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Extracellular BAG3 is elevated in early diffuse systemic sclerosis 认领 引用
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作者 Paul Freedman Margot De Marco +4 位作者 Alessandra Rosati Liberato Marzullo Nicoletta Del Papa Maria Caterina Turco Steven O’Reilly 《Military Medical Research》 SCIE CAS CSCD 2026年第1期169-172,共4页
Dear Editor,Systemic sclerosis(SSc)is an autoimmune connective tissue disease in which there are vascular abnormalities,inflammation,and fibrosis[1].These three characteristics primarily affect the skin and lungs.Of a... Dear Editor,Systemic sclerosis(SSc)is an autoimmune connective tissue disease in which there are vascular abnormalities,inflammation,and fibrosis[1].These three characteristics primarily affect the skin and lungs.Of all the autoimmune rheumatic diseases,SSc has the highest all-cause mortality rate,and the underlying pathogenic processes that mediate disease are still obscure,with wide diff erences in presentation and progression[2,3]. 展开更多
关键词 Systemic sclerosis Bcl-2 associated athanogene-3(BAG3) Cytokine Lung disease Stimulator of interferon genes(STING)
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Investigation of stressful life events in patients with systemic sclerosis 认领 引用 被引量：1
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作者 Yue CHEN Ji-zhong HUANG +2 位作者 Yu QIANG Jin WANG Mao-mao HAN 《Journal of Zhejiang University-SCIENCE B》 SCIE CAS 2008年第11期853-856,共4页
Objective: To assess the occurrence of stressful life events in the year before the initiation of systemic sclerosis. Methods: A consecutive series of 40 patients with systemic sclerosis (mean age (56.3±11.9) yea... Objective: To assess the occurrence of stressful life events in the year before the initiation of systemic sclerosis. Methods: A consecutive series of 40 patients with systemic sclerosis (mean age (56.3±11.9) years, mean disease duration (4.3±3.1) years; 32 females and 8 males), including 28 with diffuse cutaneous scleroderma and 12 with limited cutaneous scleroderma, were evaluated. A control group of 40 healthy subjects free of systemic sclerosis also was included. Socioeconomic status was inves- tigated and Paykel's interview for recent life events (a semi-structured research interview covering 64 life events) was conducted. Results: Patients with systemic sclerosis showed higher percentages of lower education (72.5%) and working class (82.5%), and reported more stressful life events (P<0.05), such as exits (P<0.05), undesirable events (P<0.01), and uncontrolled events (P<0.001), when compared with the control. More events that had an objective negative impact (P<0.001) were also reported in systemic sclerosis patients than in the control. These results are in accordance with a multifactorial model of pathogenesis in systemic sclerosis. Conclusion: We reported a strong relationship between stressful life events and the initiation of systemic sclerosis. Our findings are consistent with current understanding of the extensive links of behavioral responses to stress with neurophysiological and biochemical processes. 展开更多
关键词 Systemic sclerosis （SSc） Life events Stress Pathogenesis
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Expression of MMP-9 and TIMP-1 in Lesions of Systemic Sclerosis and Its Implications 认领 引用 被引量：3
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作者 孟赤 陈旭娥 +2 位作者 李家文 吴艳 刘厚君 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 2008年第4期480-482,共3页
In order to investigate the role of MMP-9 and TIMP-1 in the pathogenesis of systemic sclerosis, the expression of MMP-9 and TIMP-1 was immunohistochemically detected in skin lesions of the patients with diffuse cutane... In order to investigate the role of MMP-9 and TIMP-1 in the pathogenesis of systemic sclerosis, the expression of MMP-9 and TIMP-1 was immunohistochemically detected in skin lesions of the patients with diffuse cutaneous systemic sclerosis, skin lesions of the patients with limited cutaneous systemic sclerosis, and skin tissues of normal subjects. The results showed that the expression of MMP-9 in lesions of diffuse cutaneous systemic sclerosis was significantly lower than that of normal skins （P〈0.05）. However, no significant difference in the level of MMP-9 in the limited cutaneous systemic sclerosis and normal skin was found. Meanwhile, the expression of TIMP-1 in lesions of diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis were significantly higher than that of normal skins （both P〈0.05）. It was suggested that the expression of MMP-9 and TIMP-1 might play an important role in the development of systemic sclerosis. 展开更多
关键词 MMP-9 TIMP-1 systemic sclerosis
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Adipose-derived stem cells:Pathophysiologic implications vs therapeutic potential in systemic sclerosis 认领 引用 被引量：6
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作者 Irene Rosa Eloisa Romano +2 位作者 Bianca Saveria Fioretto Marco Matucci-Cerinic Mirko Manetti 《World Journal of Stem Cells》 SCIE 2021年第1期30-48,共19页
Adipose-derived stem cells(ADSCs)residing in the stromal vascular fraction(SVF)of white adipose tissue are recently emerging as an alternative tool for stem cell-based therapy in systemic sclerosis(SSc),a complex conn... Adipose-derived stem cells(ADSCs)residing in the stromal vascular fraction(SVF)of white adipose tissue are recently emerging as an alternative tool for stem cell-based therapy in systemic sclerosis(SSc),a complex connective tissue disorder affecting the skin and internal organs with fibrotic and vascular lesions.Several preclinical and clinical studies have reported promising therapeutic effects of fat grafting and autologous SVF/ADSC-based local treatment for facial and hand cutaneous manifestations of SSc patients.However,currently available data indicate that ADSCs may represent a double-edged sword in SSc,as they may exhibit a pro-fibrotic and anti-adipogenic phenotype,possibly behaving as an additional pathogenic source of pro-fibrotic myofibroblasts through the adipocyte-to-myofibroblast transition process.Thus,in the perspective of a larger employ of SSc-ADSCs for further therapeutic applications,it is important to definitely unravel whether these cells present a comparable phenotype and similar immunosuppressive,anti-inflammatory,anti-fibrotic and pro-angiogenic properties in respect to healthy ADSCs.In light of the dual role that ADSCs seem to play in SSc,this review will provide a summary of the most recent insights into the preclinical and clinical studies employing SVF and ADSCs for the treatment of the disease and,at the same time,will focus on the main findings highlighting the possible involvement of these stem cells in SSc-related fibrosis pathogenesis. 展开更多
关键词 Systemic sclerosis Adipose-derived stromal vascular fraction Adiposederived stem cells Therapeutic approaches Pathogenesis Adipocyte-to-myofibroblast transition
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Is spleen circulation impaired in systemic sclerosis and what is the role of liver fibrosis? 认领 引用 被引量：1
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作者 Giovanni Tarantino Angelo Spanò +5 位作者 Gabriella Loi Anna Parisi Marianna Tarantino Giuseppina Brancaccio Giovanni Battista Gaeta Antonio Riccio 《World Journal of Gastroenterology》 SCIE CAS CSCD 2011年第12期1606-1613,共8页
AIM:To investigate the spleen vascular involvement and the presence of liver fibrosis in a population of subjects with established systemic sclerosis(SSc).METHODS:In a cross-sectional fashion,17 patients with SSc were... AIM:To investigate the spleen vascular involvement and the presence of liver fibrosis in a population of subjects with established systemic sclerosis(SSc).METHODS:In a cross-sectional fashion,17 patients with SSc were compared with 18 patients suffering from hepatitis C virus(HCV) -related liver cirrhosis,grade A and B Child-Pugh classification.Eighteen non elderly subjects,apparently healthy,were used as the control group.Splenic artery resistivity index(SARI) at doppler ultraSound,transient elastography of liver and nailfold capillaroscopy were the main outcomes.RESULTS:Transient elastography values of SSc patients were similar to those of controls;5.2±1.1 vs 4.5 ±1,(P=0.07).Median Alanine amino transferase(ALT) concentrations of cirrhotic patients were greater than those of controls and SSc patients,i.e.66.5(36-89) U/L vs 29(22-34) U/L and 31(22-41) U/L,respectively,(P =0.005).SARI determinations in cirrhotic patients,although significantly higher than those found in controls and SSc patients,showed some degree of overlap with SSc patients,i.e.0.59 vs 0.52 and 0.57,respectively,(P =0.04).Mean systolic blood pressure was significantly higher in SSc patients than in cirrhotics and controls,i.e.142 mmHg vs 128.2 mmHg and 127 mmHg,respectively,(P=0.005).Mean diastolic blood pressure behaved in a similar fashion,i.e.84 mmHg vs 72.2 mmHg and 76.9 mmHg(P=0.005).Nailfold Capillaroscopy grades and diastolic blood pressure values correlated well with SARI results.CONCLUSION:An enhanced resistivity of the splenic artery was found in patients suffering from SSc;they did not have evidence of splenomegaly as well as no liver fibrosis or any other form of liver damage. 展开更多
关键词 Splenic artery resistivity index Doppler ultrasound Transient elastography Nailfold capillaroscopy Systemic sclerosis Liver fibrosis
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Gastrointestinal manifestations of systemic sclerosis:An updated review 认领 引用 被引量：2
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作者 Adan Luquez-Mindiola Alexis Javier Atuesta Andres Jose Gómez-Aldana 《World Journal of Clinical Cases》 SCIE 2021年第22期6201-6217,共17页
Systemic sclerosis is an autoimmune disease characterized by vascular disease,fibrosis of the skin,and internal organ dysfunction.Gastrointestinal involvement is the most frequent complication of internal organs,impac... Systemic sclerosis is an autoimmune disease characterized by vascular disease,fibrosis of the skin,and internal organ dysfunction.Gastrointestinal involvement is the most frequent complication of internal organs,impacting up to 90%of patients.Gastrointestinal involvement can affect any region of the gastrointestinal tract from the mouth to the anus,with a predominance of disorders being observed at the level of the upper digestive tract.The gastrointestinal involvement primarily involves the esophagus,small bowel,and rectum.The severity of gastrointestinal involvement affects quality of life and is a marker of worse prognosis and mortality in these patients.In this review,we describe the current findings regarding gastrointestinal involvement by this entity. 展开更多
关键词 Systemic sclerosis Gastrointestinal Diagnosis Treatment
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Transforming growth factor-β signaling in systemic sclerosis 认领 引用 被引量：7
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作者 Nolan B.Ayers Chenming Sun Shi-You Chen 《Journal of Biomedical Research》 CAS CSCD 2018年第1期3-12,共10页
Systemic sclerosis（SSc） is a complex, multiorgan autoimmune disease of unknown etiology. Manifestation of the disease results from an interaction of three key pathologic features including irregularities of the anti... Systemic sclerosis（SSc） is a complex, multiorgan autoimmune disease of unknown etiology. Manifestation of the disease results from an interaction of three key pathologic features including irregularities of the antigen-specific immune system and the non-specific Immune system, resulting in autoantibody production, vascular endothelial activation of small blood vessels, and tissue fibrosis as a result of fibroblast dysfunction. Given the heterogeneity of clinical presentation of the disease, a lack of universal models has impeded adequate testing of potential therapies for SSc. Regardless, recent research has elucidated the roles of various ubiquitous molecular mechanisms that contribute to the clinical manifestation of the disease. Transforming growth factor β（TGF-β） has been identified as a regulator of pathological fibrogenesis in SSc. Various processes, including cell growth, apoptosis, cell differentiation, and extracellular matrix synthesis are regulated by TGF-β,a type of cytokine secreted by macrophages and many other cell types. Understanding the essential role TGF-β pathways play in the pathology of systemic sclerosis could provide a potential outlet for treatment and a better understanding of this severe disease. 展开更多
关键词 systemic sclerosis transforming growth factor-β mechanism therapeutics
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Exploring the treatment of Systemic Sclerosis with Danggui (Angelica sinensis, AS)-Sini Decoction using GEO combined with Network pharmacology and Molecular docking 认领 引用 被引量：1
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作者 Hui-Mei Shi Xin-Li +5 位作者 Bei Jing Ya-Chun Zheng Di Zhang Shi-Quan Chang Zhen-Ni Chen Guo-Ping Zhao 《Medical Data Mining》 2022年第1期34-46,共13页
Background:To explore the relevant targets of the Chinese herbal medicine compound Danggui-Sini Decoction(DGSND)for the treatment of systemic sclerosis(SSc).Method:We used TCMSP to enlist ingredients and Swiss Target ... Background:To explore the relevant targets of the Chinese herbal medicine compound Danggui-Sini Decoction(DGSND)for the treatment of systemic sclerosis(SSc).Method:We used TCMSP to enlist ingredients and Swiss Target Prediction for targets fishing,and the protein names by UniProt for organized as gene symbol.Strawberry Perl was used to integrate the active ingredients and the drugs action target of DGSND,with Cytoscape 3.9.0 software to construct"Active ingredient-Drug target"network.Then,GEO database,GeneCards database,TTDdatabase,DisGENent database and MalaCards database for SSc-related disease target prediction,and then analyzed the active drug targets of DGSND and SSc-related targets of Danggui-Sini Decoction treat SSc.Then,the above results we combined with STRING database to visualize the Protein-protein interaction(PPI)network for the core targets of SSc,and performed Gene ontology(GO)functional analysis and Kyoto Encyclopedia of Genomics(KEGG)signaling pathway enrichment analysis for SSc-related targets treated with DGSND Result:DGSND contains 223 active ingredients including Sitogluside,Benzoylpaeoniflorin,(-)-Asarinin Palbinone,Glycyro,4'DMEP etc.which acts on Signal transducer and activator of transcription 3(STAT3),Tumor Necrosis Factor(TNF),Vascular endothelial growth factor(VEGFA),Nuclear factor kappa-B(NFκB1),Interleukin(IL1β,IL17),Mitogen-activated protein kinase(MAPK)and Janus Kinase(JAK)and many other genes totaling 176,mainly involved in 4 more biological processes 3 molecular functions and 3 cellular components.Conclusion:DGSND is primarily used to treat SSc by regulating calcium homeostasis,inflammatory signaling pathways and neural cell repair and apoptosis-related pathways within the body. 展开更多
关键词 Danggui-Sini Decoction systemic sclerosis network pharmacology
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Cardiac manifestations in systemic sclerosis 认领 引用 被引量：6
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作者 Sevdalina Lambova 《World Journal of Cardiology》 2014年第9期993-1005,共13页
Primary cardiac involvement, which develops as a direct consequence of systemic sclerosis(SSc), may manifest as myocardial damage, fibrosis of the conduction system, pericardial and, less frequently, as valvular disea... Primary cardiac involvement, which develops as a direct consequence of systemic sclerosis(SSc), may manifest as myocardial damage, fibrosis of the conduction system, pericardial and, less frequently, as valvular disease. In addition, cardiac complications in SSc may develop as a secondary phenomenon due to pulmonary arterial hypertension and kidney pathology. The prevalence of primary cardiac involvement in SSc is variable and difficult to determine because of the diversity of cardiac manifestations, the presence of subclinical periods, the type of diagnostic tools applied, and the diversity of patient populations. When clinically manifested, cardiac involvement is thought to be an important prognostic factor. Profound microvascular disease is a pathognomonic feature of SSc, as both vasospasm and structural alterations are present. Such alterations are thought to predict macrovascular atherosclerosis over time. There are contradictory reports regarding the prevalence of atherosclerosis in SSc. According to some authors, the prevalence of atherosclerosis of the large epicardial coronary arteries is similar to that of the general population, in contrast with other rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus. However, the level of inflammation in SSc is inferior. Thus, the atherosclerotic process may not be as aggressive and not easily detectable in smaller studies. Echocardiography(especially tissue Doppler imaging), single-photon emission computed tomography, magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies. Screening for subclinical cardiac involvement via modern, sensitive tools provides an opportunity for early diagnosis and treatment, which is of crucial importance for a positive outcome. 展开更多
关键词 Systemic sclerosis Cardiac involvement
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Coexistence of systemic sclerosis and ankylosing spondylitis:A case report and literature review 认领 引用 被引量：1
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作者 HU Shulin ZUO Xiaoxia LI Yisha 《中南大学学报（医学版）》 CAS CSCD 北大核心 2018年第11期1263-1265,共3页
Systemic sclerosis(SSc)is an autoimmune disease characterized by thickening of the skin and organ fibrosis.Ankylosing spondylitis(AS)is a type of arthritis with long-term inflammation of the axial joints.Previous stud... Systemic sclerosis(SSc)is an autoimmune disease characterized by thickening of the skin and organ fibrosis.Ankylosing spondylitis(AS)is a type of arthritis with long-term inflammation of the axial joints.Previous studies presented 5 cases of concomitant AS and SSc.However,there was only 1 patient of those 5 cases complaining of muscle weakness while all patients had approximately normal creatine kinase(CK).Here we reported a young male who met the criteria for SSc and AS while showing significantly elevated CK.Human leukocyte antigen(HLA)typing results indicated the genetic susceptibility to these two diseases.The patient was prescribed prednisone(30mg/d)and cyclophosphamide.After 2 months,the patient's skin became soft with normal CK. 展开更多
关键词 systemic sclerosis ankylosing spondylitis creatine kinase
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Implications for the therapeutic role of Imatinib in systemic sclerosis: a single-arm meta-analysis 认领 引用
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作者 Qi Zhang Bo Zhang Nengming Lin 《Journal of Chinese Pharmaceutical Sciences》 CAS CSCD 2019年第3期195-202,共8页
Sclerotic chronic graft-versus-host disease (ScGVHD) or systemic sclerosis (SSc) is one of the most severe manifestations of chronic graft-versus-host disease (cGVHD) since the involvement of major organs significantl... Sclerotic chronic graft-versus-host disease (ScGVHD) or systemic sclerosis (SSc) is one of the most severe manifestations of chronic graft-versus-host disease (cGVHD) since the involvement of major organs significantly affects the mortality and morbidity of SSc patients. Currently, there are no effective therapeutic approaches or standard “second-line” therapy for SSc. Imatinib, a clinical tyrosine kinase inhibitor used to treat chronic myeloid leukemia and gastrointestinal tumor, has shown potential therapeutic effects in treating ScGVHD or SSc. Due to the current limitations of clinical trials using Imatinib in the treatment of SSc, the results vary among different studies, and the applications of Imatinib in SSc is still in vagueness. Here we conducted a single-arm meta-analysis to quantitatively and systematically interpret the results of previous studies, as to evaluate the potential therapeutic effect of Imatinib in SSc. The pooled clinical response rate (CRR) showed that Imatinib had higher CRR in SSc patients with longer disease duration (CRR = 0.550) and lung involvement (CRR = 0.601) than those without (P<0.001). Therefore, it was encouraging to con duct future clinical studies regarding Imatinib therapy in SSc patients, since the global response rate of Imatinib was higher than expected in specific subgroup patients. 展开更多
关键词 Imatinib Systemic sclerosis Sclerotic chronic graft-versus-host disease
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Detection of Ⅴ,Ⅲ and Ⅰ Type Collagens of Dermal Tissues in Skin Lesions of Patients with Systemic Sclerosis and Its Implication 认领 引用 被引量：4
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作者 刘彤 张键 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 2008年第5期599-603,共5页
This study investigated the contents and distribution of collagen Ⅴ （Col Ⅴ） in skin lesions of the patients with systemic sclerosis （SSc） and its roles in the pathogenesis. The contents and distribution for α1 ... This study investigated the contents and distribution of collagen Ⅴ （Col Ⅴ） in skin lesions of the patients with systemic sclerosis （SSc） and its roles in the pathogenesis. The contents and distribution for α1 chain of collagen type Ⅰ, Ⅲ and V [α1 （Ⅰ）, α1 （Ⅲ） and α1 （Ⅴ）] in skin lesions of 36 patients with SSc （9 cases of mild fibrosis, 14 moderate, and 13 severe） were detected by using im- munohistochemical SP method. Six cases of normal skin tissues served as controls. The results showed that there was diffuse distribution for three kinds of collagens in dermis. The deep staining α1 （Ⅰ） and α1 （Ⅲ） masses or bands were seen in reticular layer, while α1 （Ⅴ） was distributed more ho- mogeneously. From control to weak, moderate and severe fibrosis stages, α1 （Ⅰ）, α1 （Ⅲ） and α1 （V） showed a gradually increased trend in skin lesions （P〈0.05）. α1 （Ⅴ） was obviously elevated in skin lesions at early stage and persisted in whole fibrotic process and risen in greater contents, while α1 （Ⅰ） and α1 （Ⅲ） were to go higher late and were apparently elevated at moderate and late stages. Com- pared with α1 （Ⅰ）, α1 （Ⅴ） took leading increase at early stage in skin lesions （P〈0.01）, and had more elevated contents than α1 （Ⅲ） at moderate and late stages. The fibrotic changes in dermal reticular layer occurred earlier than those in papillary layer, and the abnormalities of α1 （Ⅴ）/α1 （I） ratio ap- peared before α1 （Ⅲ）/α1 （Ⅰ） ratio. It was concluded that a lot of α1 （Ⅴ） began to deposit in greater contents prior to α1 （Ⅰ） and α1 （Ⅲ） at early stage in SSc and persisted in whole fibrotic process. The changes of α1 （Ⅴ） contents in reticular layer occurred earlier than those in papillary layer, and it sug- gested that the fibrosis in reticular layer appeared earlier. 展开更多
关键词 sclerosis, systemic V type collagen immunohistochemical staining
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Research on hsa-miR-155-3p and hsa-miR-155-5p as biomarkers for systemic sclerosis and their role in regulating biological behavior 认领 引用
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作者 WANG Bao-yue SUN Xiao-lin WANG Yong-fu 《Journal of Hainan Medical University》 CAS 2024年第3期32-41,共10页
Objective: This study was to investigate the role of hsa-miR-155-3p and hsa-miR-155-5p as biomarkers and regulators of biological behavior in Systemic Sclerosis. Methods: A total of 10 SSc patients and 10 healthy cont... Objective: This study was to investigate the role of hsa-miR-155-3p and hsa-miR-155-5p as biomarkers and regulators of biological behavior in Systemic Sclerosis. Methods: A total of 10 SSc patients and 10 healthy controls were selected for the study. The expression levels of hsa-miR-155-3p and hsa-miR-155-5p in peripheral blood mononuclear cells of SSc patients and healthy controls were measured using RT-qPCR. The diagnostic value of these miRNAs was explored using Receiver Operating Characteristic curve analysis. Pearson or Spearman correlation analysis was performed to assess the correlation between miRNAs and clinical indicators in SSc patients. Potential target genes of hsa-miR-155-3p and hsa-miR-155-5p were predicted using miRDB, Targetscan, and miRDIP databases. GO functional annotation, KEGG pathway enrichment analysis, protein-protein interaction network construction, and selection of central genes were conducted. Results: The expression levels of hsa-miR-155-3p and hsa- miR-155-5p were significantly higher in PBMCs of SSc patients compared to healthy controls (P<0.001). The ROC curve analysis showed that hsa-miR-155-3p and hsa-miR-155-5p had a high diagnostic value for SSc (AUC=1, P<0.001). Correlation analysis revealed that hsa- miR-155-3p, hsa-miR-155-5p, and clinical indicators such as high-resolution CT, neutrophil percentage, lymphocyte percentage, and albumin to globulin ratio were correlated (P<0.05). The signaling pathways enriched with target genes of hsa-miR-155-3p and hsa-miR-155- 5p were closely associated with the occurrence and development of SSc fibrosis, immunity, and inflammation. Conclusions: hsa-miR-155-3p and hsa-miR-155-5p may be involved in regulating the occurrence and development of SSc fibrosis, immunity, and inflammation. They have the potential to serve as biomarkers for clinical diagnosis and treatment of SSc. 展开更多
关键词 miR-155-3p miR-155-5p Systemic sclerosis Correlation Biomarker
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Juvenile Systemic Sclerosis: About 9 Cases 认领 引用
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作者 Kaoutar Danaoui Houda Nassih +4 位作者 Khadija Oujennane Rabiy El Qadiry Aicha Bourrahouat Said Amal Imane Ait Sab 《Open Journal of Pediatrics》 2024年第2期320-326,共7页
Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutane... Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). Both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutaneous involvement in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening complications such as lung, heart, and visceral organ fibrosis, and vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but patients are susceptible to significant morbidity, leading to severe disfigurement and functional impairment. Treatment for scleroderma aims to control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts a retrospective descriptive analysis aiming to document the clinical manifestations, management approaches, and outcomes of systemic sclerosis in a cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric B department of Mohammed VI University, Hospital Center in Marrakech, Morocco. 展开更多
关键词 Scleroderma Systemic Sclerosis Children Pediatric
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Network pharmacological study and molecular docking verification of Capparis spinosa in the treatment of systemic sclerosis 认领 引用
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作者 Jia Guo Hua Bian +4 位作者 Yu Shan Jing-Jing Zhao Xiao-Wen Zhang Shuang Chen Bo Bian 《Journal of Hainan Medical University》 CAS 2022年第6期29-34,共6页
Objective:To explore the molecular mechanism of Capparis spinosa in the treatment of systemic sclerosis((SSC))based on network pharmacology.Methods:GEO,Genecards,Pharmgkb,TTD and Drugbank databases were used to obtain... Objective:To explore the molecular mechanism of Capparis spinosa in the treatment of systemic sclerosis((SSC))based on network pharmacology.Methods:GEO,Genecards,Pharmgkb,TTD and Drugbank databases were used to obtain SSC targets,related literatures and Swisstargetprediction databases were used to obtain the main components of Citrus and their corresponding targets,and intersection was used to obtain prediction targets.Log in to the String database to analyze the protein interaction of the prediction target(PPI),further used Cytoscape to obtain the core gene by network topology analysis,and the core gene was docked with the main components of Capparis spinosa.The prediction targets were analyzed by gene ontology(GO)analysis and Kyoto encyclopedia of genes and genomes(KEGG)pathway analysis using R software.Results:A total of 15 active components and their targets were obtained,3171 SSC targets were obtained,and 66 predicted targets were obtained by intersection.Ten PPI core genes such as VEGFA,TNF,AKT1,PTGS2 and MMP9 were obtained by topological analysis.GO analysis involved many biological processes such as reactive oxygen species metabolic process、protein kinase B signaling、regulation of inflammatory response、phosphatidylinositol 3-kinase signaling and so on.KEGG pathway analysis showed PI3K-Akt signaling pathway,Proteoglycans in cancer,Focal adhesion,Rap1 signaling pathway and other signaling pathways.Conclusion:The molecular mechanism of Capparis spinosa in the treatment of SSC is predicted by the method of network pharmacology,which provides theoretical basis and data support for the basic research of Citrus officinalis in the treatment of SSC. 展开更多
关键词 Capparis spinosa Systemic sclerosis Network pharmacology Molecular docking
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Lower Extremity Ulcers in Patients with Systemic Sclerosis 认领 引用
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作者 Marta B. Bean Chris T. Derk 《Open Journal of Rheumatology and Autoimmune Diseases》 2022年第1期35-45,共11页
Introduction: Cutaneous manifestations of systemic sclerosis (SSc) include skin ulceration;4% - 12% of patients with SSc develop lower extremity ulcers of various etiologies. Limited data, significant morbidity, and s... Introduction: Cutaneous manifestations of systemic sclerosis (SSc) include skin ulceration;4% - 12% of patients with SSc develop lower extremity ulcers of various etiologies. Limited data, significant morbidity, and substantial cost of wound care led us to undertake this study to describe and identify risk factors. Methods: After Institutional Review Board approval, we identified 30 patients with SSc and lower extremity ulcers over a 10-year period at a single center with an SSc clinic, which were included in a descriptive analysis. Results: Median age of onset of lower extremity ulcers was 59.5 years (range 20 - 84). Ninety percent of patients were female, 60% were Caucasian, 63% had limited SSc, 13% diffuse SSc and 23% an overlap syndrome. Immunomodulators or steroids were prescribed in 53%;hypercoagulable state identified in 16%. Ulcers were attributed to venous stasis (27%), SSc (20%), trauma (20%), arterial disease (17%), and multifactorial/unknown (17%). In patients with ulcers attributed to SSc, age at onset was lower (45.5 vs 59.5 years). Biopsies generally did not contribute to management. Multidisciplinary treatment was routine;20% required amputation, 10% endovascular intervention, 20% frequent surgical debridement, 10% hyperbaric oxygen, 26% local treatment and antibiotics and 13% received immunosuppression for wound treatment. Conclusion: Lower extremity ulcers are a serious clinical problem in patients with SSc. The clinical exam, venous dopplers, ankle-brachial indices and assessment of vascular risk factors helped define causality. In younger patients, ulcers were more frequently attributed to SSc and these patients were more likely to be on immunosuppressants/DMARDS, possibly indicating severe phenotype of SSc. 展开更多
关键词 Lower Extremity Ulcers Systemic Sclerosis Scleroderma Peripheral Vascular Disease Venous Stasis
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Cardiac disease in systemic sclerosis:Integrating pathobiology with clinical management 认领 引用
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作者 Henry Sutanto Betty Rachma Yuliasih 《Rheumatology & Autoimmunity》 2026年第1期16-38,共23页
Systemic sclerosis(SSc)is a complex autoimmune disorder in which cardiovascular involvement remains a major determinant of morbidity and mortality.Cardiac injury in SSc results from the interplay of microvascular dysf... Systemic sclerosis(SSc)is a complex autoimmune disorder in which cardiovascular involvement remains a major determinant of morbidity and mortality.Cardiac injury in SSc results from the interplay of microvascular dysfunction,immune-mediated inflammation,and progressive interstitial and replacement fibrosis,leading to myocardial disease,arrhythmias,pericardial abnormalities,and coronary microvascular ischemia.Many of these manifestations evolve silently,becoming clinically apparent only after substantial structural or electrical remodeling has occurred,highlighting the critical need for systematic surveillance.Current consensus algorithms recommend a core annual cardiovascular assessment—including symptom-directed clinical evaluation,electrocardiography,transthoracic echocardiography with tissue Doppler and strain imaging,and measurement of natriuretic peptides and high-sensitivity troponin—with escalation to cardiac magnetic resonance imaging,ambulatory rhythm monitoring,or stress testing when abnormalities are detected.Management integrates standard heart failure and arrhythmia therapies with judicious use of immunomodulation in patients with active inflammatory cardiomyopathy.However,evidence for targeted immunosuppressive treatment of primary cardiac involvement in SSc remains limited,and the optimal frequency,modality,and biomarker combinations for screening continue to be refined.This review synthesizes emerging mechanisms,diagnostic strategies,and therapeutic considerations,and outlines research priorities aimed at improving early detection and outcomes in cardiovascular manifestations of SSc. 展开更多
关键词 autoimmune disease cardiovascular disease rheumatology scleroderma systemic sclerosis
miRNA profiles in early systemic sclerosis,organ complications,and treatment response 认领 引用
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作者 Jing Huang Sijia Liu +4 位作者 Kun Wang Yisha Li Hui Luo Xiaoxia Zuo Honglin Zhu 《Chinese Medical Journal》 SCIE CAS CSCD 2026年第13期2048-2050,共3页
To the Editor:Systemic sclerosis(SSc)is a complex autoimmune disease characterized by immune dysfunction and excessive collagen deposition,leading to fibrosis across multiple organs and tissues.[1]Key features include... To the Editor:Systemic sclerosis(SSc)is a complex autoimmune disease characterized by immune dysfunction and excessive collagen deposition,leading to fibrosis across multiple organs and tissues.[1]Key features include skin thickening,Raynaud’s phenomenon(RP),and internal organ involvement with interstitial lung disease(ILD),pulmonary arterial hypertension,renal crisis,and myocardial involvement.Early diagnosis of SSc complications is crucial for effective management and improved patient outcomes,emphasizing the necessity of research to identify robust diagnostic indicators. 展开更多
关键词 immune dysfunction internal organ involvement systemic sclerosis early diagnosis miRNA profiles organ complications interstitial lung disease ild pulmonary skin thickeningraynaud s
Predictors of systemic sclerosis patients with interstitial lung disease progression:A retrospective study 认领 引用 被引量：1
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作者 Xi Cao Shanshan Wang +4 位作者 Wenyi Yuan Mian Liu Xiaoyan Xin Xiang Fang Dandan Wang 《Rheumatology & Autoimmunity》 2025年第3期206-216,共11页
Background:Lung involvement is one of the most common and serious internal organ damages in systemic sclerosis(SSc).The aim of this study was to establish a prediction model to assess the risk factors of progression i... Background:Lung involvement is one of the most common and serious internal organ damages in systemic sclerosis(SSc).The aim of this study was to establish a prediction model to assess the risk factors of progression in SSc patients with interstitial lung disease(ILD),thereby assisting clinicians in the early identification of high‐risk patients.Methods:Eligible patients were registered in our database of adult patients hospitalized in the Rheumatology Department of the Nanjing Drum Tower Hospital from January 1,2013 to January 1,2022,and the overall cohort was divided into the derivation cohort and the internal validation cohort.ILD progression was defined as deteriorating symptoms and the extent of lung fibrosis on follow‐up high‐resolution computed tomography.A prediction nomogramfor ILD progression was established in the derivation cohort by least absolute shrinkage and selection operator and multivariable logistic regression and validated in the internal validation cohort.The total points of the nomogram were calculated to delineate high‐risk and low‐risk groups.The Kaplan‐Meier curve was used to determine the association between SSc patients with or without ILD progression,as well as between high‐risk and low‐risk groups.Results:We included 273 patients,227(83.2%)were females and 46(16.8%)were males,the mean age was(45.1±15.5)years,the disease duration was 3(25th–75th percentiles:1–8)years,126 of whom developed ILD progression during follow‐up(1–5 years).The final SSc‐ILD progression prediction model was composed of six significant risk factors:male,type 2 diabetes mellitus,pericardial effusion,anti‐Ro52,hypoproteinemia,and elevated C‐reactive protein.An SSc‐ILD progression probability‐predictive nomogram was established.In two cohorts,the AUCs were 0.832(95%confidence interval[CI]:0.774–0.890)and 0.818(95%CI:0.722–0.914),respectively.The calibration curve,decision curve analysis,and clinical impact curve all showed good performance of the model.The Kaplan–Meier curve suggested that the occurrence of ILD progression was associated with poor prognosis,and the curve of high‐risk and low‐risk groups defined by our model demonstrated excellent prognostic utility.Conclusion:This new model demonstrated good capability for assessing the risk in SSc‐ILD progression,which may be helpful for early identification and intervention. 展开更多
关键词 inflammation interstitial lung disease nomogram progression risk prediction model systemic sclerosis
Do the incidence and severity of lower urinary tract symptoms measured by the International Consultation on Incontinence Questionnaires correlate with urodynamic findings in patients with systemic sclerosis? 认领 引用
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作者 Amr Abou Faddan Manal Hassanien +1 位作者 Esraa Ahmed Talaat Rabea Ahmed Gadelkareem 《Current Urology》 CAS CSCD 2025年第4期274-279,共6页
Background:Systemic sclerosis(SSC)affects the urinary bladder and many other body organs.Systemic sclerosis commonly manifests as lower urinary tract symptoms(LUTS),which are usually reported using validated questionn... Background:Systemic sclerosis(SSC)affects the urinary bladder and many other body organs.Systemic sclerosis commonly manifests as lower urinary tract symptoms(LUTS),which are usually reported using validated questionnaires.This study aimed to correlate questionnaire-reported LUTS with urodynamic findings in patients with SSC.Materials and methods:This cross-sectional study was performed at our center between August 2018 and July 2021.Lower urinary tract symptoms were assessed using the International Consultation on Incontinence Questionnaire(ICIQ)Female Lower Urinary Tract Symptoms Modules and ICIQMale Lower Urinary Tract SymptomsModule and urodynamic studies.Univariate analyses were then performed on variables affecting detrusor activity.Results:This study included 22 patients(5 men,17 women).The mean±standard deviation age,body mass index,and disease duration were 37.2±11.1 years,25.5±4.2 kg/m2,and 6.2±5.3 years,respectively.Lower urinary tract symptoms occurred in 19(86.4%)patients,of whom18(94.7%)hadmild tomoderate symptoms.The ICIQscores for voiding and filling symptoms were higher than those for incontinence.The most frequent organ involvements included Raynaud’s phenomenon in 22(100%),skin conditions in 20(90.9%),gastrointestinal tractmaladies in 17(77.3%),joint disorders in 16(72.7%),and lung diseases in 14(63.6%)patients.Abnormal detrusor contractions occurred in only 5 patients(22.7%).Univariate analyses showed that disease duration<5 years(p=0.010),nonobstructive uroflowmetry findings(p=0.024),absence of incontinence(p=0.024)and telangiectasia(p=0.010),and negative rheumatoid factors(p=0.043)were significantly associated with normal detrusor contractions.Conclusions:Mild to moderate severity of ICIQ-measured LUTS affected most patients with SSC but was not correlated with urodynamic findings.Normal detrusor contractions were significantly associated with shorter disease duration,nonobstructive uroflowmetry findings,absence of incontinence and telangiectasia,and negative rheumatoid factors. 展开更多
关键词 Detrusor contractility Filling symptoms Lower urinary tract symptoms Systemic sclerosis Urodynamics
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