A Poliangeíte Microscópica (MPA) é uma doença inflamatória que afeta os vasos sanguíneos de todo o corpo (vasculite sistêmica) e que pode causar a morte dos tecidos (necrotizante). Ela atinge predominantemente os vasos sanguíneos menores, como pequenas artérias, arteríolas, capilares e vênulas, em diversos órgãos.
Introdução
O que você precisa saber de cara
A Poliangeíte Microscópica (MPA) é uma doença inflamatória que afeta os vasos sanguíneos de todo o corpo (vasculite sistêmica) e que pode causar a morte dos tecidos (necrotizante). Ela atinge predominantemente os vasos sanguíneos menores, como pequenas artérias, arteríolas, capilares e vênulas, em diversos órgãos.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 24 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 55 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
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Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.415
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.
Isolated antineutrophil cytoplasmic antibody-associated interstitial lung disease: A systematic review of an emerging condition.
To summarise the clinical characteristics, radiological and histopathological features, treatment approaches, and outcomes of isolated antineutrophil cytoplasmic antibody-associated interstitial lung disease (ANCA-ILD). A systematic literature review conducted according to the PRISMA statement. Twenty-four studies comprising 794 patients were included. Isolated ANCA-ILD predominantly affected older adults, between 50 and 70 years of age, with a slight male predominance (57%). MPO-ANCA positivity was observed in approximately 70% of patients, whereas PR3-ANCA positivity accounted for 10-15%. Fibrotic patterns predominated, with usual interstitial pneumonia (UIP) or UIP-like changes reported in 45-65% of cases, followed by NSIP in 15-30%. Glucocorticoids were prescribed in 58% of patients, immunosuppressive therapies in 31%, and antifibrotic treatment in approximately 26% of those reported in more recent cohorts. Functional decline occurred in approximately 30-50% of patients, and radiological progression in 30-45%. Acute exacerbations were reported in 19-25%, and up to 40% fulfilled criteria for progressive pulmonary fibrosis during follow-up. Progression to systemic ANCA-associated vasculitis (AAV) occurred in approximately 10-40% of cases, with timing ranging from the first year to several years later. This evolution occurred mainly toward microscopic polyangiitis (MPA), whereas granulomatosis with polyangiitis was infrequent, representing ≤10-20% of cases. Survival was generally comparable to ANCA-negative idiopathic interstitial pneumonia and AAV-ILD. Isolated ANCA-ILD is an emerging, increasingly recognised fibrotic interstitial lung disease phenotype characterised by frequent MPO-ANCA positivity and two partly independent trajectories: (1) an IPF-like fibrotic course and (2) progression to systemic AAV, most commonly MPA.
Evolving Therapeutic Strategies in ANCA-Associated Vasculitis: Current Standards and Emerging Targets for GPA and MPA.
Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are severe autoimmune disorders characterized by necrotizing small-vessel inflammation, and are classified among anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). Standard induction therapy combines glucocorticoids (GCs) with rituximab (RTX) or cyclophosphamide (CYC), with growing emphasis on GC minimization and selective use of avacopan in patients at high risk of GC toxicity. For maintenance therapy, fixed-interval RTX generally outperforms conventional oral agents and biomarker-guided re-dosing in unselected populations, yet treatment should be individualized. Persistent challenges include treatment-related toxicity, refractory manifestations, and defining safe discontinuation strategies. Expanding knowledge of AAV immunopathogenesis has driven the development of novel, mechanism-based therapies. These include agents targeting B cells and plasma cells (anti-CD38, anti-CD19, proteasome inhibition, CAR-T cells), complement components, and T-cell co-stimulation or cytokine networks (abatacept, IL-6 and JAK-inhibitors). Collectively, these advances are shifting AAV care from broad immunosuppression toward precision immunotherapy aimed at durable remission with reduced GC exposure and minimized long-term toxicity.
Cost effectiveness of avacopan for ANCA-associated vasculitis in China.
To evaluate the cost-effectiveness of avacopan plus immunosuppressants versus glucocorticoid (GC)-based regimens for adults with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) in China, and to estimate the maximum avacopan unit price consistent with willingness-to-pay (WTP) thresholds. A nine-state Markov model (active disease; three remission states; three relapse states; end-stage renal disease [ESRD]; death) was developed from the Chinese healthcare system perspective. Baseline characteristics were derived from the ADVOCATE trial. The intervention was avacopan plus cyclophosphamide (CYC) or rituximab (RTX) with reduced-dose GCs; the comparator was CYC/RTX plus standard GCs. Treatment shares followed trial allocation (35.2% CYC; 64.8% RTX). Outcomes included total costs, life-years (LYs), quality-adjusted life years (QALYs), and incremental cost-effectiveness ratios (ICERs), using a WTP threshold of one time China's 2024 GDP per capita per QALY. Uncertainty was assessed through one-way, probabilistic, and scenario analyses. Threshold price analyses back calculated the avacopan unit price at WTPs of one time, 1.2 times, and 1.5 times GDP per capita. Avacopan increased QALYs (5.81 vs. 5.26) and LYs (8.25 vs. 7.77) and increased total costs ($73,478 vs. $70,110), yielding an ICER of $6,146/QALY. At a WTP of $13,445/QALY, avacopan was cost-effective; results were robust in sensitivity and scenario analyses. The maximum cost-effective price was $11.12-$12.70 per 10 mg at WTPs of 1.0-1.5 times GDP per capita. Avacopan-based regimens are cost-effective versus GC-based therapy for GPA/MPA in China and support value-based pricing of $11.12-$12.70 per 10 mg.
Risk stratification for renal outcomes in ANCA-associated vasculitides using established scores and histopathological criteria.
Glomerulonephritis is a common, organ-threatening manifestation in ANCA-associated vasculitis (AAV). Various clinico-histological scores have been developed assessing the risk of kidney failure, including the simplified ANCA kidney risk score (AKRiS). This retrospective study included 220 patients with biopsy-confirmed kidney involvement of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) recruited from four tertiary European referral centers. We collected biometric and clinical baseline data, disease activity parameters, AKRiS classes and histomorphological features. We examined the occurrence of a composite endpoint (ie all-cause mortality or kidney failure defined as persistent estimated glomerular filtration rate [eGFR] decline <15 mL/min/1.73 m² or kidney replacement therapy after ≥6 months, respectively) within individual AKRiS and precursor risk classes. GPA and MPA patients exhibited largely comparable baseline characteristics and treatment regimens. Histological analysis revealed that MPA biopsies showed significantly more sclerosis, interstitial fibrosis and tubular atrophy (IFTA) than their GPA counterparts. Histologically, most patients fell into focal and mixed classes, which mainly transitioned into low- and medium-risk AKRiS categories. Higher risk classifications predominantly derived from crescentic and sclerotic classes. Patients categorized as medium risk in the precursor score most frequently transitioned to lower AKRiS scores. AKRiS stratification was superior to the ANCA Renal Risk Score (ARRS) in the prediction of adverse outcomes over the entire period (AUC 0.76, C-Index 0.899). The AKRiS score was found to be effective in predicting kidney failure and a strong composite kidney endpoint in a large European AAV cohort, despite slight differences in histological and event patterns between GPA and MPA under comparable treatment regimens.
Publicações recentes
Effective performance of the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for Antineutrophil-Cytoplasmic-Antibody-Associated Vasculitis in Pediatric Patients: an ARChiVe Study.
Risk factors for severe infections during induction therapy of patients with microscopic polyangiitis.
Reversible choroidal ischemia as a rare sight-threatening manifestation of microscopic polyangiitis presenting with crescentic glomerulonephritis.
[Vasculitides and anti-GBM disease].
Recurrent Pulmonary Nodules, from Pulmonary Cryptococcosis to ANCA Associated Vasculitis: A Case Report.
📚 EuropePMC862 artigos no totalmostrando 200
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 protocolsIsolated antineutrophil cytoplasmic antibody-associated interstitial lung disease: A systematic review of an emerging condition.
Autoimmunity reviewsEvolving Therapeutic Strategies in ANCA-Associated Vasculitis: Current Standards and Emerging Targets for GPA and MPA.
Clinical reviews in allergy & immunologyWhen rare matters: aortic valve destruction in myeloperoxidase antineutrophil cytoplasmic antibody-associated microscopic polyangiitis.
Polish archives of internal medicineCost effectiveness of avacopan for ANCA-associated vasculitis in China.
Clinical and experimental rheumatologyRisk stratification for renal outcomes in ANCA-associated vasculitides using established scores and histopathological criteria.
Journal of nephrologyA Rare Occurrence of Microscopic Polyangiitis and Aortitis.
The Journal of the Association of Physicians of IndiaAdvances in the treatment of antineutrophil cytoplasmic antibody associated vasculitis: current recommendations, clinical trials and real word data.
Polish archives of internal medicineEngineered chimeric myeloperoxidase (MPO) antibodies as candidate reference materials for MPO-ANCA detection.
Journal of immunological methodsPredictors of glucocorticoid-free clinical remission in patients with newly diagnosed microscopic polyangiitis and granulomatosis with polyangiitis: a retrospective cohort study using a nationwide registry in Japan (J-CANVAS).
Arthritis research & therapyEarly VV-ECMO enabling immunosuppressive therapy in severe diffuse alveolar hemorrhage due to ANCA-associated vasculitis.
Respiratory medicine case reportsAnti-Myeloperoxidase (MPO)-Positive Granulomatosis With Polyangiitis Presenting With Pulmonary and Cutaneous Vasculitic Flares in End-Stage Renal Disease: A Diagnostic and Therapeutic Challenge.
CureusClinical utility of 18F-FDG PET/CT in patients with microscopic polyangiitis and interstitial lung disease: a retrospective cohort study.
Clinical and experimental rheumatologyAKT1 but not AKT2 single nucleotide polymorphisms are associated with the risk of microscopic polyangiitis.
PeerJPulmonary manifestations of granulomatosis with polyangiitis and microscopic polyangiitis.
Seminars in arthritis and rheumatismTrends in mortality due to GPA/MPA across Europe: insights from a decade of death registrations.
Rheumatology (Oxford, England)New insights into clinical spectrum of antineutrophil cytoplasmic autoantibody associated disease.
Current opinion in nephrology and hypertensionEffectiveness and safety of rituximab for remission maintenance therapy in microscopic polyangiitis and granulomatosis with polyangiitis in Japan: A retrospective multicenter cohort study (J-CANVAS).
Modern rheumatologyAssociation Between Bronchiectasis and Serious Infections in Microscopic Polyangiitis and Granulomatosis With Polyangiitis From the J-CANVAS.
International journal of rheumatic diseasesANCA testing in real-world clinical practice: diagnostic performance and predictive value in a Spanish cohort.
Rheumatology (Oxford, England)Avacopan for severe pulmonary hemorrhage requiring extracorporeal membrane oxygenation in a patient with MPO-ANCA positive vasculitis.
CEN case reportsClinical utility of proteinase 3-antineutrophil cytoplasmic antibody at diagnosis in predicting subsequent relapse in patients with microscopic polyangiitis.
Frontiers in medicineA Red Eye in an Elderly Patient: A Case Report Highlighting the Importance of Urgent Diagnosis.
CureusComparison of Rituximab and Cyclophosphamide for Induction Therapy in Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis: A Systematic Review of Randomized Trials and Comparative Cohort Evidence.
CureusPeriglomerular granulomatosis: a rare finding in microscopic polyangiitis.
Clinical and experimental nephrologyANCA-associated vasculitis in the United Arab Emirates: a 17-year case series with review from Gulf literature.
Annals of Saudi medicineRepeat kidney biopsy reveals new-onset IgA nephropathy in a patient with ANCA-associated vasculitis.
CEN case reportsDiagnosis of Microscopic Polyangiitis by EBUS-Guided Transbronchial Mediastinal Cryobiopsy: A Case Report.
Diagnostics (Basel, Switzerland)Bronchial artery embolization for control of life-threatening endobronchial haemorrhage during cardiac surgery in a patient with microscopic polyangiitis: a case report.
BMC anesthesiologyKidney Transplantation in Childhood-Onset ANCA-Associated Vasculitis: Long-Term Outcomes and Prognostic Factors.
Clinical journal of the American Society of Nephrology : CJASNSimilar and yet not quite the same: unmasking distinct type I interferon signatures in ANCA vasculitis.
Kidney internationalPlasma Exchange as an Adjunctive Therapeutic Option for Severe and Refractory Antineutrophil Cytoplasmic Antibody-Negative Microscopic Polyangiitis and Granulomatosis with Polyangiitis.
Medicina (Kaunas, Lithuania)M-CSF, inducing CD163 macrophages, is associated with severity and prognosis of glomerulonephritis in microscopic polyangiitis.
Arthritis research & therapyRapamycin Modulates the Polarisation of CD4+ T Cells Towards TH1 Cells in Patients with Active Granulomatosis with Polyangiitis and Microscopic Polyangiitis.
Journal of clinical medicineUpdate on antineutrophil cytoplasmic antibody vasculitis.
Current opinion in nephrology and hypertensionAntineutrophil Cytoplasmic Antibody-Associated Vasculitides in Systemic Sclerosis: A Unique Clinical Overlap With Significant Implications for Treatment and Outcomes.
Arthritis & rheumatology (Hoboken, N.J.)Microscopic polyangiitis complicated with pulmonary hepatitis B virus infection: A case report and literature review.
Infectious medicineInterstitial granulomatous dermatitis secondary to microscopic polyangiitis.
JAAD case reportsMethotrexate in the Management of Laryngotracheal Stenosis: A Narrative Review.
Journal of voice : official journal of the Voice FoundationShifting Sands or ANCA-ed in Place? The Epidemiology of ANCA-Associated Vasculitis over 2 Decades at a Large Tertiary Centre.
Glomerular diseasesGranulomatosis With Polyangiitis (Wegener Granulomatosis): Then and Now.
Archives of pathology & laboratory medicineSerum Metrnl as a potential biomarker for renal involvement in ANCA-associated vasculitis.
Clinical and experimental immunologyAnti-proteinase 3 Antibody (PR3)-Anti-neutrophil Cytoplasmic Antibody (ANCA)-Positive Vasculitis: A Rare Presentation in a 90-Year-Old Female.
CureusVenous 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 diseasesTrimethoprim-sulfamethoxazole and the risk of early severe infection in elderly-onset myeloperoxidase-antineutrophil cytoplasmic antibody-associated vasculitis.
BMC nephrologyBeyond Infection and Lupus Flare: MPO-ANCA Microscopic Polyangiitis Causing Diffuse Alveolar Hemorrhage in a Patient With Systemic Lupus Erythematosus.
Clinical case reportsRecurrent 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 clinicaDermatomyositis 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 internationalMicroscopic Polyangiitis in a Nonagenarian: Diagnostic and Therapeutic Challenges in Geriatric Vasculitis.
Annals of geriatric medicine and researchLower Myeloperoxidase-ANCA Titres at Diagnosis Are Associated with End-Stage Kidney Disease Progression During Follow-Up in Rituximab-Treated Patients with Microscopic Polyangiitis.
Medicina (Kaunas, Lithuania)ANCA-negative Pauci-immune Vasculitis, an Often-missed Clinical Entity: A Case Report.
The Journal of the Association of Physicians of IndiaInitial Circulating CD138 Predicts End-Stage Kidney Disease in Patients with Microscopic Polyangiitis.
Yonsei medical journalIs Chest Imaging Alone Adequate for Diagnosing Interstitial Lung Disease in Microscopic Polyangiitis Based on the 2022 Criteria?
Yonsei medical journalSexual Function in Patients With Systemic Vasculitis: An Overlooked Aspect of Patient Care. Results From a Cross-sectional Study.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesDistinct 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 openModification of 2022 ACR/EULAR ANCA-associated vasculitis classification criteria in a Japanese cohort (J-CANVAS).
Clinical and experimental rheumatologyPhenotype, serotype, and data-driven clustering reveal complementary dimensions of heterogeneity in ANCA-associated vasculitis: a multicentre Japanese cohort (J-CANVAS).
Rheumatology internationalWhen vision loss signals vasculitis: central retinal artery occlusion leading to microscopic polyangiitis diagnosis-a case report.
Modern rheumatology case reportsMyalgia as initial presentation of microscopic polyangiitis: diagnostic utility of kidney biopsy.
Modern rheumatology case reportsPre-existing diabetes mellitus and PR3-ANCA positivity as predictors of relapse in ANCA-associated vasculitis: a retrospective cohort study.
Clinical rheumatologyImmune cell crosstalk between ANCA-associated vasculitis and IgG4-related disease: an unresolved pathogenic link.
Frontiers in immunologyProgressive Purpura in Microscopic Polyangiitis: A Case Report.
CureusAnticoagulation-free VV-ECMO for a child with intractable pulmonary hemorrhage: A case report.
PerfusionA 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 radiologyTargeting the complement system in ANCA-associated vasculitis management.
Rheumatology (Oxford, England)Microscopic Polyangiitis With Non-fibrotic Hypersensitivity Pneumonitis Patterns on Chest Computed Tomography: A Case Report.
CureusTreatment of ANCA-Associated Glomerulonephritis Complicated by Bacteremia and Vertebral Osteomyelitis: A Challenging Medical Situation.
Case reports in nephrologyThe safety profiles of avacopan on microscopic polyangiitis and granulomatosis with polyangiitis: a real-world pharmacovigilance analysis.
Frontiers in immunology[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 medicineChallenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis.
Nature reviews. RheumatologyMicroscopic Polyangiitis with Alveolar Hemorrhage Complicated by Myasthenia Gravis: A Case Report.
Internal medicine (Tokyo, Japan)Antineutrophil cytoplasmic antibody related vasculitis with a unique imaging presentation of organizing pneumonia. The key role of lung ultrasound.
Multidisciplinary respiratory medicineMass Balance and Metabolic Profiling of Avacopan, a Selective C5a Receptor 1 Antagonist, in Healthy Humans.
Current drug metabolismPeripheral Ulcerative Keratitis as an Initial Manifestation of Granulomatosis with Polyangiitis in a Pediatric Patient: A Case Report.
Ocular immunology and inflammationReal-world effectiveness of rituximab or intravenous cyclophosphamide versus non-use in patients with severe microscopic polyangiitis and granulomatosis with polyangiitis: a retrospective cohort study of J-CANVAS.
Modern rheumatologyDiabetes mellitus and cardiac disease as key predictors of rituximab-induced acute thrombocytopenia in ANCA-associated vasculitis.
Rheumatology (Oxford, England)Tongue myokymia due to microscopic polyangiitis.
BMJ case reportsEfficacy 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)Antineutrophil cytoplasmic antibody-associated vasculitis: insights into relapse risk and future management directions.
Frontiers in immunologyProteinase 3-Antineutrophil Cytoplasmic Antibody (PR3-ANCA)-Associated Vasculitis: A Case Report.
CureusPsoriasis Increases the Risk of ANCA Associated Vasculitis: Insights from A Propensity Score-Matched Study.
ImmunoTargets and therapyMedication adherence and real-world factors of first line induction treatment selection in ANCA-associated vasculitis.
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOGMicroscopic polyangiitis with isolated cardiopulmonary involvement: A case report.
MedicineLong-term efficacy of rituximab versus intravenous cyclophosphamide for severe ANCA-associated vasculitis in multicenter REVEAL cohort study.
Journal of internal medicinePulmonary nontuberculous mycobacteria disease and anti-neutrophil cytoplasmic antibody positivity: a retrospective analysis of long-term clinical outcomes including vasculitis onset.
Clinical and experimental rheumatologyClinical 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 diseasesA 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 LiegeAvacopan-associated Liver Injury in Japanese Patients with ANCA-associated Vasculitis: Clinical, Genetic, and Mechanistic Perspectives.
Internal medicine (Tokyo, Japan)A Case of Vanishing Bile Duct Syndrome Caused by a Treatment Regimen Including Avacopan for Microscopic Polyangiitis.
Internal medicine (Tokyo, Japan)Real-world outcomes of rituximab- and cyclophosphamide-based induction therapy regimens alone and in combination over 24 months in ANCA-associated vasculitis.
Clinical and experimental rheumatologyEfficacy and safety of rituximab and low dosage of glucocorticoids for ANCA associated vasculitis interstitial lung disease: a proof-of-concept study.
Respiratory medicine[Pulmonary manifestations of ANCA-associated vasculitis in imaging].
Radiologie (Heidelberg, Germany)Current 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.
MedicineBeyond 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 diseasesTreatment With Avacopan in ANCA-Associated Vasculitis With Kidney Involvement.
Kidney international reportsUnexpected adverse event caused by avacopan: a case of drug-induced hypersensitivity syndrome in microscopic polyangiitis.
Modern rheumatology case reportsEvaluation of Nailfold Capillaroscopy as a Novel Tool in the Assessment of Eosinophilic Granulomatosis with Polyangiitis.
Journal of clinical medicineNeutrophils and Platelets as Key Players in the Pathogenesis of ANCA-Associated Vasculitis and Potential Sources of Disease Activity Biomarkers.
Diagnostics (Basel, Switzerland)Detection of Irpex lacteus in the Lung in a Case with Microscopic Polyangiitis-mimicking Manifestations Positive for Myeloperoxidase-antineutrophil Cytoplasmic Antibody.
Internal medicine (Tokyo, Japan)Microscopic polyangiitis with histopathologic evolution in serial renal biopsies during treatment of idiopathic pulmonary fibrosis.
CEN case reportsLivedo racemosa as the presenting symptom of microscopic polyangiitis.
JAAD case reportsAvacopan is effective in inducing remission for MPA/GPA, regardless of changes in serum C5a levels: a single-center study in Japan.
BMC rheumatologyClinical 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 : CEOREarly progression of capillaritis manifestations and poor renal outcome after induction therapy failure in microscopic polyangiitis.
Rheumatology (Oxford, England)Reversible dementia associated with communicating hydrocephalus secondary to spinal hypertrophic pachymeningitis in ANCA-associated vasculitis.
Modern rheumatology case reportsPathologic Evidence of Granulomatosis With Polyangiitis as a Cause of Atrioventricular Block.
JACC. Case reportsAssociation Between Hearing Loss and Systemic Small-Vessel Vasculitis: Audiological Aspects Across Disease Types.
Medicina (Kaunas, Lithuania)Microscopic Polyangiitis Initially Suspected of Being Anti-synthetase Syndrome.
Internal medicine (Tokyo, Japan)ANCA-Negative Microscopic Polyangiitis With Gastrointestinal and Renal Involvement: A Diagnostic and Therapeutic Challenge.
CureusHemoptysis Revealing Microscopic Polyangiitis: A Case Report.
CureusMicroscopic Polyangiitis With a Twist: A Rare Case of CRAO and Brainstem Infarction.
Journal of neuroimmunologyImmunopathology 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 internationalMyeloperoxidase-Antibody Positivity and Progression to Microscopic Polyangiitis in Interstitial Lung Disease: A Case Series.
Respirology case reportsWunderlich Syndrome as an unusual presentation of Microscopic Polyangiitis: A Case report with Review of Literature.
ARP rheumatologyCentral nervous system involvement and mimickers in ANCA associated vasculitis.
Journal of neurologyVasculitic Neuropathy With Iron Deficiency-Related Chronic Inflammation Followed by Elevated Serum Vascular Endothelial Growth Factor: A POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome Mimic.
CureusIncidence and prevalence of ANCA-associated vasculitis in Oslo, Norway, applying different criteria-based case definitions: a population-based cohort study.
RMD openEpidemiological patterns and in-hospital mortality in ANCA-associated vasculitis: Insights from Spain's National Health Data (2016-2022).
Autoimmunity reviewsMicroscopic polyangiitis presenting with retinal frosted branch angiitis.
The Lancet. RheumatologyPulmonary and Renal Predictors of Mortality in ANCA-Associated Vasculitis: A Regional Experience from Türkiye.
BiomedicinesEmergency Surgery for Gastrointestinal Complications in Patients With Granulomatosis With Polyangiitis and Microscopic Polyangiitis.
CureusANCA-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)Safety of glucocorticoid dose reduction in microscopic polyangiitis: a multicentre REVEAL cohort study.
Modern rheumatologyNation wide cohort study of Japanese patients with ANCA associated vasculitis using rituximab Effectiveness and safety after two years.
Modern rheumatologyAging in Granulomatosis with Polyangiitis and Microscopic Polyangiitis: From Pathophysiology to Clinical Management.
Drugs & agingGranulomatosis with Polyangiitis Presenting as Pancreatic Pseudotumor and Peripancreatic Lymphadenitis: Diagnostic Challenges and Review of 55 Cases.
The American journal of case reportsClinical significance of anti-neutrophil cytoplasmic antibody in idiopathic interstitial pneumonia: a retrospective observational study.
BMC pulmonary medicineComplement factors as biomarkers in ANCA-associated vasculitis in remission.
Clinical and experimental immunologyCurrent Understanding of the Pathogenesis of ANCA-Associated Vasculitis and Novel Treatment Options Targeting Complement Activation.
Life (Basel, Switzerland)Polyangiitis overlap syndrome is a high-risk clinical phenotype for relapse: Case series from the KEIO-vasculitis cohort.
Modern rheumatologyAdjusted Global Antiphospholipid Syndrome Score Is Associated with End-Stage Kidney Disease in Patients with ANCA-Associated Vasculitis: A Single-Centre Pilot Study.
Yonsei medical journalNervous system involvement in ANCA-associated vasculitis: Single center experience from Latin America.
Seminars in arthritis and rheumatismAtypical Presentation of Microscopic Polyangiitis in an Elderly Woman.
CureusMicroscopic polyangiitis with severe anemia as the first clinical manifestation.
World journal of emergency medicineMeta-analysis revealed HLA susceptibility markers in ANCA-associated vasculitis and its clinical subtypes.
Rheumatology (Oxford, England)Overlap Syndrome of Primary Sjögren Syndrome with Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis Based on the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) Criteria.
Diagnostics (Basel, Switzerland)Performance of EMA algorithm, 2022 ACR/EULAR criteria, and EMA-ACR/EULAR algorithm in classifying pediatric ANCA-associated vasculitis: a national cohort study in China.
World journal of pediatrics : WJPRituximab in the Treatment of Subarachnoid Hemorrhage and Widespread Bleeding in Microscopic Polyangiitis : A Case Report.
Journal of Korean Neurosurgical SocietyDamage accrual and predictors of mortality in ANCA-associated vasculitis: a retrospective observational study.
Rheumatology internationalComparison of Outcomes Between Japanese Patients With Older-Onset Granulomatosis With Polyangiitis/Microscopic Polyangiitis and Younger-Onset Patients in Daily Clinical Practice: A Two-Center Retrospective Study in Japan.
International journal of rheumatic diseasesCardiovascular involvement in ANCA-associated vasculitis.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationNeutrophil single-cell analysis identifies a type II interferon-related subset for predicting relapse of autoimmune small vessel vasculitis.
Nature communicationsNecrotizing Soft Tissue Infections Caused by Morganella morganii: A Case Report and Review of the Literature.
CureusCardiac Magnetic Resonance Imaging Findings in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitides: A Systematic Review.
ACR open rheumatologyFalse Positive C-ANCA Caused by Antinuclear Antibody: a Case Report and Literature Review.
Clinical laboratorySafety of Avacopan for the Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Combined Data From Three Clinical Trials.
ACR open rheumatologyThoracic Manifestations of ANCA-associated Vasculitis: Review of the 2022 American College of Rheumatology-European Alliance of Associations of Rheumatology Classification Criteria.
Radiographics : a review publication of the Radiological Society of North America, IncUrinary Kim-1 Correlates with Interstitial Nephritis Activity in Patients with Microscopic Polyangiitis.
Current issues in molecular biologyLong-Term Clinical Landscapes of Spinal Hypertrophic Pachymeningitis With Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis.
NeurologyClinical Characteristics of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Patients Proven by Muscle Biopsy and Its Relevance to Kidney Involvement.
Nephrology (Carlton, Vic.)Current Diagnosis and Treatment of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Review Including a Comparison of Characteristics in Europe and Japan.
Journal of clinical medicineCutaneous-limited, initially strongly unilateral microscopic polyangiitis.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGOutcomes in Mexican Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Kidney Involvement.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesIdiopathic polyarteritis nodosa - does it still exist? Viewpoint 1: as our knowledge makes progress, idiopathic polyarteritis nodosa is fading away.
Rheumatology (Oxford, England)Highlights from the breakout session: optimising new treatments.
Rheumatology (Oxford, England)Interstitial lung disease, antineutrophil cystoplasmic antibodies and microscopic polyangiitis.
Rheumatology (Oxford, England)Efficacy and safety of avacopan in patients aged 65 years and older with ANCA-associated vasculitis: a post hoc analysis of data from the ADVOCATE trial.
Rheumatology (Oxford, England)In-Depth Analysis of Disease Manifestations in Antineutrophil Cytoplasmic Antibody-Associated Vasculitides Identifies Distinct Clinical Phenotypes.
ACR open rheumatologyRisk factors for poor prognosis in ANCA-associated vasculitis with interstitial lung disease: a systematic review and meta-analysis.
Clinical rheumatologyPractical Management of ANCA-Associated Vasculitis: A Clinician's Perspective.
Glomerular diseasesMortality in children and adolescents with autoimmune inflammatory rheumatic diseases admitted to the pediatric intensive care unit.
Pediatric rheumatology online journalMicroscopic polyangiitis With Pauci-Immune Glomerulonephritis Associated With Gastrointestinal Tuberculosis.
Case reports in nephrologyThe association between serum lipids at diagnosis and renal outcome in microscopic polyangiitis patients.
PeerJA Rare Case of Microscopic Polyangitis With Associated Transient Hypophysitis.
JCEM case reportsCorrespondence on 'Real-life use of the PEXIVAS reduced-dose glucocorticoid regimen in granulomatosis with polyangiitis and microscopic polyangiitis' by Nagle et al.
Annals of the rheumatic diseasesCase report: Spontaneous renal hemorrhage in anti-neutrophil cytoplasmic antibody-associated vasculitis.
Frontiers in immunologyReal-life use of the PEXIVAS reduced-dose glucocorticoid regimen in granulomatosis with polyangiitis and microscopic polyangiitis.
Annals of the rheumatic diseases[ANCA-associated vasculitides: what nephrologists need to know].
Nephrologie & therapeutiqueIdentification of novel clinical subtypes in patients with microscopic polyangiitis using cluster analysis: multicenter REVEAL cohort study.
Frontiers in immunologyANCA-associated vasculitis in patients with rheumatoid arthritis: A single-center cohort study.
Seminars in arthritis and rheumatismRisk factors for cytomegalovirus reactivation during the treatment of ANCA-associated vasculitis: A retrospective cohort study of the J-CANVAS study.
Modern rheumatologyIncidence of ANCA-associated vasculitis and polyarteritis nodosa in Norfolk, UK, from 2011 to 2020.
Rheumatology (Oxford, England)Proteinase 3 (PR3)-Positive Perinuclear Anti-neutrophil Cytoplasmic Antibodies (pANCA) Vasculitis With Concurrent Invasive Aspergillosis Infection.
CureusRetinal Vasculitis as a Rare Presentation of Microscopic Polyangiitis.
CureusPathology of Pulmonary Vascular Disease with Radiologic Correlation.
Radiologic clinics of North AmericaSTAT4 gene polymorphism may be associated with microscopic polyangiitis susceptibility in a Chinese Guangxi population: A case-control analysis based on propensity score matching.
Human immunologyTreatment With Avacopan in Patients With Respiratory Tract Manifestations of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
ACR open rheumatologyAneurysmal rupture in microscopic polyangiitis: a case-based review.
Clinical rheumatologyConcurrent Takayasu Arteritis and Microscopic Polyangiitis in a Young Female: A Complex Case of Large- and Small-Vessel Vasculitis.
CureusPresence and Propensities of Cutaneous Immunofluorescence in ANCA-Associated Vasculitis.
Journal of cutaneous pathologyClinical Profiles, Survival, and Lung Function Outcomes in ANCA-Associated Interstitial Lung Disease: An Observational Study.
Journal of clinical medicineHow We Treat ANCA-Associated Vasculitis: A Focus on the Maintenance Therapy.
Journal of clinical medicineImpact of corticosteroids on improving lung compliance in a patient with pulmonary microscopic polyangiitis.
BMJ case reportsDifferences in phenotypes, treatments, and outcomes of ANCA-associated vasculitis across Europe, Japan and the USA in 2020.
Rheumatology (Oxford, England)Reclassification of the overlap syndrome of Behçet's disease and antineutrophil cytoplasmic antibody-associated vasculitis in patients with Behçet's disease.
The Korean journal of internal medicineAssociações
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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.
- 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.
- Isolated antineutrophil cytoplasmic antibody-associated interstitial lung disease: A systematic review of an emerging condition.
- Evolving Therapeutic Strategies in ANCA-Associated Vasculitis: Current Standards and Emerging Targets for GPA and MPA.
- Cost effectiveness of avacopan for ANCA-associated vasculitis in China.
- Risk stratification for renal outcomes in ANCA-associated vasculitides using established scores and histopathological criteria.
- Effective performance of the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for Antineutrophil-Cytoplasmic-Antibody-Associated Vasculitis in Pediatric Patients: an ARChiVe Study.
- Risk factors for severe infections during induction therapy of patients with microscopic polyangiitis.
- Reversible choroidal ischemia as a rare sight-threatening manifestation of microscopic polyangiitis presenting with crescentic glomerulonephritis.
- [Vasculitides and anti-GBM disease].
- Recurrent Pulmonary Nodules, from Pulmonary Cryptococcosis to ANCA Associated Vasculitis: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:727(Orphanet)
- MONDO:0019124(MONDO)
- GARD:3652(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q1934069(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
