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computed Tomography systemic sclerosis

A Boucly, B Girerd, D Bourlier, S Nemlaghi, J Caliez, L Savale, X Jaïs, P Dorfmüller, G Simonneau, O Sitbon, M Humbert, D Montani
Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterized by preferential remodelling of pulmonary venules and angioproliferation. PVOD term includes idiopathic, heritable (biallelic mutations of EIF2AK4 gene), drugs and toxins induced (alkylating agents, organic solvents) and connectivite-associated forms (especially systemic-sclerosis associated form). PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation. Lung biopsy is contraindicated in PVOD due to high risk of life-threatening bleeding...
February 28, 2018: Revue des Maladies Respiratoires
Audrey Benyamine, Xavier Heim, Noémie Resseguier, Daniel Bertin, Carine Gomez, Mikaël Ebbo, Jean-Robert Harlé, Gilles Kaplanski, Pascal Rossi, Nathalie Bardin, Brigitte Granel
OBJECTIVES: We aimed to assess the clinical significance of Krebs von den Lungen-6 (KL-6) in the diagnosis and severity of interstitial lung disease (ILD) in a French cohort of patients with systemic sclerosis (SSc). METHODS: Serum KL-6 concentrations were measured with chemiluminescent enzyme immunoassay (CLEIA) in 75 SSc patients. Patients were divided into two groups according to the presence of interstitial lung disease (SSc-ILD versus SSc-without ILD) on chest High-Resolution Computed Tomography...
February 17, 2018: Rheumatology International
Elana J Bernstein, Dinesh Khanna, David J Lederer
Interstitial lung disease (ILD) affects approximately 40-60% of adults with systemic sclerosis (SSc) and is the leading cause of death and hospitalization in this population (1-4). Despite the life-threatening nature of ILD and the availability of existing therapies (5, 6), there are no clinical practice guidelines that recommend screening for ILD in SSc. Although pulmonary function tests (PFTs) are widely used by rheumatologists as a screening test for SSc-ILD, studies have shown that they are neither sensitive nor specific for the detection of ILD in this population (3)...
February 9, 2018: Arthritis & Rheumatology
Nadia D Morgan, Ami A Shah, Maureen D Mayes, Robyn T Domsic, Thomas A Medsger, Virginia D Steen, John Varga, Mary Carns, Paula S Ramos, Richard M Silver, Elena Schiopu, Dinesh Khanna, Vivien Hsu, Jessica K Gordon, Heather Gladue, Lesley A Saketkoo, Lindsey A Criswell, Chris T Derk, Marcin A Trojanowski, Victoria K Shanmugam, Lorinda Chung, Antonia Valenzuela, Reem Jan, Avram Goldberg, Elaine F Remmers, Daniel L Kastner, Fredrick M Wigley, Pravitt Gourh, Francesco Boin
Racial differences exist in the severity of systemic sclerosis (SSc). To enhance our knowledge about SSc in African Americans, we established a comprehensive clinical database from the largest multicenter cohort of African American SSc patients assembled to date (the Genome Research in African American Scleroderma Patients (GRASP) cohort).African American SSc patients were enrolled retrospectively and prospectively over a 30-year period (1987-2016), from 18 academic centers throughout the United States. The cross-sectional prevalence of sociodemographic, clinical, and serological features was evaluated...
December 2017: Medicine (Baltimore)
Hideaki Yamakawa, Tamiko Takemura, Tae Iwasawa, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Hideya Kitamura, Tomohisa Baba, Shinichiro Iso, Koji Okudela, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Pulmonary emphysema combined with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) occurs more often in smokers but also in never-smokers. This study aimed to describe a new finding characterized by peculiar emphysematous change with SSc-associated ILD (SSc-ILD). METHODS: We conducted a retrospective review of 21 consecutive patients with SSc-ILD diagnosed by surgical lung biopsy and focused on the radio-pathological correlation of the emphysematous change...
January 30, 2018: BMC Pulmonary Medicine
Lara Walkoff, Darin B White, Jonathan H Chung, Dennis Asante, Christian W Cox
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...
January 16, 2018: Journal of Thoracic Imaging
Carolina Alves, Sónia Batista, Otília C d'Almeida, Lívia Sousa, Luís Cunha, Rui Bernardes, Miguel Castelo-Branco
We investigated the relationship between retinal layers and normal-appearing white matter (WM) integrity in the brain of patients with relapsing-remitting multiple sclerosis (MS), using a combined diffusion tensor imaging and high resolution optical coherence tomography approach. Fifty patients and 62 controls were recruited. The patients were divided into two groups according to presence (n = 18) or absence (n = 32) of optic neuritis. Diffusion tensor data were analyzed with a voxel-wise whole brain analysis of diffusion metrics in WM with tract-based spatial statistics...
January 15, 2018: Human Brain Mapping
D Sánchez-Cano, N Ortego-Centeno, J L Callejas, V Fonollosa Plá, R Ríos-Fernández, C Tolosa-Vilella, G Espinosa-Garriga, D Colunga-Argüelles, M V Egurbide-Arberas, M Rubio-Rivas, M Freire, J J Ríos-Blanco, L Trapiella-Martínez, M Rodríguez-Carballeira, A Marín-Ballvé, X Pla-Salas, C P Simeón-Aznar
OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group...
March 2018: Rheumatology International
Hayley Barnes, Anne E Holland, Glen P Westall, Nicole Sl Goh, Ian N Glaspole
BACKGROUND: Approximately one-third of individuals with interstitial lung disease (ILD) have associated connective tissue disease (CTD). The connective tissue disorders most commonly associated with ILD include scleroderma/systemic sclerosis (SSc), rheumatoid arthritis, polymyositis/dermatomyositis, and Sjögren's syndrome. Although many people with CTD-ILD do not develop progressive lung disease, a significant proportion do progress, leading to reduced physical function, decreased quality of life, and death...
January 3, 2018: Cochrane Database of Systematic Reviews
Jiuliang Zhao, Hongnan Mo, Xiaoxiao Guo, Qian Wang, Dong Xu, Yong Hou, Zhuang Tian, Yongtai Liu, Hui Wang, Jinzhi Lai, Mengtao Li, Xiaofeng Zeng
The aim of this study was to investigate the utility of red blood cell distribution width (RDW) as a simple and readily available marker of occurrence of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc). One hundred and forty-five consecutive patients with SSc were recruited to the single-center cross-sectional study. Demographic characteristics, hematological parameters, Modified Rodnan Skin Score, and World Health Organization functional classification were determined. Diagnosis of PAH was based on screening by echocardiography and was confirmed by right heart catheterization...
November 21, 2017: Clinical Rheumatology
Songül Çildağ, Mehmet Burak Çildağ
Background/aim: This study aimed to assess the relationship between skin fibrosis as determined by sonoelastography and the degree of pulmonary involvement as determined by high-resolution computed tomography (HRCT) in patients with diffuse cutaneous systemic sclerosis (dcSSc).Materials and methods: This prospective study included 40 patients with dcSSc. All patients with HRCT scans underwent conventional ultrasonography and sonoelastography to determine skin thickness and degree of fibrosis. The degree of fibrosis was classified according to color-scale sonoelastography...
November 13, 2017: Turkish Journal of Medical Sciences
F Ahlhelm, K Shariat, S Götschi, S Ulmer
CLINICAL PROBLEM: Intracerebral cysts are common findings in imaging of the neurocranium and are not always clinically significant. The pathological spectrum of intracerebral cysts is, however, very broad and in addition to incidental findings includes developmental disorders, malformation tumors, primary and secondary neoplasms and infectious etiologies, such as cerebral abscess formation, cysticercosis or residuals after congenital cytomegalovirus infections. Intracerebral cystic defects may be caused by inflammatory central nervous system (CNS) diseases, such as multiple sclerosis as well as by mitochondriopathies, leukodystrophy, electrolyte disturbances or osmotic demyelination syndrome or brain infarctions, e...
February 2018: Der Radiologe
Viktor Martyanov, Grace-Hyun J Kim, Wendy Hayes, Shuyan Du, Bishu J Ganguly, Oumar Sy, Sun Ku Lee, Galina S Bogatkevich, Gary L Schieven, Elena Schiopu, Roberta Gonçalves Marangoni, Jonathan Goldin, Michael L Whitfield, John Varga
BACKGROUND: There are no effective treatments or validated clinical response markers in systemic sclerosis (SSc). We assessed imaging biomarkers and performed gene expression profiling in a single-arm open-label clinical trial of tyrosine kinase inhibitor dasatinib in patients with SSc-associated interstitial lung disease (SSc-ILD). METHODS: Primary objectives were safety and pharmacokinetics. Secondary outcomes included clinical assessments, quantitative high-resolution computed tomography (HRCT) of the chest, serum biomarker assays and skin biopsy-based gene expression subset assignments...
2017: PloS One
Abdel Gaffar A Mohammed, Ammar Alshihre, Ibrahim Abdulrazag Al-Homood
There is increasing interest in rituximab (RTX) as an alternative to cyclophosphamide for the treatment of interstitial lung diseases (ILDs) associated with systemic sclerosis (SSc). However, no report has addressed its efficacy in Saudi patients with SSc-ILD. To assess the efficacy of RTX treatment in Saudi patients with SSc-ILD, hospital records were reviewed between 2013 and 2016. Four female patients received at least 4 cycles of RTX (I cycle, consisting of two infusions of 1000 mg 2 weeks apart). Pulmonary function tests (PFTs) and chest high-resolution computed tomography (HRCT) were performed before and after treatment to assess the response...
October 2017: Annals of Thoracic Medicine
Mario Meloni, Rita Farris, Paolo Solla, Marcello M Mascia, Francesco Marrosu, Antonino Cannas
INTRODUCTION: Large expansions of the noncoding GGGGCC repeat (more than 30) in the first intron of the C9ORF72 gene have been demonstrated to cause amyotrophic lateral sclerosis and frontotemporal dementia. Recent papers have investigated the possible pathogenic role and associated clinical phenotypes of hexanucleotide expansions with intermediate repeat lengths ranging between 20 and 29 repeats. CASE REPORT: We report a case of a 71-year-old Sardinian female patient with a long history of psychiatric disorders such as mixed anxiety-depressive disorder associated with somatization disorder and histrionic personality who developed a slowly progressive cerebellar syndrome, mild cognitive impairment, pyramidal signs, and rapid eye movement sleep behavior disorder with imaging abnormalities on the DaTSCAN single-photon emission computed tomography indicating an alteration in the presynaptic dopaminergic system...
November 2017: Neurologist
Nihal Martis, Viviane Queyrel-Moranne, David Launay, Rémi Neviere, Jean-Gabriel Fuzibet, Charles-Hugo Marquette, Sylvie Leroy
OBJECTIVE: Exercise limitation in patients with systemic sclerosis (SSc) is often multifactorial and related to complications such as interstitial lung disease (ILD), pulmonary vasculopathy (PV), left ventricular dysfunction (LVD), and/or peripheral/muscular limitation (PML). We hypothesized that cardiopulmonary exercise testing (CPET) could not only suggest and rank competing etiologies, but also highlight peripheral impairment. METHODS: Clinical, resting pulmonary function testing, and CPET data from patients with SSc referred for exercise limitation between October 2009 and November 2015 were retrospectively analyzed in this bi-center study...
November 1, 2017: Journal of Rheumatology
J F Yu, Y B Jin, J He, Y An, Z G Li
Interstitial lung diseases (ILDs) are a diverse group of pulmonary disorders characterized by various patterns of inflammation and fibrosis in the interstitium of the lung. The underlying pathogenesis of ILDs is complex and associated with multiple rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, pollymyositis and dermatomyositis, Sjögren's syndrome, and systemic lupus erythematosus. As the disease progresses, excessive pulmonary fibrosis impairs alveolar gas exchange and damages pulmonary function...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Carolina de Souza Müller, Danny Warszawiak, Eduardo Dos Santos Paiva, Dante Luiz Escuissato
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are prevalent complications of systemic sclerosis (SSc) and are currently the leading causes of death related to the disease. The accurate recognition of these conditions is therefore of utmost importance for patient management. A study was carried out with 24 SSc patients being followed at the Rheumatology Department of the Hospital de Clínicas of Universidade Federal do Paraná (UFPR) and 14 healthy volunteers, with the objective of evaluating the usefulness of lung magnetic resonance imaging (MRI) when assessing ILD in SS patients...
September 2017: Revista Brasileira de Reumatologia
Geetakiran Arakkal, Sudha Rani Chintagunta, Vasavilatha Chandika, Sudha Vani Damarla, Srinivas Manchala, B Udaya Kumar
BACKGROUND: Systemic sclerosis is a multisystem disorder characterized by microangiopathy, dysregulation of the immune system and massive deposition of collagen in the connective tissue of the skin, blood vessels and various internal organs. Although the mortality from renal crises has dropped significantly due to the use of angiotensin-converting enzyme inhibitors, cardiac and pulmonary involvement accounts for significant morbidity and mortality. We studied 28 patients with systemic sclerosis at Gandhi Medical College and Hospital, Hyderabad, over a period of two years for cardiopulmonary involvement...
November 2017: Indian Journal of Dermatology, Venereology and Leprology
Michelle J Connolly, Sharif Abdullah, Deborah A Ridout, Benjamin E Schreiber, Jamanda A Haddock, J Gerry Coghlan
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. We sought to test whether CT signs of PVOD (CTS-PVOD) were frequent in SSc-PAH and whether they were associated with pulmonary oedema on therapy and worse survival...
December 1, 2017: Rheumatology
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