Hands and feet radiologic involvements in systemic sclerosis

Author(s):  
Fatemeh Badiee ◽  
Alireza Fatemi ◽  
Reza Zahedpasha ◽  
Hadi Gharib ◽  
Mohammadhassan Jokar ◽  
...  

Abstract Aim Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by vascular and fibrosing involvement of the skin and internal organs. In this study we determined the prevalence and characteristics of radiological hands and feet involvements in Iranian SSc patients identified disease–phenotype associations. Methods 43 SSc patients (41 women and 2 men), with a median age of 44.79 years (ranges 26 to 70 years) and a mean disease duration of 11.78 years (ranges 2 to 28 years) were studied in this cross-sectional study. Results 42 patients had radiological changes both in hands and feet. Only one patient had changes just in hand. The most frequent changes that we found in hand was Juxta-articular Osteoporosis (93%), Acro-osteolysis (58.2%), Joint Space Narrowing (55.8%). The prevalence of joint space narrowing or acro-osteolysis were higher in subjects with active skin involvement (modified Rodnan skin score (mRSS)>14) (16/21 v 4/16 for patients with inactive skin involvement (mRSS<14); p = 0.002). The most frequent changes that we found in foot were Juxta-articular Osteoporosis (93%), Acro-osteolysis (46.5%), Joint Space Narrowing (58.1%), subluxation (44.2%). The presence of anti-ccp antibody was detected in 4 (9.3%), while positive rheumatoid factor was found in 13 (30.2%) of SSc patients. Conclusion This study corroborates that an arthropathy is common in SSc patients. The introduction of the specific radiological involvements of SSc needs to be confirmed by further studies. in order to define the appropriate prognosis and treatment of patients.

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 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1604.1-1604
Author(s):  
D. Temiz Karadağ ◽  
O. Gundogdu ◽  
G. Lettieri ◽  
M. C. Padula ◽  
A. Padula ◽  
...  

Background:Previous studies have shown that Optical Coherence Tomography (OCT) is a reliable biomarker of skin fibrosis and significantly correlates with the severity of the skin involvement in Systemic Sclerosis (SSc)1,2.Objectives:Aim of this cross-sectional study was to evaluate the performance of skin OCT to discriminate between SSc and healthy controls (HC) and to compare results with the current gold standard, the modified Rodnan skin score (mRss), in a different SSc study cohort.Methods:Dorsal forearm skin of consecutive diffuse cutaneous SSc (dcSSc) patients and matched-HC was scanned by an investigator blinded to the clinical data using Vivosight scanner (Michelson Diagnostics, Kent, UK). Minimum Optical Density (MinOD), Maximum OD (MaxOD) and OD at 300 micron-depth (OD300) were measured. Clinical involvement was assessed by a blinded operator using the mRss and results were compared with imaging data. Statistical analysis was performed using GraphPad Prism software V.7.0.Results:A total of 88 OCT images were obtained from 22 dcSSc patients [20 Female, mean age 49 (±11) years, 12 with < 5 years disease duration) and 22 HC (20 Female, mean age 50.7 (±6.7) years]. All OCT measures (MinOD, MaxOD and OD300) were significantly lower in SSc patients than in HC (p=0.011, p<0.0001, p<0.0001 respectively). MaxOD and OD300 were significantly different between the four groups (0-3) of patients based on the mRss at the site of analysis (p=0.035, p=0.001 respectively). Skin OCT showed a good performance in discriminating SSc skin vs HC (overall AUC 0.72, 0.8 and 0.89 for MinOD, MaxOD and OD300 respectively).Conclusion:These results confirm in a cohort different from those of the previous studies that skin OCT is able to reflect the severity of skin involvement in SSc. Longitudinal studies are needed to validate its potential as surrogate outcome measure of skin fibrosis in SSc patients.References:[1]Abignano G et al. Ann Rheum Dis 2013; 2. Pires NSM et al. Ann Rheum Dis 2018.Disclosure of Interests:None declared


2020 ◽  
Vol 66 (1) ◽  
pp. 48-54
Author(s):  
Juliana Markus ◽  
Rogério de Melo Costa Pinto ◽  
Abadia Gilda Buso Matoso ◽  
Roberto Ranza

SUMMARY INTRODUCTION Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.


2018 ◽  
Vol 9 (2) ◽  
Author(s):  
Vincent Vincent ◽  
Sumartini Dewi ◽  
Rachmat Gunadi Wachjudi

Introduction: Systemic Sclerosis (SSc) is a chronic autoimmune disease, characterized by vasculopathy, specific autoimmune, and fibrosis. Assesment of skin fibrosis by modified Rodnan Skin Score (mRSS) can not detect the minimal changes of skin fibrosis within lessthan 3 months. A biomarker is needed to assess the minimal changes of skin fibrosis progressivity with a more objective, quantitative, and rapid way. Procollagen type-1 N-Terminal Propeptide (P1NP), a degradation product of collagen type-1, may become a potential biomarker for skin fibrosis. This study aims to evaluate the correlation between skin fibrosis by mRSS with P1NP serum in systemic sclerosis.Methods: This was a cross-sectional study performed among systemic sclerosis patients at Rheumatology outpatient clinic, Dr.Hasan Sadikin Hospital Bandung, from May 2016 to July 2016. Skin fibrosis was measured by mRSS. P1NP level was determined by ELISA. Data were analyzed using Rank-Spearman Correlation.Result: There were thirty-seven subjects, with mean age 37 (SD ±7) years old. Most of subjects were female (91.9%). Subjects consisted of 23 (62.2%) limited SSc and 14 (37.8%) diffuse SSc. Six subjects (16.2%) were DMARD naïve. We found median (range) P1NP serum was 43.85 (9.81-127.90) ng/dL, while the median of MRSS was 14 (3-36). There is a moderate correlation between MRSS and P1NP serum (r=0.443, p=0.003)Conclusion: There was a significant correlation between mRSS and P1NP serum in systemic sclerosis patient at Dr. Hasan Sadikin Hospital Bandung.Keywords: systemic sclerosis, P1NP, modified rodnan skin score


2018 ◽  
Vol 27 (6) ◽  
pp. 537-542 ◽  
Author(s):  
Gizem Cengiz ◽  
Kemal Erol ◽  
Kevser Gok ◽  
Salih Ozgocmen

Objective: The aim of the study was to compare characteristics of pain in terms of neuropathic pain (NeP) and to assess the association between the neuropathic component and quality of life (QoL) in patients with systemic sclerosis (SSc) and rheumatoid arthritis (RA). Subjects and Methods: Fifty-four patients (47 females, 7 males) with SSc and 53 patients (46 females, 7 males) with RA were assessed for outcome measures including disease activity, physical functions, mental condition and health-related QoL (HRQoL) measures (Short Form-36; Hospital Anxiety and Depression Scale), and pain. NeP was assessed by the Douleur Neuropathique 4 (DN4) and PainDetect questionnaires in this cross-sectional study. Results: The patients had similar education, smoking status, functioning, and HRQoL. However, the patients with RA declared a more severe visual analogue scale of pain and a higher BMI than those with SSc. The NeP component was detected in 42.6% (n = 23) of the SSc patients and in 45.3% (n = 24) of the RA patients (p > 0.05) according to DN4. On PainDetect, possible NeP was detected in 13.0% (n = 7) versus 15.1% (n = 8), whereas 16.7% (n = 9) versus 17.0% (n = 9) were likely to have NeP in SSc and RA, respectively (p > 0.05). Most of the NeP characteristics were similar in SSc and RA, except for numbness and painful cold, which were notably more common in patients with SSc. Having the NeP component (according to DN4) had no influence on functioning and HRQoL in SSc; however, the NeP component revealed a heavier burden of disease regarding functional status, HRQoL, and psychometric components in RA. Conclusion: The NeP component was similar between patients with SSc and RA. However, NeP was associated with a heavier burden of disease in patients with RA.


2021 ◽  
Vol 12 (1) ◽  
pp. 265
Author(s):  
Paweł Żebryk ◽  
Jan Krzysztof Nowak ◽  
Tomasz Piorunek ◽  
Tatiana Mularek-Kubzdela ◽  
Mariusz Puszczewicz

Vitamin D exhibits immunomodulatory effects in autoimmune diseases. We aimed to evaluate the associations of vitamin D levels with clinical and laboratory features of systemic sclerosis (SSc) in a Polish cohort. The study was prospective in design. SSc patients who met ACR-EULAR 2013 criteria underwent comprehensive clinical and laboratory investigations using the European Scleroderma Trials and Research group (EUSTAR) methodology. We assessed patients’ sera for 25(OH)D3 using a radioimmunoassay, and the cutoff value for vitamin D deficiency was set at 20 ng/mL. Statistical analyses were performed using the Mann–Whitney U test, the Fisher’s exact, and the Spearman’s rho, where appropriate, with a significance threshold set at 0.05. We recruited 68 SSc patients (85% female). The mean 25(OH)D3 level was 21.6 ± 10 ng/mL, and 50% of subjects (n = 34) presented vitamin D deficiency (mean 13.7 ± 3.9 ng/mL). Vitamin D-deficient SSc patients exhibited higher prevalence of arterial hypertension (p = 0.002), proteinuria (p = 0.002), and lung fibrosis (p = 0.032), as well as higher CRP (p = 0.035). The modified Rodnan skin score correlated negatively with 25(OH)D3 in diffuse cutaneous SSc (dcSSc). We found no correlation with the disease duration, age, joints, and the heart. Vitamin D deficiency was common in the studied population of Polish SSc patients and was associated with arterial hypertension, proteinuria, lung involvement, and increased CRP.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Hana Štorkánová ◽  
Sabína Oreská ◽  
Maja Špiritović ◽  
Barbora Heřmánková ◽  
Kristýna Bubová ◽  
...  

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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