Ultrasound Elastography Assessment of Skin Involvement in Systemic Sclerosis: Lights and Shadows

Objective.To assess skin elasticity in systemic sclerosis (SSc) by using a new imaging modality, ultrasound elastography (UE).Methods.Our study included 18 consecutive patients with SSc and 15 healthy controls. Modified Rodnan skin score, physical examination, and assessment of organ involvement were performed. UE was carried out on the middle forearm and on the fingers of the dominant arm. The echo signals recorded in real time during freehand operations of probe compression and relaxation produced images representing tissue elasticity, consisting of translucent colored bands superimposed on the B-mode ultrasonographic images. The color scale varied within a large band spectrum from red, indicative of soft and highly elastic tissue, to blue, which denoted hard and barely elastic tissue.Results.On the forearm of all patients, UE showed a homogeneous blue area corresponding to the dermis visualized in a B-mode ultrasonographic image; in controls, a blue pattern was never detected and a predominance of green with sporadic areas of pale blue was observed. At sequential evaluations, UE of fingers produced inconstant and changeable colored areas.Conclusion.The imaging pattern observed in the forearm of patients with SSc may represent the reduction of strain in the dermis due to loss of elasticity. The variable pattern obtained by finger evaluation demonstrated that UE can assess skin involvement in SSc only in those areas where the dermis is known to be thicker and where the bone hyperreflection is minimal. Further studies are needed to confirm our results and determine the validity of this new imaging modality.

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

The Journal of Rheumatology ◽

10.3899/jrheum.140873 ◽

2015 ◽

Vol 42 (3) ◽

pp. 449-455 ◽

Cited By ~ 19

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.

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Assessment of rheumatic disease

Oxford Textbook of Rheumatology ◽

10.1093/med/9780199642489.003.0028 ◽

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.

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Mycophenolic acid drug monitoring in patients with systemic sclerosis associated with diffuse skin and/or pulmonary involvement: A monocentric and retrospective French study

Journal of Scleroderma and Related Disorders ◽

10.1177/2397198320944342 ◽

2020 ◽

pp. 239719832094434

Author(s):

Paul Legendre ◽

Benoit Blanchet ◽

Raphael Porcher ◽

Alice Bérezné ◽

Marie Allard ◽

...

Keyword(s):

Carbon Monoxide ◽

Mycophenolate Mofetil ◽

Systemic Sclerosis ◽

Mycophenolic Acid ◽

Vital Capacity ◽

Area Under The Curve ◽

Skin Involvement ◽

Skin Score ◽

Lung Capacity ◽

Modified Rodnan Skin Score

Objective: To explore pharmacokinetic/pharmacodynamic relationship between mycophenolic acid area under the curve and clinical response at 1 year on skin involvement or interstitial lung disease in patients with systemic sclerosis. Method: Retrospective, monocentric study based on French Scleroderma Database in patients receiving mycophenolate mofetil who experienced a limited sampling strategy to estimate individual mycophenolic acid area under the curve plus two pulmonary function tests and skin evaluation after 1 month and 1 year. Efficacy criterions were variations of modified Rodnan skin score, forced vital capacity, and diffusing lung capacity for carbon monoxide at 1 year. Results: We included 52 patients; mean age was 49 years (range 17–79), and 36 (69%) were females. Fifty patients (96%) had skin sclerosis, 39 (75%) had diffuse skin involvement with a median modified Rodnan skin score of 14 (0–38). Thirty-eight (76%) had interstitial lung disease, with median forced vital capacity and diffusing lung capacity for carbon monoxide of 81% (37–127) and 56% (28–103) from predicted values, respectively. Twenty-five (51%) patients had pulmonary fibrosis. Mycophenolate mofetil was given for 10 months (0–173) at a median dose of 2000 mg/day (500–3000). In the entire population, no relationship was found between area under the curve and modified Rodnan skin score (p = 0.085), forced vital capacity (p = 0.80), or diffusing lung capacity for carbon monoxide (p = 0.72) variations at 1 year. Conclusion: In this retrospective study, we failed to document any relationship between mycophenolic acid area under the curve and skin involvement or interstitial lung disease evolution. Routine monitoring of mycophenolic acid in systemic sclerosis patients treated with mycophenolate mofetil cannot be recommended based on our results.

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Development of a Modified Hand Mobility in Scleroderma (HAMIS) Test and its Potential as an Outcome Measure in Systemic Sclerosis

The Journal of Rheumatology ◽

10.3899/jrheum.140286 ◽

2014 ◽

Vol 41 (11) ◽

pp. 2186-2192 ◽

Cited By ~ 9

Author(s):

Gunnel Sandqvist ◽

Jan-Åke Nilsson ◽

Dirk M. Wuttge ◽

Roger Hesselstrand

Keyword(s):

Systemic Sclerosis ◽

Construct Validity ◽

Disease Duration ◽

Skin Involvement ◽

Skin Score ◽

Left Hand ◽

Finger Flexion ◽

Modified Rodnan Skin Score ◽

The Right ◽

Dorsal Extension

Objective.To modify the hand mobility in scleroderma (HAMIS) test by reducing the number of items and amount of equipment needed, and to evaluate the construct validity of this modified HAMIS (mHAMIS).Methods.Our retrospective study is based on 266 patients previously examined using the original HAMIS test. Data were divided into 3 groups depending on disease duration after onset: (1) 0–3 years, (2) 3.1–5 years, and (3) 5.1–9 years. Disease variables included were skin involvement using the disease subset and the modified Rodnan skin score (mRSS), and digital lesions. Cronbach’s alpha coefficient was calculated separately for limited (lcSSc) and diffuse systemic sclerosis (dcSSc) for the right and left hand, and for the groups with different disease duration. The construct validity of the mHAMIS was assessed by searching for a correlation with hand skin score.Results.An mHAMIS test consisting of finger flexion, finger extension, finger abduction, and dorsal extension was developed. The internal consistency of this test was 0.78, 0.83, and 0.73 in the 3 groups with different disease duration. In the whole study group, mHAMIS showed a significant correlation with mRSS and hand skin score (rs = 0.39 and 0.43, respectively), and was able to discriminate between lcSSc and dcSSc (p = 0.001), and between patients with and without ulcers (p = 0.015).Conclusion.The mHAMIS involves 4 easily measurable items and has the potential to be a relevant clinical measure of outcome in the evaluation of fibrotic skin involvement in SSc.

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Plasma Hsp90 levels in patients with systemic sclerosis and relation to lung and skin involvement: a cross-sectional and longitudinal study

Scientific Reports ◽

10.1038/s41598-020-79139-8 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Hana Štorkánová ◽

Sabína Oreská ◽

Maja Špiritović ◽

Barbora Heřmánková ◽

Kristýna Bubová ◽

...

Keyword(s):

Systemic Sclerosis ◽

Vital Capacity ◽

Pulmonary Function Tests ◽

Healthy Controls ◽

Skin Involvement ◽

Cross Sectional ◽

Reactive Protein ◽

Modified Rodnan Skin Score ◽

Hsp 90 ◽

Sectional Analysis

AbstractOur previous study demonstrated increased expression of Heat shock protein (Hsp) 90 in the skin of patients with systemic sclerosis (SSc). We aimed to evaluate plasma Hsp90 in SSc and characterize its association with SSc-related features. Ninety-two SSc patients and 92 age-/sex-matched healthy controls were recruited for the cross-sectional analysis. The longitudinal analysis comprised 30 patients with SSc associated interstitial lung disease (ILD) routinely treated with cyclophosphamide. Hsp90 was increased in SSc compared to healthy controls. Hsp90 correlated positively with C-reactive protein and negatively with pulmonary function tests: forced vital capacity and diffusing capacity for carbon monoxide (DLCO). In patients with diffuse cutaneous (dc) SSc, Hsp90 positively correlated with the modified Rodnan skin score. In SSc-ILD patients treated with cyclophosphamide, no differences in Hsp90 were found between baseline and after 1, 6, or 12 months of therapy. However, baseline Hsp90 predicts the 12-month change in DLCO. This study shows that Hsp90 plasma levels are increased in SSc patients compared to age-/sex-matched healthy controls. Elevated Hsp90 in SSc is associated with increased inflammatory activity, worse lung functions, and in dcSSc, with the extent of skin involvement. Baseline plasma Hsp90 predicts the 12-month change in DLCO in SSc-ILD patients treated with cyclophosphamide.

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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

Indonesian Journal of Rheumatology ◽

10.37275/ijr.v11i2.133 ◽

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.

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

Biomarkers ◽

10.1080/1354750x.2019.1587509 ◽

2019 ◽

Vol 24 (4) ◽

pp. 373-378 ◽

Cited By ~ 1

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

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A Curl-Based Approach to Ultrasound Elastography

Volume 2: Biomedical and Biotechnology Engineering ◽

10.1115/imece2010-39180 ◽

2010 ◽

Cited By ~ 1

Author(s):

Ali Baghani ◽

Reza Zahiri Azar ◽

Septimiu Salcudean ◽

Robert Rohling

Keyword(s):

Imaging Modality ◽

Ultrasound Elastography ◽

Frame Rate ◽

Tissue Elasticity ◽

Inversion Algorithm ◽

Direct Inversion ◽

The Past ◽

Important Benefit ◽

Clinical Interest ◽

Tissue Elastography

The past two decades have witnessed the development of a new medical imaging modality: tissue elastography. The contrast in the images produced by an elastography system is based on the tissue elasticity, hence these images are called elastograms. Tissue elasticity is of clinical interest, because it is often correlated with pathology [1]. Different approaches to tissue elastography have emerged [2, 3]. In this article we report a tissue elastography system and its implementation on an ultrasound machine which provides consistent elastograms of a commercial quality assurance elastography phantom. The system uses our previously developed high frame rate sequencing and phase compensation techniques to measure axial and lateral motions at a typical frame rate of 1.25 kHz [4]. The system uses the curl of the displacements in a direct inversion algorithm to reconstruct elasticity. The most important benefit of this method is that the obtained elastograms are not dependent on the boundary conditions or the shape, size or position of the exciter, and as a result, the elastograms have fewer artifacts originating from these factors. The curl of the displacement has been used in magnetic resonance elastography (MRE) before, together with the direct inversion of the wave equation [5] and promising results have been obtained.

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