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1.
RMD Open ; 6(2)2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32611651

RESUMO

A few weeks after the peak of the global 2019 novel coronavirus disease pandemic, cases of shock, multisystem inflammation and severe myocarditis have occurred in children and adolescents, generating some concerns and above all many questions. An almost immediate association raised with shock syndrome related to Kawasaki disease (KD). However, in light of bo/th experience and literature have taught us about severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) infection, and what already known on the epidemiology of KD, we suggest here the hypothesis of a new 'post-viral' systemic inflammatory disease related to excessive adaptive immune response rather than a form of KD caused by SARS-COV-2. We discuss analogies and differences between the two forms.


Assuntos
Infecções por Coronavirus , Síndrome de Linfonodos Mucocutâneos , Pandemias , Pneumonia Viral , Vasculite Sistêmica , Betacoronavirus/isolamento & purificação , Criança , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/fisiopatologia , Diagnóstico Diferencial , Gerenciamento Clínico , Humanos , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Síndrome de Linfonodos Mucocutâneos/imunologia , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , Pneumonia Viral/imunologia , Pneumonia Viral/fisiopatologia , Vasculite Sistêmica/imunologia , Vasculite Sistêmica/fisiopatologia , Vasculite Sistêmica/terapia , Terminologia como Assunto
2.
Autoimmun Rev ; 19(5): 102514, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32171919

RESUMO

In recent years, insight into immune pathogenesis and treatment of primary systemic vasculitides (PSV) has increased considerably, and has led to the development of many clinically relevant biomarkers. This review aims to provide an update on the main biomarkers discovered and their potential application to precision medicine in vasculitis. Genetic and molecular profiling of patients and promising biomarkers discoveries are very important for personalized medicine; however, there are very limited data in PSV. Genetic studies including mainly genome-wide association studies (GWAS) had led to important discoveries in disease pathogenesis of PSV while whole exome sequencing studies lead to discovery of monogenic vasculitides. Although there are numerous studies addressing novel biomarkers in PSV, few of these biomarkers are currently being used in routine clinical practice in the management of patients with PSV. Current studies indicate that ANCA types identify distinct prognostic subsets of ANCA vasculitis patients. Today, biomarkers-driven treatment algorithms are not available in PSV.


Assuntos
Medicina de Precisão , Vasculite Sistêmica/diagnóstico , Vasculite Sistêmica/terapia , Biomarcadores/análise , Estudo de Associação Genômica Ampla , Humanos , Vasculite Sistêmica/genética
3.
BMJ Case Rep ; 12(12)2019 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-31801777

RESUMO

Bartonella species are fastidious, Gram-negative aerobic rods and a well-recognised pathogen responsible for culture-negative endocarditis. The histopathological appearance of glomerulonephritis (GN) caused by Bartonella endocarditis may include a pauci-immune GN similar to that usually seen in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Herein, we present an unusual case report of Bartonella endocarditis masquerading as ANCA-positive vasculitis, with crescentic GN. A 66-year-old woman, who had undergone aortic valve replacement 2 years prior to admission, presented with confusion and loss of vision in her right nasal field. Following an extensive diagnostic evaluation, the main findings were right central retinal artery occlusion, ground-glass appearance on chest CT and ANCA-positive, anti PR-3 negative, rapidly progressive GN. The patient was scheduled to start treatment with rituximab for presumed ANCA-positive GN, when a positive serological test for Bartonella henselae was received. In view of this result, a diagnosis of endocarditis was made, based on fulfilment of five Duke minor criteria, namely fever, predisposition, arterial emboli, immunological phenomena and serological evidence of active infection with an organism consistent with infective endocarditis. Immunosuppressive treatment was withheld and antibiotic treatment initiated. This case report emphasises the need for maintaining a high index of suspicion regarding the diagnosis of Bartonella infection, which might mimic ANCA-associated GN.


Assuntos
Infecções por Bartonella/diagnóstico , Endocardite Bacteriana , Glomerulonefrite , Vasculite Sistêmica/diagnóstico , Idoso , Antibacterianos/uso terapêutico , Bartonella , Infecções por Bartonella/tratamento farmacológico , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Humanos , Vasculite Sistêmica/tratamento farmacológico , Transtornos da Visão/microbiologia
4.
Best Pract Res Clin Rheumatol ; 33(4): 101424, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31810548

RESUMO

Giant cell arteritis (GCA) is the most common vasculitis in individuals older than 50 years in Western countries. In addition to the typical pattern of cranial ischemic manifestations, large vessel vasculitis (LVV) involvement has emerged as a common feature of GCA. Patients with predominant LVV manifestations differ from those with the cranial pattern. They are usually affected at a younger age and often have nonspecific manifestations such as constitutional syndrome, fever of unknown origin, or refractory/atypical polymyalgia rheumatica (PMR). In these patients, cranial manifestations are often absent. Furthermore, patients with isolated PMR should be followed up because of the potential risk of severe vascular complications in the setting of an underlying GCA. Whereas temporal artery biopsy and/or color duplex ultrasound of the temporal arteries is useful for the diagnosis of cranial GCA, Doppler sonography of the subclavian and axillary arteries, fluorine-18-fluorodeoxyglucose-positron emission tomography/computed tomography, magnetic resonance, and computed tomography-angiography are very useful to identify the presence of LVV, and they may play a potential role in the follow-up of these patients.


Assuntos
Arterite de Células Gigantes , Polimialgia Reumática , Vasculite Sistêmica , Adulto , Fluordesoxiglucose F18 , Arterite de Células Gigantes/diagnóstico por imagem , Arterite de Células Gigantes/tratamento farmacológico , Humanos , Polimialgia Reumática/diagnóstico por imagem , Polimialgia Reumática/tratamento farmacológico , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Vasculite Sistêmica/diagnóstico por imagem , Vasculite Sistêmica/tratamento farmacológico
5.
RMD Open ; 5(2): e001003, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31673411

RESUMO

Objectives: To analyse the current evidence for the management of large vessel vasculitis (LVV) to inform the 2018 update of the EULAR recommendations. Methods: Two systematic literature reviews (SLRs) dealing with diagnosis/monitoring and treatment strategies for LVV, respectively, were performed. Medline, Embase and Cochrane databases were searched from inception to 31 December 2017. Evidence on imaging was excluded as recently published in dedicated EULAR recommendations. This paper focuses on the data relevant to giant cell arteritis (GCA). Results: We identified 287 eligible articles (122 studies focused on diagnosis/monitoring, 165 on treatment). The implementation of a fast-track approach to diagnosis significantly lowers the risk of permanent visual loss compared with historical cohorts (level of evidence, LoE 2b). Reliable diagnostic or prognostic biomarkers for GCA are still not available (LoE 3b).The SLR confirms the efficacy of prompt initiation of glucocorticoids (GC). There is no high-quality evidence on the most appropriate starting dose, route of administration, tapering and duration of GC (LoE 4). Patients with GCA are at increased risk of dose-dependent GC-related adverse events (LoE 3b). The addition of methotrexate or tocilizumab reduces relapse rates and GC requirements (LoE 1b). There is no consistent evidence that initiating antiplatelet agents at diagnosis would prevent future ischaemic events (LoE 2a). There is little evidence to guide monitoring of patients with GCA. Conclusions: Results from two SLRs identified novel evidence on the management of GCA to guide the 2018 update of the EULAR recommendations on the management of LVV.


Assuntos
Cegueira/prevenção & controle , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Takayasu/tratamento farmacológico , Anticorpos Monoclonais Humanizados/administração & dosagem , Anticorpos Monoclonais Humanizados/uso terapêutico , Antirreumáticos/administração & dosagem , Antirreumáticos/uso terapêutico , Biomarcadores/metabolismo , Quimioterapia Combinada , Feminino , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/metabolismo , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Masculino , Metotrexato/administração & dosagem , Metotrexato/uso terapêutico , Estudos Observacionais como Assunto , Avaliação de Resultados em Cuidados de Saúde , Ensaios Clínicos Controlados Aleatórios como Assunto , Recidiva , Gestão de Riscos , Vasculite Sistêmica/patologia , Arterite de Takayasu/complicações
6.
J Immunotoxicol ; 16(1): 191-200, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31684787

RESUMO

In preclinical toxicity studies, species-foreign proteins administered to animals frequently leads to formation of anti-drug antibodies (ADA). Such antibodies may form circulating immune complexes (CIC) with the administered protein. These CIC can activate the classical complement pathway, thereby forming complement-bound CIC (cCIC); if large of amounts of CIC or cCIC is formed, the clearance mechanism may become saturated which potentially leads to vascular immune complex (IC) deposition and inflammation. Limited information is available on the effect of different treatment related procedures as well as biomarkers of IC-related vascular disease. In order to explore the effect of different dose regimens on IC formation and deposition, and identification of possible biomarkers of IC deposition and IC-related pathological changes, C57BL/6J and BALB/c mice were dosed subcutaneously twice weekly with bovine serum albumin (BSA) for 13 weeks without adjuvant. After 6 and 13 weeks, CIC and cCIC were detected in plasma; after 13 weeks, IC deposition was detected in kidney glomeruli. In particular immunohistochemistry double-staining was shown to be useful for detection of IC deposition. Increasing dosing frequency or changing BSA dose level on top of an already established CIC and cCIC response did not cause changes in IC deposition, but CIC and cCIC concentrations tended to decrease with increased dose level, and increased cCIC formation was observed after more frequent dosing. The presence of CIC in plasma was associated with glomerular IC deposits in the dose regimen study; however, the use of CIC or cCIC as potential biomarkers for IC deposition and IC-related pathological changes, needs to be explored further.


Assuntos
Complexo Antígeno-Anticorpo/análise , Glomerulonefrite/imunologia , Soroalbumina Bovina/toxicidade , Vasculite Sistêmica/imunologia , Animais , Complexo Antígeno-Anticorpo/imunologia , Biomarcadores/análise , Via Clássica do Complemento/efeitos dos fármacos , Via Clássica do Complemento/imunologia , Modelos Animais de Doenças , Estudos de Viabilidade , Feminino , Glomerulonefrite/sangue , Glomerulonefrite/induzido quimicamente , Glomerulonefrite/diagnóstico , Humanos , Imuno-Histoquímica , Glomérulos Renais/irrigação sanguínea , Glomérulos Renais/imunologia , Glomérulos Renais/patologia , Masculino , Camundongos , Soroalbumina Bovina/administração & dosagem , Soroalbumina Bovina/imunologia , Vasculite Sistêmica/sangue , Vasculite Sistêmica/induzido quimicamente , Vasculite Sistêmica/diagnóstico , Testes de Toxicidade/métodos
7.
Arthritis Rheumatol ; 71(11): 1780-1787, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31309732

RESUMO

The systemic large vessel vasculitides consist mainly of giant cell arteritis and Takayasu arteritis. Both diseases affect the large blood vessels and can lead to ischemia and end-organ damage. Ultrasound is an imaging technique that can depict inflammation of the vessel wall in large and medium vessel vasculitis. In this article, we critically review the current evidence for the clinical use of ultrasound for systemic large vessel vasculitides, regarding the clinical applicability, technical requirements, challenges, and cost. A roadmap for the development of a fast-track ultrasound clinic for giant cell arteritis is also provided.


Assuntos
Arterite de Células Gigantes/diagnóstico por imagem , Arterite de Takayasu/diagnóstico por imagem , Ultrassonografia/métodos , Aorta Abdominal/diagnóstico por imagem , Artéria Axilar/diagnóstico por imagem , Artéria Carótida Primitiva/diagnóstico por imagem , Humanos , Artéria Subclávia/diagnóstico por imagem , Vasculite Sistêmica/diagnóstico por imagem , Artérias Temporais/diagnóstico por imagem , Artéria Vertebral/diagnóstico por imagem
9.
Arterioscler Thromb Vasc Biol ; 39(8): 1520-1541, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31189432

RESUMO

Vasculitis is a systemic disease characterized by immune-mediated injury of blood vessels. Current treatments for vasculitis, such as glucocorticoids and alkylating agents, are associated with significant side effects. Furthermore, the management of both small and large vessel vasculitis is challenging because of a lack of robust markers of disease activity. Recent research has advanced our understanding of the pathogenesis of both small and large vessel vasculitis, and this has led to the development of novel biologic therapies capable of targeting key cytokine and cellular effectors of the inflammatory cascade. In parallel, a diverse range of imaging modalities with the potential to monitor vessel inflammation are emerging. Continued expansion of combined structural and molecular imaging using positron emission tomography with computed tomography or magnetic resonance imaging may soon provide reliable longitudinal tracking of vascular inflammation. In addition, the emergence of radiotracers able to assess macrophage activation and immune checkpoint activity represents an exciting new frontier in imaging vascular inflammation. In the near future, these advances will allow more precise imaging of disease activity enabling clinicians to offer more targeted and individualized patient management.


Assuntos
Vasculite Sistêmica/diagnóstico por imagem , Vasculite Sistêmica/tratamento farmacológico , Eosinófilos/imunologia , Humanos , Depleção Linfocítica , Imagem por Ressonância Magnética , Imagem Molecular , Poliarterite Nodosa/diagnóstico por imagem , Poliarterite Nodosa/imunologia , Tomografia por Emissão de Pósitrons , Vasculite Sistêmica/imunologia , Tomografia Computadorizada por Raios X
10.
Clin Exp Rheumatol ; 37 Suppl 117(2): 130-136, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31162033

RESUMO

OBJECTIVES: Cardiovascular (CV) events are highly prevalent in systemic necrotising vasculitides (SNV). Visceral/subcutaneous adipose tissue (VAT/SAT) ratio has been shown to be associated with CV events in various diseases. We aimed to assess the relevance of abdominal adipose tissue measurement to predict major CV events (MCVEs) in SNV. METHODS: Patients with SNV were successively included in a longitudinal study assessing MCVEs and other sequelae. Dual x-ray absorptiometry was performed to evaluate abdominal adipose tissue. Patients were prospectively followed for MCVEs, defined as myocardial infarction, unstable angina, stroke, arterial revascularisation and/or hospitalisation for or death from CV causes. RESULTS: One hundred and twenty consecutive SNV patients were included and analysed (54 males, mean age 53±18 years). High CV risk was found in 28 (23.3%) patients. In univariate analysis, age, male gender, VDI, VAT/SAT ratio and serum troponin level were significantly associated with high CV risk, whereas age and VAT/SAT ratio remained independently associated with high CV risk. Variables associated with high tertile of VAT/SAT ratio included age and metabolic risk factors. After median follow-up of 42 months, 19 (16%) patients experienced MCVEs. Hazard ratios for incident MCVEs compared with 1st tertile of VAT/SAT ratio were 7.22 (1.02-51.3; p=0.048) and 9.90 (3.15-31.2; p=0.0002) in the 2nd and 3rd tertile, respectively. CONCLUSIONS: Abdominal visceral adipose tissue is a reliable surrogate marker of CV risk and predicts incident MCVEs in SNV patients. Abdominal adipose tissue should be probably evaluated routinely in these patients to assess CV risk.


Assuntos
Gordura Abdominal/metabolismo , Doenças Cardiovasculares , Vasculite Sistêmica/complicações , Tecido Adiposo/metabolismo , Adulto , Idoso , Doenças Cardiovasculares/diagnóstico , Feminino , Humanos , Gordura Intra-Abdominal , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Vasculite Sistêmica/metabolismo
11.
Clin Exp Rheumatol ; 37 Suppl 117(2): 3-19, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31162034

RESUMO

Systemic vasculitis are disabling complex disorders potentially involving any organ and system. Tremendous efforts have been made recently in this field with novel insights into pathogenesis and new therapy in the pipeline. Following the previous annual reviews of this one year in review series, in this paper we provide a critical digest of the most recent literature regarding pathogenesis, clinical manifestations and therapy, with the ultimate aim of addressing whether the existing data may open new avenues for precision medicine in these disorders.


Assuntos
Vasculite Sistêmica , Humanos , Vasculite Sistêmica/diagnóstico , Vasculite Sistêmica/terapia
13.
Int J Rheum Dis ; 22(6): 1152-1156, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30968563

RESUMO

Mitochondrial diseases are a group of disorders presenting mainly during infancy due to pathological dysfunction of the mitochondrial respiratory chain. We report a case of mitochondrial disease in an elderly woman complaining of generalized myalgia. A 69-year-old woman was admitted due to fatigue, general weakness, and a drowsy mental status. A brain magnetic resonance imaging (MRI) demonstrated multifocal lesions of increased T2 signal intensity, and laboratory findings were consistent with Fanconi syndrome. During her hospital course, she developed seizures, stress-induced cardiomyopathy, and respiratory failure. A muscle biopsy demonstrated ragged-red fibers in the muscle tissues seen in mitochondrial myopathy. We confirmed an 8 kb deletion in her mitochondrial DNA. Following treatment with l-carnitine, coenzyme Q10, and supportive measures, brain lesions on MRI scans disappeared, and the general symptoms gradually improved.


Assuntos
Síndrome de Fanconi/diagnóstico , Miopatias Mitocondriais/diagnóstico , Vasculite Sistêmica/diagnóstico , Idade de Início , Idoso , Diagnóstico Diferencial , Síndrome de Fanconi/genética , Síndrome de Fanconi/terapia , Feminino , Humanos , Miopatias Mitocondriais/genética , Miopatias Mitocondriais/terapia , Valor Preditivo dos Testes , Prognóstico
14.
Neurol Clin ; 37(2): 249-265, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30952408

RESUMO

"Neuroimaging plays a vital role in the diagnosis of primary and secondary vasculitic disorders. There multiple neuroimaging options available to accurately describe the underlying clinical deficits of involved cases. Noninvasive neuroimaging modalities provide less risk and when interdigitated, form the basis for a more conclusive understanding of the disease process. There are instances in which invasive cerebral angiography may be needed to image the intricate and at times, small involved vessels. Neuroradiologists should be included in the multidisciplinary team of physicians caring for patients with vasculitides and in research to provide more sensitive and safe modalities for accurate diagnosis."


Assuntos
Neuroimagem/métodos , Vasculite Sistêmica/diagnóstico por imagem , Humanos
15.
Neurol Clin ; 37(2): 383-397, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30952415

RESUMO

There have been significant advances in the understanding of the vasculitides in the past several years, leading to more precise classification and nosology. Ophthalmologic manifestations may be the presenting feature of and a clue to the diagnosis of vasculitis, or develop in the course of the illness owing to a common disease mechanism. Precise diagnosis and prompt treatment prevents short- and long-term ophthalmologic sequela.


Assuntos
Oftalmopatias/etiologia , Vasculite Sistêmica/complicações , Vasculite Sistêmica/diagnóstico , Humanos
16.
Neurol Clin ; 37(2): 399-423, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30952416

RESUMO

The diagnosis of primary central and peripheral nerve vasculitides should be established with certainty if suspected before commencing potent immunosuppressive therapy. The aim of induction therapy is to rapidly control the underlying inflammatory response and stabilize the blood-brain and blood-nerve barriers, followed by maintenance immunosuppression tailored to the likeliest humoral and cell-mediated autoimmune inflammatory vasculitic processes.


Assuntos
Doenças do Sistema Nervoso Periférico/terapia , Vasculite Sistêmica/terapia , Vasculite do Sistema Nervoso Central/terapia , Humanos , Doenças do Sistema Nervoso Periférico/etiologia , Vasculite Sistêmica/complicações
17.
Neurol Clin ; 37(2): 465-473, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30952419

RESUMO

Systemic and localized vasculitis affects the skin and subcutis, due to large vascular beds and hemodynamic factors, such as stasis in lower extremities, and environmental influences, as occur in cold exposure. Initial cutaneous manifestations of vasculitides include diverse and dynamic patterns of discoloration, swelling, hemorrhage, and necrosis. One-half of affected patients present with localized, self-limited disease to the skin without any known trigger or associated systemic disease, known as idiopathic cutaneous leukocytoclastic vasculitis. Skin biopsy and dermatopathology contribute relevant information; however, they require correlation with clinical history, physical examination, and laboratory findings to reach an accurate diagnosis.


Assuntos
Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologia , Vasculite Sistêmica/complicações , Humanos
18.
Arthritis Rheumatol ; 71(10): 1747-1755, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31008556

RESUMO

OBJECTIVE: Individuals with deficiency of adenosine deaminase 2 (DADA2), a recently recognized autosomal recessive disease, present with various systemic vascular and inflammatory manifestations, often with young age at disease onset or with early onset of recurrent strokes. Their clinical features and histologic findings overlap with those of childhood-onset polyarteritis nodosa (PAN), a primary "idiopathic" systemic vasculitis. Despite similar clinical presentation, individuals with DADA2 may respond better to biologic therapy than to traditional immunosuppression. The aim of this study was to screen an international registry of children with systemic primary vasculitis for variants in ADA2. METHODS: The coding exons of ADA2 were sequenced in 60 children and adolescents with a diagnosis of PAN, cutaneous PAN, or unclassifiable vasculitis (UCV), any chronic vasculitis with onset at age 5 years or younger, or history of stroke. The functional consequences of the identified variants were assessed by ADA2 enzyme assay and immunoblotting. RESULTS: Nine children with DADA2 (5 with PAN, 3 with UCV, and 1 with antineutrophil cytoplasmic antibody-associated vasculitis) were identified. Among them, 1 patient had no rare variants in the coding region of ADA2 and 8 had biallelic, rare variants (minor allele frequency <0.01) with a known association with DADA2 (p.Gly47Arg and p.Gly47Ala) or a novel association (p.Arg9Trp, p.Leu351Gln, and p.Ala357Thr). The clinical phenotype varied widely. CONCLUSION: These findings support previous observations indicating that DADA2 has extensive genotypic and phenotypic variability. Thus, screening ADA2 among children with vasculitic rash, UCV, PAN, or unexplained, early-onset central nervous system disease with systemic inflammation may enable an earlier diagnosis of DADA2.


Assuntos
Adenosina Desaminase/genética , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/genética , Peptídeos e Proteínas de Sinalização Intercelular/genética , Poliarterite Nodosa/genética , Adenosina Desaminase/deficiência , Adolescente , Idade de Início , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Peptídeos e Proteínas de Sinalização Intercelular/deficiência , Masculino , Mutação , Dermatopatias Vasculares/genética , Vasculite Sistêmica/genética
19.
Autoimmun Rev ; 18(6): 593-606, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30959208

RESUMO

Systemic vasculitis is diverse group of autoimmune disorders which are characterized by inflammation of blood vessel walls with deep aching and burning pain. Their underlying etiology and pathophysiology still remain poorly understood. Extracellular vesicles (EVs), including exosomes, microvesicles (MVs), and apoptotic bodies, are membrane vesicular structures that are released either during cell activation, or when cells undergo programmed cell death, including apoptosis, necroptosis, and pyroptosis. Although EVs were thought as cell dusts, but now they have been found to be potently active since they harbor bioactive molecules, such as proteins, lipids, nucleic acids, or multi-molecular complexes. EVs can serve as novel mediators for cell-to-cell communications by delivery bioactive molecules from their parental cells to the recipient cells. Earlier studies mainly focused on MVs budding from membrane surface. Recent studies demonstrated that EVs may also carry molecules from cytoplasm or even from nucleus of their parental cells, and these EVs may carry autoantigens and are important in vasculitis. EVs may play important roles in vasculitis through their potential pathogenic involvements in inflammation, autoimmune responses, procoagulation, endothelial dysfunction/damage, angiogenesis, and intimal hyperplasia. EVs have also been used as specific biomarkers for diagnostic use or disease severity monitoring. In this review, we have focused on the aspects of EV biology most relevant to the pathogenesis of vasculitis, discussed their perspective insights, and summarized the exist literature on EV relevant studies in vasculitis, therefore provides an integration of current knowledge regarding the novel role of EVs in systemic vasculitis.


Assuntos
Doenças Autoimunes/patologia , Vesículas Extracelulares/patologia , Vasculite Sistêmica/patologia , Humanos
20.
Biomed Res Int ; 2019: 5381453, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30906775

RESUMO

Objective: To determine mortality and predictive factors for lower intestinal perforation (LIP) among patients with autoimmune rheumatic diseases. Methods: This retrospective, single-center, observational study analyzed mortality rates in 31 autoimmune rheumatic disease patients with LIP who were admitted to our hospital from January 2002 to June 2017. The primary outcome was the mortality rate during hospitalization. Results: The median age at the time of LIP was 61 years, and the survival rate at discharge was 64.5%. Eleven patients died of sepsis during hospitalization. Cox univariable analysis for mortality during hospitalization showed that absence of abdominal pain (hazard ratio (HR) 5.61, 95% confidence interval (CI) 1.38-22.9), higher age (HR 1.06, 95% CI 1.01-1.11), chronic kidney disease (HR 6.89, 95% CI 1.85-25.7), systemic vasculitis (HR 3.95, 95% CI 1.14-13.6), higher blood urea nitrogen (HR 1.02, 95% CI 1.01-1.04), higher serum creatinine (HR 1.41, 95% CI 1.06-1.87), and LIP due to malignancy (HR 14.3, 95% CI 1.95-105.1) significantly increased mortality. Conclusion: Abdominal pain was absent in 16% of LIP patients with autoimmune rheumatic diseases, and this absence was a poor prognostic factor in this cohort. Moreover, higher age, chronic kidney disease, systemic vasculitis, and LIP due to malignancy were associated with significantly increased mortality. Physicians should be aware of LIP in autoimmune disease patients with higher age, chronic kidney diseases, or systemic vasculitis even if patients reveal mild abdominal symptoms.


Assuntos
Dor Abdominal/mortalidade , Perfuração Intestinal/mortalidade , Insuficiência Renal Crônica/mortalidade , Doenças Reumáticas/mortalidade , Vasculite Sistêmica/mortalidade , Dor Abdominal/etiologia , Idoso , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Taxa de Sobrevida
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