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Editorial introductions
doi : 10.1097/BOR.0000000000000843
November 2021 - Volume 33 - Issue 6 - p vii-ix
Raynaud's phenomenon and digital ulcers: advances in evaluation and management
Herrick, Ariane L.
doi : 10.1097/BOR.0000000000000826
November 2021 - Volume 33 - Issue 6 - p 453-462
The aim of this review is to give an update on advances in evaluation and management of systemic sclerosis (SSc)-related Raynaud's phenomenon and digital ulceration, focusing on reports from the last 18 months. The increasing recognition of the huge impact of Raynaud's phenomenon and of digital ulceration on the everyday lives of patients with SSc has sparked enthusiasm internationally to develop better outcome measures and better treatments, and so a review is timely.
Contribution of monocytes and macrophages to the pathogenesis of systemic sclerosis: recent insights and therapeutic implications
Lescoat, Alaina,b,c,d; Lecureur, Valérieb; Varga, Johnc,d
doi : 10.1097/BOR.0000000000000835
November 2021 - Volume 33 - Issue 6 - p 463-470
To discuss recent studies addressing the role of monocytes and macrophages in the pathogenesis of systemic sclerosis (SSc) based on human and mouse models.
New mechanism-based approaches to treating and evaluating the vasculopathy of scleroderma
Flavahan, Nicholas A.
doi : 10.1097/BOR.0000000000000830
November 2021 - Volume 33 - Issue 6 - p 471-479
Utilizing recent insight into the vasculopathy of scleroderma (SSc), the review will highlight new opportunities for evaluating and treating the disease by promoting stabilization and protection of the microvasculature.
Biomarkers in systemic sclerosis: mechanistic insights into pathogenesis and treatment
Arron, Joseph R.
doi : 10.1097/BOR.0000000000000827
November 2021 - Volume 33 - Issue 6 - p 480-485
Systemic sclerosis (SSc) is heterogenous on molecular, cellular, tissue, and clinical levels. Although many biomarkers have been described in clinical studies, few have been rigorously mapped to specific molecular pathways, tissue pathologies, and clinical manifestations. A focused assessment of peripheral blood levels of C–C Motif Chemokine Ligand-18 (CCL18) and periostin illustrates how biomarkers can link molecular mediators to clinical outcomes.
Insights into origins and specificities of autoantibodies in systemic sclerosis
Tiniakou, Eleni; Crawford, Jonathan; Darrah, Erika
doi : 10.1097/BOR.0000000000000834
November 2021 - Volume 33 - Issue 6 - p 486-494
Autoantibodies are hallmark findings in systemic sclerosis (SSc), often present prior to disease onset. Clinical diagnosis and prognosis of SSc have long relied on the antitopoisomerase – anticentromere – anti-RNA polymerase antibody trichotomy. However, many more autoantibodies found in SSc are being actively investigated for insights into triggering events, mechanisms of tolerance break, and connections to tissue damage. This review examines recent studies on SSc autoantibodies and the early events that lead to their development.
The dynamic organelle primary cilia: emerging roles in organ fibrosis
Bhattacharyya, Dibyendua,?; Teves, Maria E.b; Varga, Johna,?
doi : 10.1097/BOR.0000000000000841
November 2021 - Volume 33 - Issue 6 - p 495-504
Primary cilia, the antenna-like organelles on most mammalian cells, host key components of multiple morphogen signal transduction pathways. Mutations in genes responsible for primary cilia assembly and function generally result in pathological conditions known as ciliopathies, which underlie several diseases, including various forms of fibrosis. Primary cilia modulate cellular responses to extracellular cues, including TGF-? and morphogens, such as Hedgehog. Aberrant morphogen signaling is recognized as essential for the transition of mesenchymal progenitor cells to myofibroblasts, the key step in fibrosis. This article aims to provide a critical overview of recent developments and insights in primary cilia biology relevant to fibrosis.
Adipose tissue and adipose secretome in systemic sclerosis
Brezovec, Nežaa,b; Burja, Blaža,c; Lakota, Katjaa,d
doi : 10.1097/BOR.0000000000000838
November 2021 - Volume 33 - Issue 6 - p 505-513
Adipose tissue is closely associated with systemic sclerosis (SSc)-pathology, both anatomically and functionally. This review focuses on local effects of adipocytes in the context of adipose to mesenchymal transdifferentiation (AMT), effects of the adipose stromal vascular fraction on SSc pathogenesis and systemic effects of adipose tissue secretome.
Environmental triggers for connective tissue disease: the case of COVID-19 associated with dermatomyositis-specific autoantibodies
De Santis, Mariaa,b; Isailovic, Natasaa; Motta, Francescaa,b; Ricordi, Caterinab; Ceribelli, Angelaa,b; Lanza, Ezioc; Azzolini, Elenab,d; Badalamenti, Salvatoree; Voza, Antoniob,f; Selmi, Carloa,b
doi : 10.1097/BOR.0000000000000844
November 2021 - Volume 33 - Issue 6 - p 514-521
The aim of the present review is to analyze the link between autoimmune diseases and environmental factors, in particular severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection (COVID-19) as it shares numerous features with the interstitial lung disease associated with connective tissue diseases positive for rare autoantibodies directed at highly specific autoantigens (i.e., MDA5 and RIG1) among the intracellular sensors of SARS-CoV-2 in the innate response against viruses.
Lymphocyte immunophenotyping in inflammatory myositis: a review
Franco, Chiara; Gatto, Mariele; Iaccarino, Luca; Ghirardello, Anna; Doria, Andrea
doi : 10.1097/BOR.0000000000000831
November 2021 - Volume 33 - Issue 6 - p 522-528
This is a comprehensive review of the current knowledge on predominant immune cell phenotypes involved in idiopathic inflammatory myopathies (IIM).
Skeletal muscle immunohistochemistry of acquired and hereditary myopathies
Danielsson, Olof; Häggqvist, Bo
doi : 10.1097/BOR.0000000000000828
November 2021 - Volume 33 - Issue 6 - p 529-536
The continued development in the field of immunohistochemistry (IHC) has improved the ability to diagnose muscle diseases. Many hereditary diseases are diagnosed by the absence or abnormal localization of proteins. Detection of secondary pathological protein expression is also used in diagnostics, and to study disease processes. We relate and discuss recent reports, where IHC has been an important tool in the investigation of muscle diseases.
Polymyositis: does it really exist as a distinct clinical subset?
Leclair, Valériea,b,c; Notarnicola, Antonellad; Vencovsky, Jirie; Lundberg, Ingrid E.d
doi : 10.1097/BOR.0000000000000837
November 2021 - Volume 33 - Issue 6 - p 537-543
To summarize information on polymyositis; diagnosis, definitions, published data and opinions.
Differential diagnosis of necrotizing myopathy
Selva-O’Callaghan, Alberta; Trallero-Araguás, Ernestob; Milisenda, Jose C.c; Grau-Junyent, Josep M.c
doi : 10.1097/BOR.0000000000000836
November 2021 - Volume 33 - Issue 6 - p 544-553
Necrotizing myopathy is a broad term. It includes patients with the recently described immune-mediated necrotizing myopathies (IMNM) who have specific antibodies, such as anti-hydroxy-3-methylglutaryl-CoA reductase or anti-signal recognition particle, seronegative phenotypes that can be associated with cancer, and other types of myositis and connective tissue diseases involving necrotic muscle fibers as a characteristic pathologic feature. Necrotizing myopathies that are not immune-mediated, such as those caused by drugs, dystrophies, infections, or even hypothyroidism are also included. The purpose of this review is to address the differential diagnosis of these disorders.
Anti-HMGCR myopathy: clinical and histopathological features, and prognosis
Kurashige, Takashi
doi : 10.1097/BOR.0000000000000832
November 2021 - Volume 33 - Issue 6 - p 554-562
This review aims to describe clinical and pathological features, prognosis and treatment in patients with anti-HMGCR antibody positive immune-mediated necrotizing myopathy (HMGCR-IMNM) based on recent findings.
Physical exercise for the management of systemic autoimmune myopathies: recent findings, and future perspectives
dos Santos, Alexandre Mouraa; Misse, Rafael Giovania; Borges, Isabela Bruna Piresa; Perandini, Luiz Augusto Buorob; Shinjo, Samuel Katsuyukia
doi : 10.1097/BOR.0000000000000829
November 2021 - Volume 33 - Issue 6 - p 563-569
The aim of this review is to present the main pieces of evidence, recent literature and to present future perspectives on the use of exercise/physical training in the treatment and improvement of the quality of life of patients with systemic autoimmune myopathies.
Optimizing reproductive health management in lupus and Sjogren's syndrome
Birru Talabi, Mehreta,b; Himes, Katherine P.c; Clowse, Megan E.B.d
doi : 10.1097/BOR.0000000000000839
November 2021 - Volume 33 - Issue 6 - p 570-578
People with childbearing capacity who are diagnosed with systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS) have specific and important reproductive health considerations.
Transcriptomics data: pointing the way to subclassification and personalized medicine in systemic lupus erythematosus
Hubbard, Erika L.; Grammer, Amrie C.; Lipsky, Peter E.
doi : 10.1097/BOR.0000000000000833
November 2021 - Volume 33 - Issue 6 - p 579-585
To summarize recent studies stratifying SLE patients into subgroups based on gene expression profiling and suggest future improvements for employing transcriptomic data to foster precision medicine.
The role of CD8+ T-cell systemic lupus erythematosus pathogenesis: an update
Chen, Ping-Min; Tsokos, George C.
doi : 10.1097/BOR.0000000000000815
November 2021 - Volume 33 - Issue 6 - p 586-591
Systemic lupus erythematosus (SLE) is a serious autoimmune disease with a wide range of organ involvement. In addition to aberrant B-cell responses leading to autoantibody production, T-cell abnormalities are important in the induction of autoimmunity and the ensuing downstream organ damage. In this article, we present an update on how subsets of CD8+ T cells contribute to SLE pathogenesis.
Immune checkpoints and the multiple faces of B cells in systemic lupus erythematosus
Stefanski, Ana-Luisaa,b; Dörner, Thomasa,b
doi : 10.1097/BOR.0000000000000825
November 2021 - Volume 33 - Issue 6 - p 592-597
B-lymphocytes are crucial in the pathogenesis of systemic lupus erythematosus (SLE), including autoantibody production, antigen presentation, co-stimulation, and cytokine secretion. Co-stimulatory and co-inhibitory molecules control interactions between B and T cells during an inflammatory response, which is essential for an appropriate host protection and maintenance of self-tolerance. Here, we review recent findings about checkpoint molecules and SLE B cells including their potential therapeutic implications and experiences from clinical trials.
Integrating genetic and social factors to understand health disparities in lupus
Ramos, Paula S.a,b
doi : 10.1097/BOR.0000000000000840
November 2021 - Volume 33 - Issue 6 - p 598-604
Both social and genetic factors are associated with health outcomes in systemic lupus erythematosus (SLE), thus playing a role in its health disparities. Despite the growing list of social and genetic factors associated with SLE outcomes, studies integrating sociocultural and individual determinants of health to understand health disparities in SLE are lacking. We review the contributions of different social and genetic factors to the disparities in SLE, and propose a socioecological model to integrate and examine the complex interactions between individual and social factors in SLE outcomes.
