167 results match your criteria Thoracic Scleroderma Imaging

Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features: Strong Association with Lymphomas and Non-Myasthenic Autoimmune Diseases.

Cancers (Basel) 2021 Jan 16;13(2). Epub 2021 Jan 16.

Institute of Pathology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany.

Thymic hyperplasia (TH) with lymphoepithelial sialadenitis (LESA)-like features (LESA-like TH) has been described as a tumor-like, benign proliferation of thymic epithelial cells and lymphoid follicles. We aimed to determine the frequency of lymphoma and autoimmunity in LESA-like TH and performed retrospective analysis of cases with LESA-like TH and/or thymic MALT-lymphoma. Among 36 patients (21 males) with LESA-like TH (age 52 years, 32-80; lesion diameter 7. Read More

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January 2021

Software-based quantitative analysis of lung parenchyma in patients with systemic sclerosis may provide new generation data for pulmonary fibrosis.

Int J Clin Pract 2021 Apr 20;75(4):e13931. Epub 2020 Dec 20.

Department of Rheumatology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey.

Objectives: To investigate lung volume and density in patients with SSc and changes in these parameters because of PF, using a software-aided image quantification method, and compare this with a matched healthy control group.

Methods: Thoracic high-resolution computed tomography (HRCT) images of patients and controls were analysed using Myrian XP Lung 3D software. Right, and left lung densities and volumes were calculated separately by a blinded operator. Read More

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Relationship between calcifications and structural lesions on hand radiography and axial calcifications on CT-scan: A retrospective study in systemic sclerosis.

Medicine (Baltimore) 2020 Oct;99(43):e22443

Department of Rheumatology, University of Lorraine, CHRU Nancy.

The objectives of this study were to describe the prevalence and characteristics of radiographic lesions of the hands, and calcifications of the spine on computer tomography scans (CT-scans), and to investigate the relationships between radiographic and CT-scan abnormalities and clinical features in a population of patients with systemic sclerosis (SSc).Subjects underwent X-ray examination of the hands, and thoracic or thoraco-abdominal and pelvic CT scan or lumbar CT scan in the year. Structural lesions on hand X ray was scored and spinal calcifications were evaluated in the anterior, intracanal and posterior segments. Read More

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October 2020

Applicability of radiomics in interstitial lung disease associated with systemic sclerosis: proof of concept.

Eur Radiol 2021 Apr 6;31(4):1987-1998. Epub 2020 Oct 6.

Institute of Diagnostic and Interventional Radiology, University Hospital Zurich, University of Zurich, Rämistrase 100, 8091, Zurich, Switzerland.

Objective: To retrospectively evaluate if texture-based radiomics features are able to detect interstitial lung disease (ILD) and to distinguish between the different disease stages in patients with systemic sclerosis (SSc) in comparison with mere visual analysis of high-resolution computed tomography (HRCT).

Methods: Sixty patients (46 females, median age 56 years) with SSc who underwent HRCT of the thorax were retrospectively analyzed. Visual analysis was performed by two radiologists for the presence of ILD features. Read More

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Gastric Juice Expression of Th-17 and T-Reg Related Cytokines in Scleroderma Esophageal Involvement.

Cells 2020 09 16;9(9). Epub 2020 Sep 16.

Department of Medical Sciences, Allergy and Clinical Immunology Unit, University of Torino & Mauriziano Hospital, 10128 Turin, Italy.

Background: Systemic sclerosis (SSc) is a connective tissue disorder which key feature is a fibrotic process. The role of Endothelin-1 (ET-1) and T-helper (Th)-1 cells in lung and skin fibrosis is well known, although Th17- and Treg-cells were found to be involved. However, no studies analyzed cytokines expression in gastric-juice of SSc patients. Read More

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September 2020

Anterior chest wall non-traumatic diseases: a road map for the radiologist.

Acta Biomed 2020 07 13;91(8-S):43-50. Epub 2020 Jul 13.

Unit of Diagnostic Imaging, Department of Medical, Surgical and Neuro Sciences and of Radiological Sciences, University of Siena, Azienda Ospedaliero-Universitaria Senese, Siena, Italy.

The anterior chest wall (AWC) non-traumatic pathologies are largely underestimated, and early detection through imaging is becoming increasingly important. This paper aims to review the major non-traumatic ACW pathologies, with a particular interest in imaging features and differential diagnosis. Read More

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Diffuse Pulmonary Ossification on High-Resolution Computed Tomography in Idiopathic Pulmonary Fibrosis, Systemic Sclerosis-Related Interstitial Lung Disease, and Chronic Hypersensitivity Pneumonitis: A Comparative Study.

J Comput Assist Tomogr 2020 Sep/Oct;44(5):667-672

From the Departments of Radiology.

Objective: The aim of the study was to compare the prevalence of diffuse pulmonary ossification (DPO) in idiopathic pulmonary fibrosis (IPF), systemic sclerosis (SSc)-related interstitial lung disease (ILD), and chronic hypersensitivity pneumonitis (HP).

Methods: High-resolution computed tomography (HRCT) from 71 IPF, 67 SSc-ILD, and 75 HP cases were independently evaluated by 2 thoracic radiologists blinded to patient data. Studies were assessed for the presence of DPO, HRCT scanning pattern, stigmata of granulomatous infection, and honeycombing. Read More

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September 2020

Chest wall muscle atrophy as a contributory factor for forced vital capacity decline in systemic sclerosis-associated interstitial lung disease.

Rheumatology (Oxford) 2021 01;60(1):250-255

Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo.

Objective: To investigate the potential contribution of accessory respiratory muscle atrophy to the decline of forced vital capacity (FVC) in patients with SSc-associated interstitial lung disease (ILD).

Methods: This single-centre, retrospective study enrolled 36 patients with SSc-ILD who underwent serial pulmonary function tests and chest high-resolution CT (HRCT) simultaneously at an interval of 1-3 years. The total extent of ILD and chest wall muscle area at the level of the ninth thoracic vertebra on CT images were evaluated by two independent evaluators blinded to the patient information. Read More

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January 2021

Giant Calcinosis in Dermatomyositis and Scleroderma Overlap.

Arthritis Rheumatol 2020 07 27;72(7):1236. Epub 2020 May 27.

University of Western Ontario, London, Ontario, Canada.

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Chest CT imaging features for prediction of treatment response in cryptogenic and connective tissue disease-related organizing pneumonia.

Eur Radiol 2020 May 10;30(5):2722-2730. Epub 2020 Feb 10.

Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.

Objectives: To investigate CT imaging features associated with poor clinical outcome after corticosteroid treatment in patients diagnosed with cryptogenic organizing pneumonia (COP) and connective tissue disease-related organizing pneumonia (CTD-OP) and to assess the difference in CT findings and treatment responses between COP and CTD-OP.

Methods: Chest CT images from 166 patients (COP, 131; CTD-OP, 35) with pathologically proven organizing pneumonia were reviewed by two thoracic radiologists. The type, distribution pattern, and extent of parenchymal abnormalities, along with other associated imaging features, were assessed for each patient. Read More

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Systemic sclerosis myocarditis has unique clinical, histological and prognostic features: a comparative histological analysis.

Rheumatology (Oxford) 2020 Sep;59(9):2523-2533

Rheumathology Division, Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome.

Objective: To outline the clinical, histological and prognostic features of systemic sclerosis (SSc) endomyocardial biopsy-proven myocarditis with respect to those of diverse endomyocardial biopsy-proven virus-negative myocarditis (VNM).

Methods: We retrospectively analysed data from three cohorts of endomyocardial biopsy-proven myocarditis: SSc-related VNM (SSc-VNM); isolated VNM (i-VNM); and VNM related to other systemic autoimmune diseases (a-VNM). The degree of myocardial fibrosis was expressed as relative percentage and fibrotic score (0-3). Read More

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September 2020

Pulmonary veno-occlusive disease: a probably underdiagnosed cause of pulmonary hypertension in systemic sclerosis.

Clin Rheumatol 2020 May 22;39(5):1687-1691. Epub 2020 Jan 22.

Cardiology Department, Hospital Garcia de Orta EPE, Avenida Torrado da Silva, 2805-267, Almada, Portugal.

Pulmonary hypertension is a serious complication of systemic sclerosis and remains one of the leading causes of mortality. Pulmonary veno-occlusive disease (PVOD), recently reclassified as pulmonary arterial hypertension (PAH) with overt features of venous/capillaries involvement, is a subgroup of group 1 pulmonary hypertension, which has been rarely reported in systemic sclerosis patients. It is symptomatically indistinguishable from idiopathic pulmonary arterial hypertension and should be suspected in those with manifestations of pulmonary arterial hypertension who have evidence of pulmonary venous congestion in the absence of left-sided heart disease. Read More

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The Renal Resistive Index in systemic sclerosis: Determinants, prognostic implication and proposal for specific age-adjusted cut-offs.

Eur J Intern Med 2019 Dec 17;70:43-49. Epub 2019 Sep 17.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Via Delle Oblate 4, 50134 Florence, Italy; Department of Geriatric Medicine, Division of Rheumatology, Azienda Ospedaliera Universitaria Careggi, Florence, Italy.

Background: Renal Resistive Index (RRI), reflects changes in both renal vascular and tubular-interstitial compartments and in systemic vascular compliance related to age and comorbidities.

Objectives: a) To investigate determinants of RRI in SSc population, b) its association with SSc-related features and c) to test its prognostic impact on organ specific worsening or death.

Methods: 380 SSc patients ≥18 years were enrolled after giving informed consent. Read More

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December 2019

Pulmonary veno-occlusive disease is highly prevalent in scleroderma patients undergoing lung transplantation.

ERJ Open Res 2019 Feb 11;5(1). Epub 2019 Feb 11.

Division of Pulmonary Medicine, Dept of Medicine, National Jewish Health, Denver, CO, USA.

http://ow.ly/vPvc30neJZV. Read More

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February 2019

Diagnosis of pulmonary hypertension.

Eur Respir J 2019 01 24;53(1). Epub 2019 Jan 24.

Dept of Pulmonary Circulation and Cardidology, Medical Center for Postgraduate Education, ECZ-Otwock, Otwock, Poland.

A revised diagnostic algorithm provides guidelines for the diagnosis of patients with suspected pulmonary hypertension, both prior to and following referral to expert centres, and includes recommendations for expedited referral of high-risk or complicated patients and patients with confounding comorbidities. New recommendations for screening high-risk groups are given, and current diagnostic tools and emerging diagnostic technologies are reviewed. Read More

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January 2019

Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.

Semin Arthritis Rheum 2019 08 13;49(1):98-104. Epub 2018 Oct 13.

Department of Internal Medicine, Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, DHU Authors (Autoimmune and Hormonal Diseases), Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), 27, rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France. Electronic address:

Background: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc).

Methods: In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema.

Results: Rate of CPFE in SSc patients with CT scan was 3. Read More

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Performance of Forced Vital Capacity and Lung Diffusion Cutpoints for Associated Radiographic Interstitial Lung Disease in Systemic Sclerosis.

J Rheumatol 2018 11 1;45(11):1572-1576. Epub 2018 Oct 1.

From the Department of Medicine, Division of Rheumatology, Division of Pulmonary and Critical Care Medicine, Department of Preventive Medicine, Institute for Public Health and Medicine, Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.

Objective: Forced vital capacity (FVC) and DLCO are used for screening of systemic sclerosis-associated interstitial lung disease (SSc-ILD). The study purpose was to determine the sensitivity, specificity, and negative predictive value (NPV) (proportion of true negative screening tests) of FVC and DLCO thresholds for SSc-ILD on chest high-resolution computed tomography (HRCT) scans.

Methods: Patients fulfilling American College of Rheumatology 2013 SSc criteria with a chest HRCT scan and pulmonary function tests (PFT) were studied. Read More

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November 2018

Longitudinal Changes in Quantitative Interstitial Lung Disease on Computed Tomography after Immunosuppression in the Scleroderma Lung Study II.

Ann Am Thorac Soc 2018 11;15(11):1286-1295

5 Department of Medicine-Pulmonary and Critical Care, and.

Rationale: The Scleroderma Lung Study II (SLS II) demonstrated significant improvements in pulmonary function and dyspnea at 24 months compared with baseline when patients with symptomatic scleroderma-related interstitial lung disease (SSc-ILD) were treated with either cyclophosphamide for 1 year (followed for another year on placebo) or mycophenolate mofetil for 2 years in a randomized, double-blind clinical trial. Physiologic and clinical outcomes of SLS II have been published previously.

Objectives: The aim of the study was to assess changes from baseline in the extent of SSc-ILD on high-resolution computed tomography (HRCT) measured in the SLS II participants using quantitative image analysis after 2 years and to determine whether these HRCT changes were correlated with the changes in physiologic and clinical measures over the same time interval. Read More

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November 2018

Vertebral fracture prevalence and measurement of the scanographic bone attenuation coefficient on CT-scan in patients with systemic sclerosis.

Rheumatol Int 2018 Oct 21;38(10):1901-1910. Epub 2018 Aug 21.

Department of Rheumatology, University Hospital Nancy, 5 rue du Morvan, 54500, Vandoeuvre les Nancy, France.

To study vertebral fracture (VF) prevalence and the scanographic bone attenuation coefficient of the first lumbar vertebra (SBAC-L1) on computed tomography scans (CT-scans) in systemic sclerosis patients. This monocentric retrospective study included patients followed from 2000 to 2014 and fulfilling ACR/EULAR 2013 criteria for systemic sclerosis and who underwent a thoracic or thoraco-abdomino-pelvic CT-scan during their follow-up. Clinical characteristics for sclerosis and osteoporosis risk factors were collected. Read More

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October 2018

Spontaneous pneumomediastinum in limited cutaneous systemic sclerosis and myositis overlap.

BMJ Case Rep 2018 Jul 30;2018. Epub 2018 Jul 30.

Rheumatology, Johns Hopkins, Baltimore, Maryland, USA.

A 58-year-old man with limited cutaneous systemic sclerosis and myositis overlap complicated by interstitial lung disease presented with several months of progressive dyspnoea and weakness. CT chest revealed extensive pneumomediastinum that was not present on imaging 6 months before this study and appeared to be spontaneous, with no preceding trauma, infection or invasive procedures. Read More

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Lung Ultrasound as a Screening Method for Interstitial Lung Disease in Patients With Systemic Sclerosis.

J Clin Rheumatol 2019 Oct;25(7):304-307

Radiodiagnosis Service.

Background: Patients with systemic sclerosis (SSc) undergo chest radiographs and high-resolution computed tomography (HRCT) of the thorax both for interstitial lung disease (ILD) detection and for disease progression monitoring.

Objective: The aim of this study was to assess whether lung ultrasound (LUS) is a useful screening tool for ILD in patients with SSc in comparison with HRCT.

Methods: This was a longitudinal cohort study carried out from December 2015 to April 2016. Read More

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October 2019

Radiological pleuroparenchymal fibroelastosis associated to limited cutaneous systemic sclerosis: a case report.

BMC Pulm Med 2018 May 18;18(1):73. Epub 2018 May 18.

Internal Medicine Department, Hôpital Hôtel-Dieu, Centre Hospitalier Universitaire de Nantes, 1 place Alexis Ricordeau, 44093, Nantes, France.

Background: Pleuroparenchymal fibroelastosis (PPFE) is a very rare interstitial lung disease (ILD) characterized by progressive fibrotic lesions of the visceral pleura and the sub-pleural parenchyma, affecting predominantly the upper lobes. PPFE may occur in different contextes like bone marrow or lung transplantations, but also in the context of telomeropathy with mutations of telomerase reverse transcriptase (TERT), telomerase RNA component (TERC) or regulator of telomere elongation helicase 1 (RTEL1) genes. PPFE-like lesions have recently been described in patients with connective tissue disease (CTD)-related ILD. Read More

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Gauzoma in A Scleroderma Patient Following Open Heart Surgery: A Case Report.

Curr Rheumatol Rev 2019 ;15(1):79-81

Department of Internal Medicine, Division of Rheumatology, Shiraz University of Medical Sciences, Shiraz, Iran.

Introduction: Gauzoma is an iatrogenic complication which occurs rarely due to surgical team negligence. Depending on the sterility of the retained tissue, it can lead to life threatening surgical complications or may remain asymptomatic for many years and be detected incidentally in imaging studies. It may be mistaken as tumors or aneurysms. Read More

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Chest CT texture analysis for response assessment in systemic sclerosis.

Eur J Radiol 2018 Apr 31;101:50-58. Epub 2018 Jan 31.

Victor Babes University Timisoara Romania, Department V Internal Medicine, Eftimie Murge Str. 2, Romania.

Purpose: To evaluate the role of CT-textural features for monitoring lung involvement in subjects with systemic sclerosis(SSc) undergoing autologous stem cell transplantation(SCT) by comparison with semi-quantitative chest-CT, pulmonary function tests(PFT) and clinical parameters (modified Rodnan skin score[mRSS]).

Methods: In a retrospective single centre analysis, we identified 23 consecutive subjects(11male) with SSc between 07/2005 and 11/2016 that underwent chest CTs before, 6 and 12 months post-SCT. Response to therapy was defined at 6 months after transplantation as stabilisation or improvement in FVC > 10% and DLCO > 10%. Read More

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The Four Corners Sign: A Specific Imaging Feature in Differentiating Systemic Sclerosis-related Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis.

J Thorac Imaging 2018 May;33(3):197-203

Department of Radiology, Mayo Clinic.

Purpose: Differentiating between systemic sclerosis-related interstitial lung disease (SSc-ILD) and idiopathic pulmonary fibrosis (IPF) is important because of the differences in workup, prognosis, and treatment. However, there is much overlap in the appearance of these 2 entities on high-resolution computed tomography. We propose that inflammation and/or fibrosis focally or disproportionately involving the bilateral anterolateral upper lobes and posterosuperior lower lobes ["Four Corners" Sign (FCS)] is specific for SSc-ILD. Read More

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Systemic sclerosis: Annual chest CT is not necessary for all patients.

Presse Med 2018 Jan 20;47(1):90-92. Epub 2017 Dec 20.

Hôpital Saint-Antoine, service d'imagerie médicale, 184, rue du faubourg Saint-Antoine, 75012 Paris, France; Université Pierre et Marie Curie, 75005 Paris, France.

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January 2018

Prognostic significance of computed tomography criteria for pulmonary veno-occlusive disease in systemic sclerosis-pulmonary arterial hypertension.

Rheumatology (Oxford) 2017 12;56(12):2197-2203

National Pulmonary Hypertension Service, Royal Free London NHS Foundation Trust.

Objectives: SSc-pulmonary arterial hypertension (SSc-PAH) is associated with worse response to therapy and survival when compared with idiopathic PAH. It is suggested that the vasculopathy in SSc may involve postcapillary pulmonary venules resulting in pulmonary veno-occlusive disease (PVOD). This may underlie the lower gas transfer and worse outcome on therapy. Read More

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December 2017

Elevated Serum Anti-GM-CSF Antibodies before the Onset of Autoimmune Pulmonary Alveolar Proteinosis in a Patient with Sarcoidosis and Systemic Sclerosis.

Tohoku J Exp Med 2017 09;243(1):77-83

Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine.

Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-schiff stain-positive lipoproteinaceous materials in the alveolar space due to impaired surfactant clearance by alveolar macrophage. Autoimmune PAP is the most common form of PAP, but rarely accompanies collagen disease or sarcoidosis. We report here a rare case of autoimmune PAP preceded by systemic sclerosis and sarcoidosis. Read More

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September 2017

Rituximab Experience in Patients With Long-standing Systemic Sclerosis-Associated Interstitial Lung Disease: A Series of 14 Patients.

J Clin Rheumatol 2017 Dec;23(8):411-415

Objectives: The objective of this study was to report the experience with rituximab treatment in a case series of patients with long-standing systemic sclerosis-associated interstitial lung disease (SSc-ILD).

Methods: We reviewed the charts of 197 SSc patients. Fourteen patients who received rituximab for SSc-ILD participated in this analysis. Read More

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December 2017

A potential contribution of psoriasin to vascular and epithelial abnormalities and inflammation in systemic sclerosis.

J Eur Acad Dermatol Venereol 2018 Feb 3;32(2):291-297. Epub 2017 Aug 3.

Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo, Japan.

Background: Antimicrobial peptides have attracted much attention as a member of disease-associated molecules in systemic sclerosis (SSc), which is pathologically characterized by immune abnormalities, vasculopathy and tissue fibrosis.

Objective: To investigate the potential contribution of one of the antimicrobial peptide psoriasin to the development of SSc.

Methods: Psoriasin expression in the skin samples and sera derived from SSc patients and its correlation with clinical parameters were analysed. Read More

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February 2018