Abstract:
:Autoinflammatory diseases are genetic or acquired clinical entities globally caused by the aberrant release of the proinflammatory cytokine interleukin-1 and mostly characterized by recurrent spontaneous inflammatory events which do not produce antigen-specific T cells or autoantibodies. Within the past decade, the list of autoinflammatory diseases has included cryopyrin-associated periodic syndromes, familial Mediterranean fever, mevalonate kinase deficiency, tumor necrosis factor receptor-associated periodic syndrome, hereditary pyogenic disorders, pediatric granulomatous autoinflammatory diseases, idiopathic febrile syndromes, complement dysregulation syndromes and Behçet's disease. Most of these conditions interact with the inflammasomes, intracellular molecular complexes coordinating the phylogenetically ancient response of the innate immune system. The pathogenetic mechanisms of these diseases have shown the evidence of disrupted interleukin-1 signaling for most of them and allowed to locate interleukin-1 as an attractive therapeutic target. The whole fresco of autoinflammatory diseases in pediatrics will be discussed in this review with the aim of establishing both diagnostic clues and treatments for each condition.
journal_name
Autoimmun Revjournal_title
Autoimmunity reviewsauthors
Rigante Ddoi
10.1016/j.autrev.2011.10.008subject
Has Abstractpub_date
2012-03-01 00:00:00pages
348-56issue
5eissn
1568-9972issn
1873-0183pii
S1568-9972(11)00215-1journal_volume
11pub_type
杂志文章,评审abstract::Studies of accuracy are often more complex to understand than clinical trials, since there can be more than one outcome and scope (screening, diagnosis, and prognosis) and because results have to be reported in more than one way, than in clinical trials (relative risk or odds ratio). Sensitivity and specificity are co...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,meta分析,评审
doi:10.1016/j.autrev.2012.07.004
更新日期:2012-12-01 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
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...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2018.08.001
更新日期:2018-10-01 00:00:00
abstract::When the diagnosis of systemic sclerosis (SSc) is made and you are told "You have SSc", it is a strange feeling for the patient, because you don't yet know what it is exactly. It is a very intense shock to hear this, and it is also difficult to try to imagine what will follow. There is no cure for SSc; there are just ...
journal_title:Autoimmunity reviews
pub_type: 杂志文章
doi:10.1016/j.autrev.2010.09.009
更新日期:2011-03-01 00:00:00
abstract::Impaired clearance of apoptotic cell material has been implicated in the pathogenesis of systemic lupus erythematosus (SLE). Besides many other molecules, C1q and DNaseI contribute to an efficient clearance of dying cells. A frequently observed factor in SLE patients is the accumulation of unusually large amounts of a...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2004.10.007
更新日期:2005-04-01 00:00:00
abstract::Autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, multiple sclerosis and inflammatory bowel disease, have complex pathogeneses and likely multifactorial etiologies. The current paradigm for understanding their development is that the disease is triggered in genetically-susceptible individ...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2011.10.022
更新日期:2012-06-01 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
abstract::Autoimmunity is increasingly accepted as the origin or amplifier of various diseases. In contrast to classic autoantibodies (AABs), which induce immune responses resulting in the destruction of the affected tissue, an additional class of AABs is directed against G-protein-coupled receptors (GPCRs; GPCR-AABs). GPCR-AAB...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2017.01.012
更新日期:2017-03-01 00:00:00
abstract:OBJECTIVE:To assess the effectiveness and safety of a protocol using medium doses of prednisone to treat lupus nephritis. METHODS:Patients receiving the 'Cruces-protocol cohort' (CPC) were paired 1:2 with patients from the 'historic cohort' (HC). The CPC received medium doses of prednisone combined with methyl-prednis...
journal_title:Autoimmunity reviews
pub_type: 杂志文章
doi:10.1016/j.autrev.2013.10.013
更新日期:2014-02-01 00:00:00
abstract::Myocarditis is a major cause of heart disease in young patients and a common precursor of heart failure due to dilated cardiomyopathy. Some auto-immune and/or auto-inflammatory diseases may be accompanied by myocarditis, such as sarcoidosis, Behçet's disease, eosinophilic granulomatosis with polyangiitis, myositis, an...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2017.05.021
更新日期:2017-08-01 00:00:00
abstract::Immune thrombocytopenia (ITP) is a rare autoimmune disease due to an abnormal T cell response, notably supported by splenic T follicular helper cells, that stimulates the proliferation and differentiation of autoreactive B cells. The antiplatelet autoantibodies they produce facilitate platelet phagocytosis by macropha...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2017.04.012
更新日期:2017-06-01 00:00:00
abstract::Autoimmune diseases refer to a spectrum of diseases characterized by an active immune response against the host, which frequently involves increased autoantibody production. The pathogenesis of autoimmune diseases is multifactorial and the exploitation of novel effective treatment is urgent. Capsaicin is a nutritional...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2016.01.009
更新日期:2016-05-01 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
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::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::Microscopic polyangiitis (MPA) is a member of the family of ANCA-associated vasculitides. Its characteristic histology shows a necrotizing small vessel vasculitis with little or absent immune deposits (pauci-immune vasculitis). In Western countries MPA shows a lower prevalence than Wegener's disease, it affects more m...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2010.07.009
更新日期:2010-10-01 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::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::Anti-RNA polymerase III antibodies (ARA) are a specific marker for Systemic Sclerosis (SSc), associated to severe disease with major organ and diffuse cutaneous involvement. In our series, ARA were found in 19 of 216 sera, in 15 cases as isolated antibodies' specificity, with a statistically negative association with ...
journal_title:Autoimmunity reviews
pub_type: 杂志文章
doi:10.1016/j.autrev.2009.02.002
更新日期:2009-06-01 00:00:00
abstract::Autoimmune Syndrome Induced by Adjuvant (ASIA) is a definition aimed to describe the common etiological process at the root of five clinical entities sharing similar symptomatology: macrophagic myofasciitis syndrome (MMF), Gulf War Syndrome (GWS), sick building syndrome (SBS), siliconosis, and post vaccination autoimm...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2017.11.037
更新日期:2018-05-01 00:00:00
abstract:OBJECTIVES:A reliable tool to evaluate flow is paramount in systemic sclerosis (SSc). We describe herein on the one hand a systematic literature review on the reliability of laser speckle contrast analysis (LASCA) to measure the peripheral blood perfusion (PBP) in SSc and perform an additional pilot study, investigatin...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2018.01.023
更新日期:2018-08-01 00:00:00
abstract::The term pulmonary renal vasculitis syndrome describes a clinical syndrome of diffuse alveolar haemorrhage (DAH) complicating acute glomerulonephritis that often heralds severe, life-threatening systemic vasculitis requiring urgent, aggressive therapy. "Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2010.05.012
更新日期:2010-08-01 00:00:00
abstract::Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular obliteration, excessive extracellular matrix deposition and fibrosis of the connective tissues of the skin, lungs, gastrointestinal tract, heart, and kidneys. Numerous infectious agents (bacterial and viral) have been proposed as possible trig...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2008.07.022
更新日期:2008-10-01 00:00:00
abstract::Endothelial dysfunction and microvascular damage constitute the hallmarks of systemic sclerosis (SSc), explaining much of the pathophysiology and clinical manifestations of the disease. Nailfold videocapillaroscopy (NVC) is an established method for the assessment of the microvasculature, aiding in distinguishing diff...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2017.05.019
更新日期:2017-08-01 00:00:00
abstract::Rheumatoid arthritis (RA) is a chronic inflammatory joint disease characterized by a distinctive pattern of bone and joint destruction. RA patients have an increased risk of death. The incidence and prevalence of RA vary across populations, statistical methods, and disease definitions. In North America and Northern Eu...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2009.11.019
更新日期:2010-03-01 00:00:00
abstract::Catastrophic antiphospholipid syndrome (CAPS) (Asherson's Syndrome), is a life-threatening condition characterized by a rapidly progressive thromboses resulting in a multiorgan dysfunction syndrome (MODS), evidence of systemic inflammatory response syndrome (SIRS) in the presence of antiphospholipid antibodies. CAPS d...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2006.06.011
更新日期:2006-12-01 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::The concept of undifferentiated spondyloarthritis has been introduced recently to describe a clinical setting where the classical features of spondyloarthritis (SpA) are not fully present. Whether this is a discrete entity was the basis of a debate during the 4th International Congress on Controversies in Rheumatology...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2017.11.006
更新日期:2018-01-01 00:00:00
abstract:OBJECTIVE:Toll-like receptor (TLR) 4 (+896 A/G) gene polymorphism has been reported to be associated with susceptibility to giant cell arteritis (GCA) with inconsistent results. To provide a more definitive conclusion, a cumulative meta-analysis of the association of TLR4 (+896 A/G) polymorphism with GCA susceptibility...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,meta分析
doi:10.1016/j.autrev.2011.06.002
更新日期:2011-10-01 00:00:00
abstract::Female predominance is a common characteristic for autoimmune diseases attributed to the combined effect of hormonal influence and genetic factors. Since X chromosome has immunologically important genes, the age related X chromosome loss could contribute to the development of autoimmunity. X chromosome monosomy (XCM) ...
journal_title:Autoimmunity reviews
pub_type: 杂志文章,评审
doi:10.1016/j.autrev.2010.03.001
更新日期:2012-03-01 00:00:00