Abstract:
:Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur. Data from literature on the incidence of the different clinical manifestations of NLS are difficult to compare because they come mostly from retrospective studies or prospective studies, but up to date no systematic follow-up was carried out. We performed a large prospective single-center study with a systematic clinical and instrumental follow-up until 9months of life, in order to evaluate the incidence and the clinical impact of NLS features. From 2004 to 2014 all infants born in our center to mothers with anti-SSA/Ro Ab were enrolled in a specific diagnostic and follow-up (FU) program. At birth, 50 infants born to mothers with anti-SSA/Ro Ab were found positive for anti-SSA/Ro Ab. Infants were tested for anti SSA/Ro Ab at 3months of life, if positive they were re-tested at 6 and 9months. At 9months anti-SSA/Ro Ab were positive in 10% of children. In two cases (4%) a CCHB was identified during pregnancy and required pacemaker implantation at birth. In 10% of cases a transient ECG alterations was found during follow-up. Hematological NLS features (anemia, neutropenia, thrombocytopenia) were found at birth and during FU in several patients, in all cases without clinical manifestations and in most cases with complete normalization at 9months. Mild and transient elevation of aminotransferases between 3 and 6months of life were found in 56% and 40% of patient, respectively; non-specific ultrasound cerebral anomalies in absence of clinical neurological signs were found at birth in 9 patients (18%), subsequently normalized. Prenatal maternal screening is of primary importance in order to early detect CCHB, which requires maternal treatment and pacemaker implantation at birth. Infants born to mothers with anti-SSA/Ro Ab should be monitored for all NLS features at birth. However, during the first months of life, these infants seem to develop only mild, transient and self-limited clinical manifestations, which in most cases are completely solved at 9months of life. This consideration, together with the evidence that only 10% of infants had anti-SSA/Ro Ab persistent in blood at 9months, suggests that follow-up of these children can be performed until 6-9months of life with good clinical safety. Moreover, a clinical and laboratory monitoring at 3months of life, when the highest incidence of hematological features and liver tests alterations are observed, is strongly recommended. In the future, it would be clarified if a follow-up until adulthood would be indicated in cases with persistent anti SSA/Ro or in all infants born to mother with anti SSA/Ro.
journal_name
Autoimmun Revjournal_title
Autoimmunity reviewsauthors
Zuppa AA,Riccardi R,Frezza S,Gallini F,Luciano RM,Alighieri G,Romagnoli C,De Carolis Sdoi
10.1016/j.autrev.2017.02.010subject
Has Abstractpub_date
2017-04-01 00:00:00pages
427-432issue
4eissn
1568-9972issn
1873-0183pii
S1568-9972(17)30050-2journal_volume
16pub_type
杂志文章,评审abstract::This Special Issue of Autoimmunity Reviews constitutes summaries of presentations at the 20th International Meeting on Immunopathology and Orphan Diseases, held in Torino, Italy, 25-28th January 2017. As such, these presentations represent the state of the art on the pathophysiology of autoimmune diseases as well as t...
journal_title:Autoimmunity reviews
pub_type: 社论
doi:10.1016/j.autrev.2017.07.015
更新日期:2017-10-01 00:00:00
abstract:BACKGROUND:Myositis is a heterogeneous group of muscular auto-immune diseases with clinical and pathological criteria that allow the classification of patients into different sub-groups. Inclusion body myositis is the most frequent myositis above fifty years of age. Diagnosing inclusion body myositis requires expertise...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2019.02.003
更新日期:2019-04-01 00:00:00
abstract::As part of a landmark review of the antigen excess, Jacobs et al. (Autoimmunity Reviews 2015;14:160-167) contrast how immunoassay "interference" by non-target biomolecules can cause spurious readings for clinical diagnostic tests. The purpose of the present Letter is to complement and analytically extend this descript...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2020.102663
更新日期:2020-11-01 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::Raynaud's phenomenon is an episodic vasospasm of the peripheral arteries, causing pallor followed by cyanosis and redness with pain and sometimes paraesthesia, and, rarely, ulceration of the fingers and toes. Primary or idiopathic Raynaud's phenomenon (Raynaud's disease) occurs without an underlying disease. Secondary...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2008.07.002
更新日期:2008-10-01 00:00:00
abstract::Type 1 diabetes (T1D) and the autoimmune thyroid diseases (AITD) are the most common autoimmune endocrine diseases. Both are organ-specific T-cell mediated diseases. Abundant epidemiological data support a strong genetic basis for both T1D and AITD. Furthermore, both diseases commonly occur in the same individuals and...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/s1568-9972(03)00080-6
更新日期:2003-10-01 00:00:00
abstract::Multiple sclerosis (MS) is a chronic immune-mediated demyelinating disease of the central nervous system characterized by relapses and remissions. The risk of acquiring this complex disease is associated with exposure to environmental factors in genetically susceptible individuals. The epidemiology of MS has been exte...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2009.11.010
更新日期:2010-03-01 00:00:00
abstract:AIMS:Oxidation reactions can modify protein activity or specificity. Recently, a novel redox-reactive family of autoantibodies was described, which indicated involvement of altered antibodies (beside altered antigens) into autoimmune reactions. The aim of our study was to determine the binding capacity alterations of e...
journal_title:Autoimmunity reviews
pub_type: 杂志文章
doi:10.1016/j.autrev.2008.04.005
更新日期:2008-07-01 00:00:00
abstract::T cells from patients with SLE produce decreased amounts of interleukin-2 (IL-2), a central cytokine in the regulation of the immune response. We discuss herein the abnormalities underlying IL-2 deficiency in SLE T cells. ...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2005.08.009
更新日期:2006-02-01 00:00:00
abstract::Hyperhomocysteinemia is independently associated with the development of coronary, cerebral and peripheral vascular disease and deep-vein thrombosis in the general population. The evidence that cardiovascular involvement is particularly frequent and advanced in patients affected with several autoimmune diseases (AD), ...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2007.03.008
更新日期:2007-08-01 00:00:00
abstract::A wide variety of rheumatic diseases has been documented in the presence of hepatitis C virus (HCV) infection and in human immunodeficiency virus (HIV) infection. In this conditions, physicians are refrained from using corticosteroids and/or immunosuppressants agents because of the risk of favouring viral replication ...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2008.07.009
更新日期:2008-12-01 00:00:00
abstract::Parkinson's disease, Alzheimer's disease and other neurodegenerative disorders share a common pathologic pathway with aggregation and deposition of misfolded proteins causing a disruption of particular neuronal networks. Several mechanisms have been implicated in the down-stream events following deposition of misfolde...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2008.04.010
更新日期:2008-06-01 00:00:00
abstract::Type 1 diabetes (T1DM) is an autoimmune disease with aberrant immune responses to specific β-cell autoantigens, resulting in insulin deficiency. Children and adolescents with T1DM may also develop organ-specific multiple autoimmunity in the context of APS (autoimmune polyendocrine syndrome) type 1, 2 or 3. The most fr...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2015.05.002
更新日期:2015-09-01 00:00:00
abstract::The ontogenetic development of both the immune and the nervous system entirely depend on external environmental signals that induce a lifelong learning process. The resulting collective immunological knowledge about the external world is transmitted in an epi-genetic fashion to the offspring, but only from the materna...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2008.12.005
更新日期:2009-03-01 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::Experimental analyses and literature survey reveal low-redundancy to the host proteins as a common denominator of immunogenic sequences mapped along tumor-, autoimmune-, and infectious disease-associated-proteins. The hypothesis that immunogenicity of peptide sequences is linked to proteomic redundancy is discussed. ...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2006.09.004
更新日期:2007-04-01 00:00:00
abstract::Different types of infection are implicated in the pathogenesis of autoimmune thyroid diseases (AITD) through molecular mimicry or other mechanisms, but their role is disputed. Human studies support direct or indirect evidence of involvement of some viral and bacterial agents, but reports have provided conflicting and...
journal_title:Autoimmunity reviews
pub_type: 杂志文章
doi:10.1016/j.autrev.2008.07.013
更新日期:2008-12-01 00:00:00
abstract::Compared to lower metazoans, vertebrates built up an exclusively new set of adhesion-related genes involved in the tissue development and in their functions. They include a large variety of extracellular matrix proteins and their heterodimeric integrin adhesive receptors. Integrins control the adhesive state of the ce...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2007.03.007
更新日期:2007-11-01 00:00:00
abstract::Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness, fatigability, and autoantibodies against protein antigens of the muscle endplate. Antibodies against acetylcholine receptor (AChR), and less frequently against muscle-Specific Kinase (MuSK) or lipoprotein related protein 4 (LRP4) occur i...
journal_title:Autoimmunity reviews
pub_type: 杂志文章
doi:10.1016/j.autrev.2014.08.039
更新日期:2014-10-01 00:00:00
abstract::Antiphospholipid syndrome is characterized with thrombotic events and/or pregnancy morbidity and antiphospholipid antibodies (aPL). The most common antigen for aPL is beta2-glycoprotein-I (beta(2)GPI), a plasma protein binding to negatively charged phospholipids. The influence of aPL on coagulation is not well underst...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2006.03.001
更新日期:2006-11-01 00:00:00
abstract::The "exposome" is a term recently used to describe all environmental factors, both exogenous and endogenous, which we are exposed to in a lifetime. It represents an important tool in the study of autoimmunity, complementing classical immunological research tools and cutting-edge genome wide association studies (GWAS)....
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2012.12.005
更新日期:2013-05-01 00:00:00
abstract:BACKGROUND:Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare multi-system autoimmune disease, characterised by a pauci-immune necrotising small-vessel vasculitis, with a relapsing and remitting course. Like many autoimmune diseases, the exact aetiology of AAV, and the factors that influenc...
journal_title:Autoimmunity reviews
pub_type: 杂志文章
doi:10.1016/j.autrev.2020.102660
更新日期:2020-11-01 00:00:00
abstract::Effective immune responses require the appropriate activation and differentiation of peripheral CD4(+) T cells. These processes need to be followed by the timely elimination of the responding T cells in order to restore T cell homeostasis. Defects in the appropriate regulation of T cell activation, expansion, and surv...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2008.07.044
更新日期:2009-01-01 00:00:00
abstract::Since the earliest reports in 2001, immunoglobulin G4 (IgG4)-related disease has been defined as an autoimmune systemic disease characterized by the lymphoplasmacytic infiltration of affected tissues leading to fibrosis and obliterative phlebitis along with elevated serum IgG4 levels. Prior to this unifying hypothesis...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2015.06.003
更新日期:2015-10-01 00:00:00
abstract::There is strong evidence that a quantitative and/or functional deficiency in CD4+CD25+ regulatory T cells (T(reg) cells) plays a key role in the pathogenesis of many human autoimmune diseases. Therefore, targeting regulatory T cells with novel forms of immunotherapy should provide a means for successfully battling aut...
journal_title:Autoimmunity reviews
pub_type: 杂志文章
doi:10.1016/j.autrev.2005.06.001
更新日期:2006-01-01 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::Autotaxin (ATX) is a 125-kD type II ectonucleotide pyrophosphatase/phosphodiesterase (ENPP2 or NPP2) originally discovered as an unknown "autocrine motility factor" in human melanoma cells. In addition to its pyrophosphatase/phosphodiesterase activities ATX has lysophospholipase D (lysoPLD) activity, catalyzing the co...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2016.03.019
更新日期:2016-08-01 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::Autoimmunity results from a deficiency in tolerance establishment, i.e. a failure of the organism to eliminate or functionally neutralize auto-reactive lymphocytes. Dendritic cells (DC) are specialized migratory antigen presenting cells (APC), found as sentinels in peripheral tissues and lymphoid organs that allow the...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/S1568-9972(03)00066-1
更新日期:2004-01-01 00:00:00
abstract::Successful therapy of dementia, like any disease, depends upon understanding its pathogenesis. This review contrasts the dominant pathways to dementia which differ in Alzheimer's disease (AD) and in Down's syndrome (DS). Impaired clearance of neurotoxic amyloid beta peptides (Abeta) leads to dementia in AD. In DS over...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2012.10.013
更新日期:2013-04-01 00:00:00