Grupo de vasculites sistêmicas com forte associação com Anca. Os distúrbios são caracterizados por inflamação necrosante de vasos de pequeno e médio calibre, com pouco ou nenhum depósito de imunocomplexos nas paredes dos vasos.
Introdução
O que você precisa saber de cara
Grupo de vasculites sistêmicas com forte associação com Anca. Os distúrbios são caracterizados por inflamação necrosante de vasos de pequeno e médio calibre, com pouco ou nenhum depósito de imunocomplexos nas paredes dos vasos.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 60 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 140 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Triagem neonatal (Teste do Pezinho)
A triagem neonatal permite diagnóstico precoce e início imediato do tratamento.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
5 genes identificados com associação a esta condição.
Inhibitory receptor acting as a major negative regulator of T-cell responses (PubMed:11279501, PubMed:11279502, PubMed:16551244, PubMed:1714933, PubMed:18641304, PubMed:28484017). Acts as a decoy receptor: the affinity of CTLA4 for its natural B7 family ligands, CD80 and CD86, is considerably stronger than the affinity of their cognate stimulatory coreceptor CD28 (PubMed:11279501, PubMed:11279502, PubMed:16551244, PubMed:1714933, PubMed:28484017)
Cell membrane
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Binds peptides derived from antigens that access the endocytic route of antigen presenting cells (APC) and presents them on the cell surface for recognition by the CD4 T-cells. The peptide binding cleft accommodates peptides of 10-30 residues. The peptides presented by MHC class II molecules are generated mostly by degradation of proteins that access the endocytic route, where they are processed by lysosomal proteases and other hydrolases. Exogenous antigens that have been endocytosed by the APC
Cell membraneEndoplasmic reticulum membraneGolgi apparatus, trans-Golgi network membraneEndosome membraneLysosome membrane
Serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro) (PubMed:2033050, PubMed:28240246, PubMed:3198760). By cleaving and activating receptor F2RL1/PAR-2, enhances endothelial cell barrier function and thus vascular integrity during neutrophil transendothelial migration (PubMed:23202369). Plays a role in neutrophil transendothelial migration, probably when associated with CD177 (PubMed:22266279). Triggers inflammatory processes in n
Cytoplasmic granuleSecretedCell membraneMembrane raft
Acts as a negative regulator of T-cell receptor (TCR) signaling by direct dephosphorylation of the Src family kinases LCK and FYN, ITAMs of the TCRz/CD3 complex, as well as ZAP70, VAV, VCP and other key signaling molecules (PubMed:16461343, PubMed:18056643). Associates with and probably dephosphorylates CBL. Dephosphorylates LCK at its activating 'Tyr-394' residue (PubMed:21719704). Dephosphorylates ZAP70 at its activating 'Tyr-493' residue (PubMed:16461343). Dephosphorylates the immune system a
Cytoplasm
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Binds peptides derived from antigens that access the endocytic route of antigen presenting cells (APC) and presents them on the cell surface for recognition by the CD4 T-cells. The peptide binding cleft accommodates peptides of 10-30 residues. The peptides presented by MHC class II molecules are generated mostly by degradation of proteins that access the endocytic route, where they are processed by lysosomal proteases and other hydrolases. Exogenous antigens that have been endocytosed by the APC
Cell membraneEndoplasmic reticulum membraneGolgi apparatus, trans-Golgi network membraneEndosome membraneLysosome membrane
Medicamentos aprovados (FDA)
1 medicamento encontrado nos registros da FDA americana.
Variantes genéticas (ClinVar)
155 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
14 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Vasculite associada a anticorpos anti-neutrófilo citoplasmáticos (ANCA)
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
12 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
260 ensaios clínicos encontrados, 16 ativos.
Publicações mais relevantes
Antineutrophil cytoplasmic antibody-associated vasculitis with initial gastrointestinal symptoms: case series and literature review.
While antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis (AAV) is increasingly recognized, cases presenting with initial gastrointestinal symptoms remain underexplored. This study aimed to analyze the clinical characteristics of AAV patients with gastrointestinal onset. Seven AAV patients meeting ACR/EULAR criteria, who presented with gastrointestinal symptoms between January 2017 and 2024, were retrospectively identified. A literature review was conducted across multiple databases, including PubMed, Web of Science, Cochrane Library, Embase, CNKI, VIP, and Wanfang. In total, 23 patients were included in the study. Among the 23 AAV patients with gastrointestinal symptoms, 15 (65.2%) were male, with a median age of 54 years (range: 18-79). Common clinical manifestations included hematochezia (56.5%), weight loss (43.5%), and purpura (34.8%). Eight (34.8%) had superficial gastritis, and seven (30.4%) had colonic ulcers, as identified by gastrointestinal endoscopy. Laboratory findings revealed elevated D-dimer levels and anemia in most patients, with impaired renal function and a median hemoglobin level of 105 g/L. Anti-PR3 immunoglobulin G (IgG) and antimyeloperoxidase IgG antibodies were positive in 83.3 and 80% of cases, respectively. Abdominal computed tomography (CT) revealed wall thickening in 39.1% of patients, and chest CT identified interstitial lung disease in 73.9% of patients. Nine patients (39.1%) were initially misdiagnosed, with five (55.6% of those nine) misdiagnosed as having inflammatory bowel disease. Most patients responded well to glucocorticoid and immunosuppressive therapy, with 39.1% receiving a combination of glucocorticoids and cyclophosphamide. Gastrointestinal symptoms in AAV are rare, and misdiagnosis remains a concern. Early detection requires assessing gastrointestinal, pulmonary, and renal involvement.
Evaluation of the Long-Term Safety of Avacopan in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in the Real World (AvacoStar): Protocol for a Noninterventional Prospective Cohort Study.
Established treatments for granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) include the use of immunosuppressive agents for remission induction followed by maintenance therapy. However, patients continue to experience disease progression, organ damage, and adverse events related to current therapies. Avacopan, an oral selective C5a receptor antagonist, was approved by the European Commission in January 2022 for the treatment of adult patients with severe, active GPA or MPA in combination with rituximab (RTX) or cyclophosphamide (CYC). In the pivotal phase 3 ADVOCATE (Avacopan Development in Vasculitis to Obtain Corticosteroid Elimination and Therapeutic Efficacy) study, avacopan was noninferior to prednisone taper in achieving remission at week 26 and superior in sustaining remission at week 52; furthermore, a greater improvement in estimated glomerular filtration rate with avacopan was also observed at week 52. The AvacoStar study will generate data on the benefit and risk and safety profile of avacopan in patients in a real-world context, including in those where treatment may potentially continue beyond 1 year. The primary objective of AvacoStar is to evaluate the incidence of defined medical events of special interest in patients with antineutrophil cytoplasmic antibody-associated vasculitis commencing avacopan. These include liver injury, cardiac safety, serious infections, and malignancy. AvacoStar is a noninterventional, multinational, prospective postauthorization safety study. It will enroll up to 500 patients in Germany and the United Kingdom in 2 groups of approximately 250 participants each: those treated with avacopan (plus a standard of care at local investigators' discretion; usually an RTX- or CYC-based regimen) and a second cohort treated with a CYC- or RTX-based induction regimen without avacopan. Avacopan and the standard of care will be prescribed in the usual manner in accordance with the corresponding summary of product characteristics under the sole decision of the investigator. The treatment decision will fall within current established practice. Eligible participants will be aged ≥18 years with severe, active GPA or MPA as determined by the investigator at the time of commencing avacopan or non-avacopan standard-of-care induction therapy. Patients will be followed for up to 7 years. This study has been enrolling patients since September 11, 2023. The final report is expected in the second half of 2031; interim reports are planned every 24 months after the first patient first visit. The AvacoStar study will be the largest European prospective real-world evidence comparative study conducted to date that evaluates the long-term safety of avacopan in severe, active GPA or MPA. This study is expected to yield important insights on the use of avacopan in severe, active GPA or MPA in a real-world setting.
Invasive Ureaplasma infection in a pediatric patient: a case report.
Ureaplasma spp. are a rare cause of invasive disease outside of the neonatal period. Recent studies have shown that immunosuppression with B-cell depleting therapies, such as rituximab or obinutuzumab, can increase the risk of invasive infection. We present a case in which a patient receiving B-cell depleting therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis and pauci-immune nephritis was diagnosed with disseminated, invasive Ureaplasma infection. A 17-year-old female with documented B-cell depletion presented to the emergency department from the pediatric infectious disease clinic with persistent shoulder pain. Within the first month of admission, the patient developed multifocal abscesses with joint involvement and an echodensity resembling a possible vegetation, all of which were unresponsive to broad-spectrum antibiotics. A broad-spectrum polymerase chain reaction test detected Ureaplasma urealyticum in aspirated joint fluid from multiple anatomic sites. Clinical improvement occurred after initiation of combination therapy with intravenous doxycycline and levofloxacin. We describe a patient with documented iatrogenic B cell depletion presenting with a disseminated, multifocal invasive Ureaplasma infection including osteoarthritis, cellulitis, and soft tissue abscess. Following a 6-week course of levofloxacin, the patient's C-reactive protein, fever, and pain resolved without further evidence of disease. This case report highlights the importance of considering disseminated, invasive Ureaplasma spp. when evaluating B-cell depleted patients with challenging multifocal symptom complexes. In addition, this case report highlights the critical role of molecular diagnosis in identifying this fastidious pathogen.
Renal outcomes in ANCA-associated vasculitis without clinically apparent kidney involvement: a multicentre REVEAL cohort study.
Most studies on renal outcomes in ANCA-associated vasculitis (AAV) focus on GN; however, the prognosis of patients without clinically apparent kidney involvement is understudied. We aimed to characterize kidney prognosis in this overlooked subgroup. Estimated glomerular filtration rate (eGFR) trajectories by kidney Birmingham Vasculitis Activity Score (BVAS; 0 vs ≥1) were analysed. The primary outcome was incident chronic kidney disease (CKD) in the kidney BVAS = 0 group, defined as at least two eGFR values <60 ml/min/1.73 m2 obtained at least 90 days apart, accompanied by a decline ≥30% from baseline. The cohort included 129 patients with kidney BVAS = 0 and 228 with kidney BVAS ≥ 1. After remission induction, eGFR improved in kidney BVAS ≥ 1 but declined in kidney BVAS = 0; this worsening trajectory in the kidney BVAS = 0 group was observed regardless of treatment intensity. Over 3 years, incident CKD occurred in 30 patients in kidney BVAS = 0 (cumulative incidence 30.7%, 95% CI 21.6-42.6%). In Cox models, older age and higher baseline-to-1-month time-weighted average CRP (TWA-CRP) were associated with incident CKD. Baseline-to-1-month TWA-CRP predicted incident CKD (age-adjusted HR 1.21; 95% CI 1.06-1.39; P < 0.01), and TWA-CRP from 1 to 6 months ≥0.5 mg/dl at the 6-month landmark identified higher risk (HR 2.61; 95% CI 1.01-6.78; P = 0.049). In AAV without clinically apparent kidney involvement, TWA-CRP, as a surrogate of sustained systemic inflammation, was associated with incident CKD. Even with unremarkable urinalysis, eGFR monitoring is warranted.
The predictive value of baseline systemic inflammation response index and systemic immune-inflammation index for the risk of infection within 6 months following initial immunosuppressive treatment in patients with ANCA-associated vasculitis.
Patients with ANCA-associated vasculitis (AAV) face a high risk of severe infections, particularly within the first six months of immunosuppressive therapy, contributing significantly to early mortality. The systemic immune-inflammation index (SII) and systemic inflammation response index (SIRI) are integrative biomarkers reflecting inflammatory and immune status. This study aims to develop a model based on baseline assessment and early-treatment parameters to predict infection risk in AAV patients. In this retrospective cohort study, 185 AAV patients from a single center were enrolled. Clinical and laboratory data at diagnosis, along with treatment details were collected, and the systemic immune-inflammation index (SII) and systemic inflammation response index (SIRI) were calculated. The primary endpoint was the occurrence of infection within 6 months. Univariate and multivariable logistic regression were used to identify independent risk factors. Receiver operating characteristic (ROC) curve analysis and the area under the curve (AUC) were applied to assess the predictive performance. A nomogram was developed based on the multivariable model. Within 6 months, 77 patients (41.6%) developed infections, primarily respiratory (77.9%). Multivariable analysis identified five independent predictors: older age (OR = 1.040, 95% CI: 1.006-1.076, p=0.021), higher SII (OR = 1.000, 95% CI: 1.000-1.001, p=0.037), higher SIRI (OR = 1.293, 95% CI: 1.039-1.610, p=0.021), lower serum albumin (OR = 0.898, 95% CI: 0.826-0.975, p=0.011), and a higher cumulative dose of prednisone within the first month (OR = 1.002, 95% CI: 1.001-1.004, p=0.007). The combined model incorporating all five factors showed an improved area under the curve (AUC = 0.825) compared to the "clinical-only" model comprising age, albumin, and cumulative dose of prednisone within the first month (AUC = 0.741). By integrating the five independent predictors, the nomogram yields a quantitative estimate of infection risk through the summation of points assigned to each variable. Pre-treatment SII and SIRI are independent predictors of early infection in AAV patients. The developed nomogram, which integrates these indices with age, albumin and cumulative dose of prednisone within the first month, serves as an exploratory assessment tool for individualized risk.
Publicações recentes
Plasma Macrophage Migration Inhibitory Factor as a Biomarker of Thromboinflammatory Dysregulation in Anti-Neutrophil Cytoplasmic Antibody- Associated Vasculitis.
The neutrophil-to-lymphocyte ratio is associated with adverse outcomes in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.
The diagnostic pathway and time to diagnosis in ANCA-associated vasculitis: a retrospective study at a tertiary rheumatology center.
The Role of Kidney Biopsy as a Tool for Personalized Treatment Decision-Making in Patients with Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Nephritis.
📚 EuropePMC308 artigos no totalmostrando 197
Evaluation of the Long-Term Safety of Avacopan in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in the Real World (AvacoStar): Protocol for a Noninterventional Prospective Cohort Study.
JMIR research protocolsInvasive Ureaplasma infection in a pediatric patient: a case report.
ASM case reportsAntineutrophil cytoplasmic antibody-associated vasculitis with initial gastrointestinal symptoms: case series and literature review.
European journal of gastroenterology & hepatologyRenal outcomes in ANCA-associated vasculitis without clinically apparent kidney involvement: a multicentre REVEAL cohort study.
Rheumatology (Oxford, England)Successful management of renal rupture due to anti-neutrophil cytoplasmic antibody-associated vasculitis with transcatheter arterial embolisation: a case report.
Clinical and experimental rheumatologyThe predictive value of baseline systemic inflammation response index and systemic immune-inflammation index for the risk of infection within 6 months following initial immunosuppressive treatment in patients with ANCA-associated vasculitis.
Frontiers in immunologyPlasma Exchange in ANCA-Associated Vasculitis With Kidney Involvement: Differential Outcomes by Kidney Function in a Colombian Cohort.
Journal of clinical apheresisAntineutrophil cytoplasmic antibody-associated vasculitis presenting as penile ulcers.
Dermatology online journalLate post-transplant recurrence of an anti-myeloperoxydase antibody-associated vasculitis in a former double-positive patient: a case report.
Frontiers in immunologyDental infection is associated with early relapse in patients with ANCA-associated vasculitis.
RMD openMore than Glucose Elimination: Additional Benefits of SGLT2 Inhibitors in Glomerular Diseases.
DrugsThe Complement System in ANCA-Associated Vasculitis: Mechanistic Insights, Therapeutic Horizons, and Unmet Clinical Needs.
Immunity, inflammation and diseaseEosinophilic Granulomatosis with Polyangiitis Presenting with Intermittent Claudication.
JMA journalANCA testing in real-world clinical practice: diagnostic performance and predictive value in a Spanish cohort.
Rheumatology (Oxford, England)Early detection and risk stratification in autoimmune-related interstitial lung disease: a state-of-the-art review.
Respiratory researchAvacopan for severe pulmonary hemorrhage requiring extracorporeal membrane oxygenation in a patient with MPO-ANCA positive vasculitis.
CEN case reportsImmature leukocyte and plasma-induced cell death reveal subclinical immune activation in EGPA patients in remission.
Inflammation research : official journal of the European Histamine Research Society ... [et al.]Effectiveness, Tolerability, and Safety of Mepolizumab Injection Spacing in Patients With Eosinophilic Granulomatosis With Polyangiitis: A Retrospective Cohort Study.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesProfiling of Potential Postmarket Risk Tracking and Pharmacovigilance Data for Avacopan.
Clinical therapeuticsMaking use of avacopan in clinical practice.
Clinical kidney journalAntineutrophil cytoplasmic antibody-associated vasculitis complicated by subarachnoid hemorrhage: A case report and literature review.
MedicinePituitary involvement in ANCA-associated vasculitis: Case report and literature review.
MedicineImpact of ANCA specificity on risk of cardiovascular events and death in ANCA-associated vasculitis.
RMD openANCA-associated vasculitis in the United Arab Emirates: a 17-year case series with review from Gulf literature.
Annals of Saudi medicineA fatal case of ANCA-associated vasculitis resulting in multiple organ failure: Case report and literature review.
MedicineRepeat kidney biopsy reveals new-onset IgA nephropathy in a patient with ANCA-associated vasculitis.
CEN case reportsExploration of factors influencing pulmonary injury in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis and construction of a predictive model.
Frontiers in medicineDiagnosis of Dual c- and p-ANCA Vasculitis Following SARS-CoV-2 Infection: A Case Report.
The American journal of case reportsUrinary complement C3 fragment levels and their clinical relevance in MPO-ANCA-associated vasculitis.
Scientific reportsKidney Prognosis in ANCA-associated Vasculitis: The ANCLA Risk Score From a Latin American Historical Cohort.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesKidney Transplantation in Childhood-Onset ANCA-Associated Vasculitis: Long-Term Outcomes and Prognostic Factors.
Clinical journal of the American Society of Nephrology : CJASNEosinophilic Granulomatosis with Polyangiitis Presenting as Acute Abdomen: A Rare Case Report.
Journal of inflammation researchSimilar and yet not quite the same: unmasking distinct type I interferon signatures in ANCA vasculitis.
Kidney internationalEffective Management of a Rare Case of Pediatric ANCA-Associated Vasculitis With Rituximab and Mycophenolate Mofetil.
The American journal of case reportsM-CSF, inducing CD163 macrophages, is associated with severity and prognosis of glomerulonephritis in microscopic polyangiitis.
Arthritis research & therapyTwenty-Four Cases of Severe ANCA-Associated Vasculitis Treated With Plasma Exchange: A Clinical Analysis and Long-Term Follow-Up.
Journal of clinical apheresisUpdate on antineutrophil cytoplasmic antibody vasculitis.
Current opinion in nephrology and hypertensionA very rare cause of oliguric acute kidney disease: crescentic C3 glomerulopathy.
Oxford medical case reportsSerum CXCL6 as a biomarker for diagnosing and predicting progression of interstitial lung disease in ANCA-associated vasculitis.
Arthritis research & therapyManagement of antineutrophil cytoplasmic antibody vasculitis-associated orbital inflammatory disease: A systematic literature review.
Seminars in arthritis and rheumatismANCA-associated vasculitis combined with coexisting Aspergillus fumigatus and Mycobacterium avium complex infections: a case report.
BMC infectious diseasesMicroscopic polyangiitis complicated with pulmonary hepatitis B virus infection: A case report and literature review.
Infectious medicineMRI-guided muscle biopsy improves diagnostic yield in small- to medium-vessel vasculitis: a retrospective single-center study.
Rheumatology internationalA case of antineutrophil cytoplasmic antibody-associated vasculitis and sclerosing mediastinitis.
Modern rheumatology case reportsShifting Sands or ANCA-ed in Place? The Epidemiology of ANCA-Associated Vasculitis over 2 Decades at a Large Tertiary Centre.
Glomerular diseasesChallenging manifestations of ANCA-associated vasculitis treated with avacopan: two case reports.
Frontiers in immunologyCryptococcid Sweet Syndrome in the Setting of Hydralazine-Induced ANCA Vasculitis: A Case Report.
Journal of cutaneous pathologyUsefulness of DKK1 in Estimating Vasculitis Activity and End-Stage Kidney Disease in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Patients.
Yonsei medical journalSerum Metrnl as a potential biomarker for renal involvement in ANCA-associated vasculitis.
Clinical and experimental immunologyComparison of urine protein/creatinine ratio with 24-hour urine protein in ANCA-associated glomerulonephritis.
Clinical rheumatologyVenous Thromboembolism May Be Increased in the Early Phases of ANCA-Associated Vasculitis and Could Be Associated With a High Body Mass Index: TR-VaS Experience.
International journal of rheumatic diseasesHemoptysis as the initial presentation of ANCA-associated vasculitis in a patient with ulcerative colitis: a case report and literature review.
Clinical rheumatologyDual Anti-Neutrophil Cytoplasmic Antibody and Anti-Glomerular Basement Membrane Antibody-Positive Crescentic Glomerulonephritis in a Patient With Monoclonal Gammopathy and Elevated IgG4 Levels: A Case Report and Literature Review.
Nephrology (Carlton, Vic.)Rituximab versus intravenous cyclophosphamide for anti-neutrophil cytoplasmic antibody-associated vasculitis with severe renal impairment: a multicentre REVEAL cohort study.
Modern rheumatologyA 5-year retrospective study to determine the spectrum of crescentic glomerulonephritis in three tertiary hospitals in Gauteng Province, South Africa.
South African medical journal = Suid-Afrikaanse tydskrif vir geneeskundeRecurrent spontaneous renal hemorrhage in end-stage renal disease with ANCA-associated vasculitis: a case-based review.
Clinical rheumatologyRESER/NVAN: Spanish Registry of patients with ANCA- associated vasculitis: objectives and methodology.
Reumatologia clinicaPrognostic Value of Initial Hearing Severity in Otitis Media With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Retrospective Cohort Study.
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and NeurotologyDermatomyositis and microscopic polyangiitis overlap: a case-based review.
Rheumatology internationalPrediction of relapses in patients with small vessel vasculitides: a multicenter cohort study on histopathological risk patterns.
Rheumatology internationalClinical profile and outcome of patients with ANCA-associated vasculitis with special reference to vasculitis damage index: a longitudinal observational study.
Rheumatology internationalDimerization as a key feature of autoreactive IgA antibody responses.
Annals of the rheumatic diseasesDiagnosis and treatment of systemic vasculitis: Case report and literature review.
MedicineANCA-negative Pauci-immune Vasculitis, an Often-missed Clinical Entity: A Case Report.
The Journal of the Association of Physicians of IndiaDistinct predictors of peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitis: KEIO-vasculitis cohort.
Seminars in arthritis and rheumatismEpidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis in Lithuania, 2012-2021: a retrospective cohort study.
BMJ openRegulatory T cell therapy for myeloperoxidase-specific anti-neutrophil cytoplasmic antibody associated vasculitis.
Frontiers in immunologyA Real-World Disproportionality Analysis of Avacopan in Anti-Neutrophil Cytoplasmic Antibodies Associated Vasculitis: Insights From FDA Adverse Event Reporting System.
Pharmacology research & perspectivesCardiogenic shock and crescentic pauci-immune glomerulonephritis associated with systemic lupus erythematosus and ANCA-vasculitis overlap: a case report.
BMC nephrologyPhenotype, serotype, and data-driven clustering reveal complementary dimensions of heterogeneity in ANCA-associated vasculitis: a multicentre Japanese cohort (J-CANVAS).
Rheumatology internationalDrug- and vaccine induced ANCA-associated vasculitis: An overview.
Autoimmunity reviewsConcurrent Anti-PR3 Immunoassay and cANCA Indirect Immunofluorescence Testing Provide Complementary Information for Clinical Laboratory Detection of Antineutrophil Cytoplasmic Antibodies.
The journal of applied laboratory medicinePre-existing diabetes mellitus and PR3-ANCA positivity as predictors of relapse in ANCA-associated vasculitis: a retrospective cohort study.
Clinical rheumatologyAetiology of acute/subacute nephritic syndrome: results from kidney biopsy registries in Japan and Europe.
BMC nephrologyThe risk factors of anti-neutrophil cytoplasmic antibody-associated vasculitis-associated interstitial lung disease: a systematic review and meta-analysis.
Clinics (Sao Paulo, Brazil)Comparison of rituximab induction and maintenance regimens in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: PK/PD modelling of ANCA and gammaglobulin levels in real-world patients.
EBioMedicineAddressing clinical challenges in ANCA-associated vasculitis with real-world evidence.
Rheumatology (Oxford, England)Immune cell crosstalk between ANCA-associated vasculitis and IgG4-related disease: an unresolved pathogenic link.
Frontiers in immunologyA closer look to the lung involvement in ANCA-associated vasculitis: alveolar haemorrhage and interstitial lung disease.
Clinical and experimental medicineVasculitis syndromes: large vessel vasculitides and ANCA-associated vasculitides from a neuroradiologist's perspective.
Japanese journal of radiologyRisk of hospitalization with pneumonia in patients with giant cell arteritis and anti-neutrophil cytoplasmic antibody associated vasculitis.
Rheumatology (Oxford, England)Innate immune cross-talk: The central role of neutrophils and macrophages in ANCA-associated vasculitis.
Molecular biology of the cellTargeting the complement system in ANCA-associated vasculitis management.
Rheumatology (Oxford, England)[KDIGO-Update: Treatment of ANCA vasculitis].
Deutsche medizinische Wochenschrift (1946)Reporting Quality of Randomized Controlled Trials in Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis.
Kidney international reportsMicroscopic Polyangiitis With Non-fibrotic Hypersensitivity Pneumonitis Patterns on Chest Computed Tomography: A Case Report.
CureusSignificance of urinary and serum neutrophil gelatinase-associated Lipocalin in Antineutrophil cytoplasmic antibody-associated Vasculitis with glomerulonephritis.
International immunopharmacologyPost-marketing safety profile and clinical prioritization of adverse events with avacopan in ANCA-associated vasculitis: a FAERS pharmacovigilance analysis.
Renal failure[ANCA-associated vasculitides: 10 key messages].
La Revue du praticien[Towards more targeted therapies in the treatment of ANCA-associated vasculitides].
La Revue du praticien[Recent developments in the treatment of ANCA-associated vasculitides].
La Revue du praticien[Epidemiology and risk factors of ANCAassociated vasculitides].
La Revue du praticien[Special features of ANCA-associated vasculitides].
La Revue du praticienANCA-associated vasculitis and interstitial lung disease: challenges and open questions.
European journal of internal medicineA Case of Ruptured Posterior Inferior Cerebellar Artery Aneurysm Suggestive of Dissection in an Octogenarian Patient with Anti-Neutrophil Cytoplasmic Antibody-Related Vasculitis.
NMC case report journalChallenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis.
Nature reviews. RheumatologyUtility of serum cystatin C measured at diagnosis in evaluating cross-sectional activity and predicting all-cause mortality in patients with antineutrophil cytoplasmic antibody-associated vasculitis.
Clinical rheumatologyNeutrophil CD11b is a pivotal PANoptosis marker correlated with disease activity in antineutrophil cytoplasmic antibody-associated vasculitis.
Apoptosis : an international journal on programmed cell deathGlandular Manifestations of the Head and Neck in ANCA-Associated Vasculitides: Data from a National Cohort and Systematic Review.
Clinical reviews in allergy & immunology[Ear, nose and throat manifestations in ANCA-associated vasculitis].
La Revue de medecine interneFactors associated with pauci-immune glomerulonephritis in patients undergoing kidney biopsy with positive anti-neutrophil cytoplasmic antibody results.
Journal of nephrology[The 512th case: fever, cough, acute kidney injury].
Zhonghua nei ke za zhiManganese-loaded carbon nanoparticles ameliorate ANCA-associated vasculitis by inhibiting ferroptosis.
Journal of nanobiotechnologyDiabetes mellitus and cardiac disease as key predictors of rituximab-induced acute thrombocytopenia in ANCA-associated vasculitis.
Rheumatology (Oxford, England)Treatment of systemic vasculitis.
Current opinion in rheumatologyBiomarkers in ANCA associated vasculitis: clinical utility, pitfalls and their role in the outcomes assessment.
Frontiers in immunologySuccessful treatment of anti-neutrophil cytoplasmic antibody-associated inflammatory pseudotumour with rituximab: A case report.
MedicineEfficacy and safety of avacopan for treatment of patients with ANCA-associated vasculitis receiving cyclophosphamide.
RMD openMapping a path forward: addressing disease burden, pathways and solutions in ANCA-associated vasculitis.
Rheumatology (Oxford, England)Understanding the burden of ANCA-associated vasculitis.
Rheumatology (Oxford, England)Antineutrophil cytoplasmic antibody-associated vasculitis: insights into relapse risk and future management directions.
Frontiers in immunologyPrognostic impact of pulmonary renal syndrome in ANCA-associated vasculitis: A retrospective multicentre study.
La Revue de medecine internePlasma exchange combined with low-dose rituximab treatment: A case report of severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in the elderly.
MedicineANCA-associated vasculitis with crescentic glomerulonephritis and papillary thyroid carcinoma: A case report.
MedicineAvacopan in the treatment of relapsing polychondritis with myeloperoxidase-antineutrophil cytoplasmic antibody associated vasculitis: A case report.
World journal of nephrologyHospitalisation and mortality trends in ANCA-associated vasculitis in Mexico: results from a nationwide retrospective registry analysis.
BMJ openA 77-Year-Old Woman with Dysphonia and Possible Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis Potentially Induced by Hydralazine.
Rhode Island medical journal (2013)Cytotoxic T lymphocyte-associated protein 4 gene polymorphisms are associated with ANCA-associated vasculitis in the Guangxi population of China.
Frontiers in immunologyLC-MS/MS method for quantification of avacopan in human plasma from patients treated for antineutrophil cytoplasmic antibody-associated vasculitis.
Journal of pharmaceutical and biomedical analysisLong-term efficacy of rituximab versus intravenous cyclophosphamide for severe ANCA-associated vasculitis in multicenter REVEAL cohort study.
Journal of internal medicineEarly glucocorticoid withdrawal in severe ANCA-associated vasculitis treated with combination cyclophosphamide and rituximab: a single-centre case series.
Internal medicine journalRituximab for double-positive anti-GBM antibody and ANCA-associated glomerulonephritis: The first reported case in Asia and literature review.
Clinical nephrologyThe Prognosis of Granulomatosis With Polyangiitis: The Risk of Relapse and Mortality Based on Baseline Clinical Manifestations, Laboratory Findings, and Disease Severity: A Retrospective Cohort Study.
Health science reportsClinical value at baseline and follow-up of myeloperoxidase-antibodies in ANCA-associated vasculitis.
Frontiers in immunologyFactors Associated With Relapses and Performance of the French Vasculitis Study Group Relapse Score in a Cohort of Mexican Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesDouble Positive Anti-PR3 ANCA Vasculitis and Anti-GBM Vasculitis in a Pregnant Woman: Case Report.
Nephrology (Carlton, Vic.)ANCA and SLE overlap syndrome: treatment with intravenous cyclophosphamide complicated by CMV infection.
BMJ case reportsReduced-dose obinutuzumab induces remission in refractory ANCA-associated vasculitis: a report of 16 cases.
Frontiers in immunologyCorrelation between serum ANCA and cancer: a decade-long retrospective analysis of an Italian cohort.
Frontiers in immunologyA stronger type I interferon signature distinguishes ANCA-associated vasculitis phenotypes and predicts kidney prognosis.
Kidney international[How do I treat ANCA vasculitis with renal involvement].
Revue medicale de LiegeProteinase-3 as an autoantigen and the development of granulomatosis with polyangiitis.
Allergologia et immunopathologiaTeenage heartache: A 14-year-old with pleuritic chest pain.
Paediatrics & child healthTherapeutics update in immune-mediated rheumatic diseases: Rheumatoid arthritis, idiopathic inflammatory myositis and ANCA-associated vasculitis.
Clinical medicine (London, England)Case Report: Dual immunomodulatory and hematologic benefits of rituximab in refractory anemia of ANCA-associated vasculitis.
Frontiers in immunologyCancer risk across sex, region, and disease subtype in ANCA-associated vasculitis patients : A meta-analysis.
Zeitschrift fur RheumatologieEfficacy and safety of rituximab and low dosage of glucocorticoids for ANCA associated vasculitis interstitial lung disease: a proof-of-concept study.
Respiratory medicineIMPACT OF VITAMIN D METABOLISM DISORDERS ON THE DEVELOPMENT OF AUTOIMMUNE KIDNEY DISEASES: A SYSTEMATIC REVIEW.
Georgian medical newsAssociated factors to health-related quality of life in anti-neutrophil cytoplasmic antibodies-associated vasculitis patients: Data from the Almenara Vasculitis Cohort.
Reumatologia clinicaSimilarities and Differences Between Patients Diagnosed with ANCA-Associated Vasculitis Who Are Positive and Negative for ANCA: University Clinic Practice and Expertise.
Medicina (Kaunas, Lithuania)Low dose IL-2 administration prevents but fails to treat experimental autoimmune anti-myeloperoxidase glomerulonephritis.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association[Pulmonary manifestations of ANCA-associated vasculitis in imaging].
Radiologie (Heidelberg, Germany)Malignancies Presenting With ANCA Positivity: Two Case Reports and Diagnostic Considerations.
Immunity, inflammation and diseaseCurrent Overview of Multi-omics Analyses in Microscopic Polyangiitis and Granulomatosis with Polyangiitis.
Internal medicine (Tokyo, Japan)Diffuse pulmonary lesions caused by ANCA-associated vasculitis: A case report.
MedicineInvestigating potential biomarkers associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis using Mendelian randomization and transcriptomic analysis.
Arthritis research & therapyNon-resolving pneumonia revealing ANCA vasculitis.
BMJ case reportsHigh risk of bloodstream infections, diverse causal pathogens and association with haemodialysis and plasma exchange in ANCA-associated vasculitis.
Rheumatology (Oxford, England)Successful treatment of refractory tracheal stenosis complicating anti-neutrophil cytoplasm antibody-associated vasculitis with sirolimus.
ReumatismoBeyond Heart Failure: A Case of Missed Anti-neutrophil Cytoplasmic Antibody (ANCA)-Associated Glomerulonephritis.
CureusMortality Prediction in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Kidney Involvement: Validation of the DANGER Score.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesWeight gain following a diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis.
Rheumatology advances in practiceExploration of health-related quality of life, anxiety and depression in antineutrophil cytoplasmic antibody-associated vasculitis.
Rheumatology (Oxford, England)Revolutionizing Autoimmune Research: The Role of Caveolin-1.
Immunity, inflammation and diseaseAtypical Presentation of Intravascular Large B-cell Lymphoma After Treatment for Antineutrophil Cytoplasmic Antibody-associated Vasculitis.
Internal medicine (Tokyo, Japan)Cognitive impairment in systemic autoimmune and inflammatory diseases: A narrative review focused on ANCA-associated vasculitis, sarcoidosis, Sjögren's syndrome, systemic sclerosis, and Behçet's disease.
Autoimmunity reviewsClinical Burden and Costs of Anti-Neutrophil Cytoplasmic Antibody-ANCA-Associated Vasculitis: Main Findings from REDCap Registry of a University Hospital in Spain.
ClinicoEconomics and outcomes research : CEORAnti-neutrophil Cytoplasmic Antibody-Associated Vasculitis Presenting With Disseminated Streptococcus constellatus Pyogenic Infection.
CureusInfective endocarditis mimicking antineutrophil-cytoplasmic-antibody-associated vasculitis with glomerulonephritis: a case report.
Journal of medical case reportsPoints to consider in the management of ANCA-associated vasculitis.
Autoimmunity reviewsReversible dementia associated with communicating hydrocephalus secondary to spinal hypertrophic pachymeningitis in ANCA-associated vasculitis.
Modern rheumatology case reportsCirculating miRNAs as potential non-invasive biomarkers for ANCA-associated glomerulonephritis.
Frontiers in immunologyAvacopan for ANCA-associated vasculitis: Beyond the 52-week treatment course.
Clinical nephrologyNLRP3 inflammasome activity and pyroptosis are involved in CD206 + macrophage activation by MPO anti-neutrophil cytoplasmic antibodies.
Acta biochimica et biophysica SinicaChallenges and advances in the management of antineutrophil cytoplasmic antibody vasculitis in 2025.
Current opinion in nephrology and hypertension[LATE RELAPSE OF VASCULITIS: A CASE REPORT].
HarefuahCirculating miRNAs correlate with clinical evaluation of activity in ANCA-associated glomerulonephritis.
European journal of medical researchGene-Environment Interaction: Lessons From Complement-Mediated Kidney Disease.
Seminars in nephrologyRisk factors of poor outcomes in ANCA-associated vasculitis: a single-center study.
Renal failureImmunopathology of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis.
International journal of molecular sciencesClinical Insights and Therapeutic Strategies for the Treatment of Interstitial Lung Disease in Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Current Trends and Future Directions.
Journal of clinical medicineRisk of intracranial hemorrhage in anti-neutrophil cytoplasmic antibody-associated vasculitis treated with glucocorticoids: a systematic review of case reports.
Rheumatology internationalAnti-Neutrophil Cytoplasmic Antibody-Associated Central and Peripheral Nervous System Vasculitis.
Sultan Qaboos University medical journalGene therapy enhances deoxyribonuclease I treatment in antimyeloperoxidase glomerulonephritis.
JCI insightCentral nervous system involvement and mimickers in ANCA associated vasculitis.
Journal of neurologySex-specific clinical presentations and outcomes in ANCA-associated vasculitis presenting with severe kidney disease.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationIncidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study.
RMD openA case report of metastatic renal cell carcinoma and ANCA associated vasculitis.
BMC nephrologyThe plasma proteome reveals distinct signaling pathways associated with PR3-ANCA positive and MPO-ANCA positive vasculitis.
Frontiers in immunologyEpidemiological patterns and in-hospital mortality in ANCA-associated vasculitis: Insights from Spain's National Health Data (2016-2022).
Autoimmunity reviewsThe diagnostic and prognostic role of novel biomarkers in anti-neutrophil cytoplasmic antibody-associated vasculitis.
Frontiers in immunologyExpert consensus on interstitial lung disease associated with systemic autoimmune diseases. Executive summary.
Revista clinica espanolaClinical Significance of Peripheral Arterial Disease Evaluation in Patients with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Medicina (Kaunas, Lithuania)Anti-nuclear cytoplasmic antibody-associated vasculitis and kidney cancer: A mini review.
World journal of nephrologyGranulomatosis with polyangiitis presenting as isolated ear involvement: a case series and literature review.
Acta otorhinolaryngologica Italica : organo ufficiale della Societa italiana di otorinolaringologia e chirurgia cervico-faccialeANCA associated pulmonary fibrosis: vasculitis or not vasculitis.
Current opinion in pulmonary medicineImpact of NF-κB and reactive oxygen species on intracellular BAFF/APRIL expression in ANCA-associated vasculitis: focusing on the effect of resveratrol.
Frontiers in immunologyANCA-associated vasculitis after hantavirus infection: A case report.
MedicineMisdiagnosis of ANCA-Associated Vasculitis in Patients With Cocaine/Levamisole-Associated Autoimmune Syndrome and Cocaine-Induced Midline Destructive Lesions: A Case Series.
Immunity, inflammation and diseaseFabry disease as a trigger of immune-mediated glomerular disease: Clinical hypotheses and literature review.
NefrologiaNeutrophil Extracellular Traps in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Diagnostic and Clinical Significance-A Review of the Current Literature.
Journal of clinical medicineANCA-associated vasculitis in a middle-aged woman with HIV: a complex interplay and therapeutic challenges.
BMJ case reportsThe 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis.
Rheumatology (Oxford, England)Nation wide cohort study of Japanese patients with ANCA associated vasculitis using rituximab Effectiveness and safety after two years.
Modern rheumatology[Correlation between soluble CD146 and systemic vasculitis].
Zhonghua nei ke za zhiAdvances in the treatment of ANCA-associated vasculitis.
Nature reviews. RheumatologyA Retrospective Observational Study of Otitis Media with Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis.
Internal medicine (Tokyo, Japan)Associações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Antineutrophil cytoplasmic antibody-associated vasculitis with initial gastrointestinal symptoms: case series and literature review.
- Evaluation of the Long-Term Safety of Avacopan in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in the Real World (AvacoStar): Protocol for a Noninterventional Prospective Cohort Study.
- Invasive Ureaplasma infection in a pediatric patient: a case report.
- Renal outcomes in ANCA-associated vasculitis without clinically apparent kidney involvement: a multicentre REVEAL cohort study.
- The predictive value of baseline systemic inflammation response index and systemic immune-inflammation index for the risk of infection within 6 months following initial immunosuppressive treatment in patients with ANCA-associated vasculitis.
- Plasma Macrophage Migration Inhibitory Factor as a Biomarker of Thromboinflammatory Dysregulation in Anti-Neutrophil Cytoplasmic Antibody- Associated Vasculitis.
- The neutrophil-to-lymphocyte ratio is associated with adverse outcomes in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.
- Comment on: A systematic review of patient-reported outcome measures in patients with anti-neutrophil cytoplasmic antibody associated vasculitis.
- The diagnostic pathway and time to diagnosis in ANCA-associated vasculitis: a retrospective study at a tertiary rheumatology center.
- The Role of Kidney Biopsy as a Tool for Personalized Treatment Decision-Making in Patients with Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Nephritis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:156152(Orphanet)
- MONDO:0015492(MONDO)
- GARD:13011(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q17239200(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
