Abstract：
:Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of the liver and that is characterized by progressive inflammation, fibrosis, and stricturing of the intrahepatic and extrahepatic bile ducts. It is progressive in most patients and leads to cirrhosis. It is a rare disease, mostly affecting people of northern European descent, males greater than females. The diagnosis is best established by contrast cholangiography, which reveals a characteristic picture of diffuse, multifocal strictures and focal dilation of the bile ducts, leading to a beaded appearance. Inflammatory bowel disease (IBD) is present in ~75% of the patients with PSC, mostly ulcerative colitis (~85% of the cases). In addition to biliary cirrhosis, complications of PSC include dominant strictures of the bile ducts, cholangitis, cholangiocarcinoma, colon dysplasia and cancer in patients with IBD, gallbladder polyps and cancer, and hepatic osteodystrophy. The etiology of PSC is not clear, but studies are ongoing. The median survival without liver transplantation is 12 to 15 years after diagnosis. Currently there are no effective treatments except liver transplantation. Immunosuppressive medications have not been shown to be effective but antibiotics and anti-fibrotic agents seem promising.
journal_name
Autoimmun Revjournal_title
Autoimmunity reviewsauthors
Yimam KK,Bowlus CLdoi
10.1016/j.autrev.2014.01.040subject
Has Abstractpub_date
2014-04-01 00:00:00pages
445-50issue
4-5eissn
1568-9972issn
1873-0183pii
S1568-9972(14)00052-4journal_volume
13pub_type
杂志文章,评审abstract：:Autoimmunity causes pathological conditions resulting in autoimmune diseases (ADs). Although autoimmunity is a mystery, immunological dogma suggests that autoreactive cell reactivation (ACR) breaks self-tolerance and induces autoimmunity. Thus, ACR is a royal pathway for ADs. Cumulative evidence implicates environment...
journal_title：Autoimmunity reviews
pub_type： 杂志文章
doi：10.1016/j.autrev.2014.08.024
更新日期：2014-10-01 00:00:00
abstract：:Alpha-actinin (α-actinin) is a ubiquitous cytoskeletal protein, which belongs to the superfamily of filamentous actin (F-actin) crosslinking proteins. It is present in multiple subcellular regions of both muscle and non-muscle cells, including cell-cell and cell-matrix contact sites, cellular protrusions and stress fi...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.12.009
更新日期：2011-05-01 00:00:00
abstract：:Celiac disease, or gluten sensitive enteropathy is a relatively common disease of the jejunum, leading to malabsorption. It is an immune mediated disease, induced by gluten on the grounds of a specific genetic makeup. After gluten exposition immune processes are induced mainly by T-cells, causing typical intestinal an...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.12.001
更新日期：2011-04-01 00:00:00
abstract：BACKGROUND:Anticardiolipin antibodies of the immunoglobulin G isotype (IgG aCL) have been suggested as risk factor for arterial and venous thrombosis. No conclusive data in patients with coronary artery disease (CAD) do exist. We investigate the risk of recurrent CAD according to the presence of IgG aCL. METHODS:We pe...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,meta分析
doi：10.1016/j.autrev.2019.03.005
更新日期：2019-05-01 00:00:00
abstract：:Fas is a transmembrane receptor involved in the death program of several cell lines, including T lymphocytes. Deleterious mutations hitting genes involved in the Fas pathway cause the autoimmune lymphoprolipherative syndrome (ALPS). Moreover, defective Fas function is involved in the development of common autoimmune d...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2011.08.011
更新日期：2012-12-01 00:00:00
abstract：:Follicular helper T cells (T(FH)) have been implicated as a lineage that provides sufficient help to B cells in order to become professional antibody producers. This T helper subset is characterized by a distinctive cell-surface phenotype (CD4(+)CD57(+)CXCR5(+)) and cytokine profile (IL-21, IL-6, and IL-27) as well as...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.11.007
更新日期：2011-04-01 00:00:00
abstract：:Despite the improvement in the quality of life of patients with SLE due to scientific and technological advances, SLE remains a disease that over the years may produce irreversible damage to patients. Osteoporosis and secondary bone fractures are two of the major causes of irreparable injury in patients with SLE. Vita...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2017.09.011
更新日期：2017-11-01 00:00:00
abstract：:Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of rare diseases characterized by necrotizing inflammation of small blood vessels and the presence of ANCA. Although these autoantibodies were initially used to classify pauci-immune vasculitis, increasing clinical and experimen...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2016.03.007
更新日期：2016-07-01 00:00:00
abstract：:Giant cell arteritis (GCA) is a primary large-vessel vasculitis predominantly seen in the elderly that preferentially involves the external carotid artery and its branches. However, inflammation of the aorta and its branches occurs in a subset of patients although symptoms of aortic involvement may appear years after ...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.02.014
更新日期：2010-05-01 00:00:00
abstract：:Approximately 500 treatment recalcitrant pemphigus vulgaris patients have been treated with rituximab. They were treated according to the lymphoma protocol (N=224) or rheumatoid arthritis protocol (RAP) (N=209) patients. Others were treated with modifications or combinations of the two. The mean duration of follow-up ...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2014.12.002
更新日期：2015-04-01 00:00:00
abstract：:Mesenchymal stem cells (MSCs) are non-hematopoietic, multipotent progenitor cells which can be isolated from various human adult tissues. In recent years, MSCs have been shown to possess broad immunoregulatory capabilities, modulating both adaptive and innate immunity. This review discusses the documented immunomodula...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2011.01.005
更新日期：2011-05-01 00:00:00
abstract：:Beta2-glycoprotein I (beta2-GPI) is a major antigen for antiphospholipid antibodies (aPL) present in patients with antiphospholipid syndrome (APS). Oxidized low-density lipoprotein (oxLDL) is subsequently targeted by beta2-GPI and anti-beta2-GPI autoantibodies. Ligands specific for beta2-GPI derived from oxLDL have be...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/s1568-9972(02)00084-8
更新日期：2002-12-01 00:00:00
abstract：:Antineutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of some idiopathic systemic vasculitides, such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and, to a lesser extent, Churg-Strauss syndrome (CCS), the so-called ANCA-associated vasculitides (AAV). ANCA were first det...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2012.08.008
更新日期：2013-02-01 00:00:00
abstract：OBJECTIVE:Emerging data have shown an increased risk of malignancy among patients diagnosed with systemic sclerosis (SSc) so identification of risk factors linking both disorders might have prognostic implications. The aim of this study was to assess the clinical and treatment-related risk factors for cancer in a singl...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2017.03.012
更新日期：2017-05-01 00:00:00
abstract：:Mixed cryoglobulinemia (MC) is an immunological disorder characterized by immune-complex-mediated systemic vasculitis involving small vessels, which may present with renal, cutaneous, rheumatologic, and/or neurological manifestations. Until recently, the possible appearance of anti-neuronal autoantibodies in periphera...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2008.07.027
更新日期：2008-10-01 00:00:00
abstract：:The Churg-Strauss Syndrome is an ANCA-associated vasculitis, an inflammatory multisystem disease with preference to the respiratory tract. Peripheral and tissue eosinophilia are the pathological hallmarks of this condition. The etiopathogenesis is unknown but some cytokines appear to play a central role and could be t...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2012.07.009
更新日期：2012-12-01 00:00:00
abstract：:Autoimmune responses and inflammation are involved in the excess cardiovascular risk observed in patients with systemic inflammatory diseases. Autoimmune myocarditis is a presentation of an inflammatory reaction of the heart during the course of autoimmune disorders, with most cases seen in systemic lupus erythematosu...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2012.05.005
更新日期：2012-12-01 00:00:00
abstract：OBJECTIVE:To address changes in survival of patients with systemic lupus erythematosus (SLE) and to compare mortality statistics with associated disease specific as well as comorbid conditions. METHODS:Review of the international literature on survival of patients with SLE. RESULTS:During the first half of the 20th c...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2004.04.002
更新日期：2004-08-01 00:00:00
abstract：:Type 1 diabetes (T1D) is a chronic autoimmune disease characterized by the loss of insulin-producing pancreatic β-cells. The pathogenesis of T1D is complex and multifactorial and involves a genetic susceptibility that predisposes to abnormal immune responses in the presence of ill-defined environmental insults to the ...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2015.11.008
更新日期：2016-03-01 00:00:00
abstract：:Clinical treatment of autoimmune disorders presents a special challenge. For decades, most clinical regimens in autoimmunity has been largely symptomatic and non-disease specific. Although data from vigorous research has lead to accumulating knowledge on the pathogenic and immunological mechanisms of many autoimmune d...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2009.10.004
更新日期：2010-01-01 00:00:00
abstract：:In Multiple Sclerosis (MS) patients, conventional magnetic resonance imaging (MRI) shows a pattern of white matter (WM) disruption but may also overlook some WM damage. Diffusion tensor MRI (DT-MRI) can provide important in-vivo information about fiber direction that is not provided by conventional MRI. The geometry o...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2006.06.003
更新日期：2006-10-01 00:00:00
abstract：BACKGROUND:According to criteria for the classification of Systemic Lupus Erythematosus (SLE), thrombocytopenia is one of the disease-defining hematologic disorders. Since the recognition of Antiphospholipid Syndrome (APS), thrombocytopenia was frequently reported but several studies yielded contradictory results on th...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,meta分析
doi：10.1016/j.autrev.2019.102395
更新日期：2019-11-01 00:00:00
abstract：:The pathogenesis of Sjögren's syndrome is poorly understood. Genetic and environmental factors appear to contribute to the development of this syndrome. Viral infection is one of the most likely environmental factors. The primary lesion of Sjögren's syndrome is in the exocrine glands. A majority of the infiltrating ce...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/s1568-9972(02)00121-0
更新日期：2003-01-01 00:00:00
abstract：:E26 transformation-specific-1 (Ets-1) belongs to the Ets family of transcription factors which share a common 85-amino-acid DNA-binding domain. Ets-1 is essential to regulation of the immune system including immune cell proliferation and differentiation. Past data demonstrated Ets-1 play a role in negative regulation ...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2011.01.007
更新日期：2011-06-01 00:00:00
abstract：:A few scores predicting the short-term risk of mortality in Systemic sclerosis (SSc) have been reported to date. Our study aimed to create a predictive 15-year all-cause mortality score at the time of the diagnosis of SSc. The study was based on the Spanish Scleroderma Registry (RESCLE). The cohort was split up in der...
journal_title：Autoimmunity reviews
pub_type： 杂志文章
doi：10.1016/j.autrev.2020.102507
更新日期：2020-05-01 00:00:00
abstract：INTRODUCTION:The upcoming of biosimilars in rheumatic diseases have generated considerable interest throughout the past five years among pharmaceutical industries and regulatory agencies, their development is associated with considerable variation and heterogeneity on the variable requirements for license and marketing...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2018.09.005
更新日期：2019-02-01 00:00:00
abstract：:Autoimmune rheumatic diseases (ARD) are prevalent in women during their childbearing age. For their treatment, high doses of corticosteroid (CS) for long-term periods are often required, increasing the risk of bone loss. According to recent guidelines, bisphosphonates (BP) should be used as first line treatment to pre...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.03.002
更新日期：2010-06-01 00:00:00
abstract：:Autoimmune skin diseases represent a heterogeneous group of disorders with grossly diverging clinical manifestations but partly shared underlying immunological mechanisms. They may affect the skin as an isolated organ or among systemic diseases. In addition unspecific cutaneous symptoms or drug-induced unwanted effect...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2011.05.017
更新日期：2012-01-01 00:00:00
abstract：:Pulmonary manifestations in antiphospholipid syndrome (APS) are relatively rare compared to other clinical signs of this disease. However, pulmonary microthrombosis is among the most frequent arterial complications of APS. Timely diagnosis of pulmonary manifestations is required due both to their severity and to the h...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2006.02.002
更新日期：2006-05-01 00:00:00
abstract：OBJECTIVE:To describe the clinical and immunologic features of 6 patients with rheumatic disease and Hepatitis C Virus (HCV) chronic infection, treated with anti-TNF alpha drugs. PATIENTS AND METHODS:Six patients, with repeated positive serology for HCV infection, were affected by Rheumatoid arthritis (RA) (4 cases), ...
journal_title：Autoimmunity reviews
pub_type： 杂志文章
doi：10.1016/j.autrev.2008.05.002
更新日期：2008-12-01 00:00:00
