) ◽  
pp. 83-88 ◽  
Author(s):  
Thomas A Medsger ◽  
Thomas G Benedek

The pathology of skin involvement in systemic sclerosis (or scleroderma) was first described in detail in 1892. In this article, we trace the history of cutaneous scleroderma and the evolution of thinking of scholars who have addressed this topic. We focus on skin histopathologic abnormalities and both clinical and laboratory techniques proposed for quantifying skin thickening and mobility. We examine the development of the simple bedside physical examination method of Dr Gerald Rodnan, first published in the 1970s and subsequently modified by others in the early 1990s (modified Rodnan skin score). This method has been found to be the only completely validated technique for assessing skin thickness in systemic sclerosis. Now nearly 50 years later, the modified Rodnan skin thickness scoring system remains the gold standard for use in both systemic sclerosis clinical trials and observational studies.


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