Abstract：
OBJECTIVE:To investigate differences between childhood (cPAN)- and adult-onset polyarteritis nodosa (aPAN) patients. METHODS:cPAN patients' clinical findings at onset and outcomes were compared to those of aPAN patients from the French Vasculitis Study Group registry matched for year of enrollment and initial systemic versus cutaneous disease. Their information on medications, disease activity and damage were collected. Kaplan-Meier relapse-free survival curves and the log-rank test were used to analyze cPAN versus aPAN differences for predefined outcomes. RESULTS:Twenty-one children with systemic and 13 with cutaneous PAN were compared with 84 systemic- and 27 cutaneous-matched aPAN patients. Median follow-up exceeded 5 years for both groups. At study entry, mononeuritis multiplex was less frequent in systemic cPAN than systemic aPAN (P = 0.04), and purpura and myalgias were less frequent in cutaneous cPAN than cutaneous aPAN (P < 0.03). During follow-up, systemic cPAN relapsed more often than matched systemic aPAN (P < 0.0001), while relapse rates were similar for cutaneous disease (P > 0.05). Mostly minor relapses, predominantly involving the skin, occurred in all 4 groups. At last visit, damage accrual was comparable for cPAN and aPAN patients, but fewer systemic cPAN patients were treatment-free (15% versus 42%; P = 0.03). Two (6%) cPAN and 8 (7%) aPAN patients died. CONCLUSION:Systemic PAN is equally severe in children and adults and carries a higher risk of relapse. The main cutaneous PAN features seem not to be influenced by age at disease onset.
journal_name
Autoimmun Revjournal_title
Autoimmunity reviewsauthors
Iudici M,Quartier P,Pagnoux C,Merlin E,Agard C,Aouba A,Roblot P,Cohen P,Terrier B,Mouthon L,Guillevin L,Puéchal X,French Vasculitis Study Group.doi
10.1016/j.autrev.2018.08.001subject
Has Abstractpub_date
2018-10-01 00:00:00pages
984-989issue
10eissn
1568-9972issn
1873-0183pii
S1568-9972(18)30190-3journal_volume
17pub_type
杂志文章,评审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：: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：: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
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abstract：:Intravenous immunoglobulin (IVIg) is used for replacement therapy in immunodeficiency states and for immunomodulation of various autoimmune diseases. Several case reports and series support a beneficial role of IVIg in systemic lupus erythematosus (SLE), both as salvage immunotherapy and in control of disease activity...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2005.09.002
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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 clini...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2017.02.010
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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：:Much progress has been made in understanding autoimmune channelopathies, but the underlying pathogenic mechanisms are not always clear due to broad expression of some channel proteins. Recent studies show that autoimmune conditions that interfere with neurovascular coupling in the central nervous system (CNS) can lead...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2014.11.010
更新日期：2015-03-01 00:00:00
abstract：:Since many years immunologists have being tried to answer the tantalizing enigma of immunological tolerance. Complex mechanisms in both thymus (central tolerance) and peripheral lymphoid organs (peripheral tolerance) underly lymphocyte tolerance and its maintenance. The genesis of autoimmunity involves environmental a...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.08.019
更新日期：2011-01-01 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：: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:Adrenal insufficiency (AI) is associated with high morbidity and mortality. The aim of this systematic review was to enhance diagnostic approaches and summarize therapeutic strategies in the management of AI in patients with systematic lupus erythematosus (SLE) or antiphospholipid syndrome (APS). METHODS:A ...
journal_title：Autoimmunity reviews
pub_type： 杂志文章
doi：10.1016/j.autrev.2018.06.014
更新日期：2019-01-01 00:00:00
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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：: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：: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：:Intravenous immunoglobulin (IVIG) is efficient in various immune mediated conditions. Various cardiovascular diseases are mediated by inflammatory processes and autoimmune mechanisms. Therefore, it seems conceivable to employ IVIG as an immunomodulating therapy in such indications. In this paper we review the possible...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2008.04.001
更新日期：2008-06-01 00:00:00
abstract：:IgG4-related disease is a distinct clinical entity, whose characteristic features are the following; Serum IgG4 is prominently elevated, IgG4-positive plasma cells infiltrate in involved tissues, various mass-forming lesions with fibrosis develop in a timely and spatial manner and the response to corticosteroids is pr...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.05.003
更新日期：2010-07-01 00:00:00
abstract：:Systemic lupus erythematosus (SLE) is a chronic immune-mediated inflammatory multisystem disease. The onset of viral and bacterial infections may favor the exacerbation of the disease, amplify autoimmune processes and contribute to mortality and morbidity. The prevention of influenza and Streptococcus pneumoniae infec...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2013.07.007
更新日期：2014-02-01 00:00:00
abstract：:In addition to cause acute and chronic liver disease, hepatitis C virus (HCV) infection is frequently associated to autoimmune disorders, such as mixed cryoglobulinemia, primary glomerulonephritis, monoclonal gammopathy of undetermined significance and post-transplant proliferative disorders. Progression to malignant ...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2009.03.008
更新日期：2009-12-01 00:00:00
abstract：:The earliest experimental trials of monoclonal antibodies took place in the early 1980s using murine monoclonal antibodies directed at human lymphoma and leukemia cell antigens. Since those early trials, therapeutic advances have produced monoclonal antibodies and fusion proteins that are now used in multiple disease ...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2016.07.001
更新日期：2016-09-01 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：:Myasthenia gravis is caused by antibodies to the acetylcholine receptor, muscle-specific kinase, low-density lipoprotein receptor-related protein 4, or possibly yet unidentified antibodies. The mechanisms by which these antibodies interfere with the function of postsynaptic proteins include complement activation, anti...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2013.03.001
更新日期：2013-07-01 00:00:00
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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
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2017.11.004
更新日期：2018-01-01 00:00:00
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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：:The loss of immune tolerance to self antigens leads to the development of autoimmune responses. Since self antigens are often multiple and/or their sequences may be known, one approach to restore immune tolerance uses synthetic artificial peptides that interfere or compete with self peptides in the networks of cellula...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2010.08.017
更新日期：2010-11-01 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：: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：:Coexisting morbidities in CVID include bronchiectasis, autoimmunity and malignancies. The incidence of autoimmune disease in CVID patients may approach 20% of cases. The most common autoimmune disease found in CVID patients is autoimmune cytopenia, but rheumatoid arthritis, lupus, and now primary biliary cirrhosis hav...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2014.04.006
更新日期：2014-08-01 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 characterization of CD4(+)CD25(+) regulatory T (Treg) cells in mice, significant progress has been made in the definitions of the phenotype and the function of human Treg cells in health and in pathological conditions. Recent advances in the field leading to a better molecular definition of Treg subsets in h...
journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2011.05.004
更新日期：2011-10-01 00:00:00
