A glomerulonefrite pauci-imune (GN) é uma das causas mais frequentes de GN rapidamente progressiva (RPGN). Caracteriza-se clinicamente por manifestações renais de GNRP (hematúria, hipertensão) levando à insuficiência renal em dias ou semanas, podendo estar associada a manifestações de vasculite sistêmica (artralgia, febre, convulsões, mononeurite e envolvimento pulmonar). A GN pauci-imune é histologicamente caracterizada por GN necrosante focal e crescente, com coloração glomerular leve ou ausente para imunoglobulina e complemento por microscopia de fluorescência, que pode se manifestar como parte de uma vasculite sistêmica de pequenos vasos (incluindo poliangiite microscópica, granulomatose com poliangiite e granulomatose eosinofílica com poliangiite), ou raramente como parte de vasculite renal limitada (RLV, crescente idiopática GN). A classificação imunológica é baseada na presença ou ausência de anticorpos anticitoplasma de neutrófilos (ANCAs) circulantes, nomeadamente GN pauci-imune com ANCA e GN pauci-imune sem ANCA.
Introdução
O que você precisa saber de cara
A glomerulonefrite pauci-imune (GN) é uma das causas mais frequentes de GN rapidamente progressiva (RPGN). Caracteriza-se clinicamente por manifestações renais de GNRP (hematúria, hipertensão) levando à insuficiência renal em dias ou semanas, podendo estar associada a manifestações de vasculite sistêmica (artralgia, febre, convulsões, mononeurite e envolvimento pulmonar). A GN pauci-imune é histologicamente caracterizada por GN necrosante focal e crescente, com coloração glomerular leve ou ausente para imunoglobulina e complemento por microscopia de fluorescência, que pode se manifestar como parte de uma vasculite sistêmica de pequenos vasos (incluindo poliangiite microscópica, granulomatose com poliangiite e granulomatose eosinofílica com poliangiite), ou raramente como parte de vasculite renal limitada (RLV, crescente idiopática GN). A classificação imunológica é baseada na presença ou ausência de anticorpos anticitoplasma de neutrófilos (ANCAs) circulantes, nomeadamente GN pauci-imune com ANCA e GN pauci-imune sem ANCA.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
+ 9 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 37 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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 — Glomerulonefrite pauci-imune
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
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
ANCA positive pauci-immune glomerulonephritis as presenting feature of mantle cell lymphoma: A case report and review of literature.
Kidney involvement in mantle cell lymphoma is rare. We report a case of mantle cell lymphoma diagnosed after rapidly progressive glomerulonephritis with biopsy-proven paraneoplastic anti-neutrophil cytoplasmic antibody positive pauci-immune cresentic glomerulonephritis. Lymphoma treatment resulted in improved kidney function. This report demonstrates that pauci-immune cresentic glomerulonephritis can cause kidney impairment as the initial manifestation of mantle cell lymphoma. Physicians should be aware of the atypical presentations of the disease.
Diagnosis of Dual c- and p-ANCA Vasculitis Following SARS-CoV-2 Infection: A Case Report.
BACKGROUND Anti-neutrophil cytoplasmic antibodies (ANCAs) are associated with a group of small-vessel vasculitides collectively known as ANCA-associated vasculitis (AAV), which can lead to rapidly progressive glomerulonephritis and multisystem involvement. In addition to other autoimmune diseases, AAV has been increasingly reported following SARS-CoV-2 infection. CASE REPORT We present the case of a 70-year-old woman with a past medical history of atrial fibrillation, sick sinus syndrome, breast cancer, gastroesophageal reflux disease, and anxiety, who developed a rare phenomenon of dual ANCA-positive pauci-immune glomerulonephritis following coronavirus disease 2019 (COVID-19). She initially presented with refractory sinusitis and a persistent cough. Over 5 months, she developed iron-deficiency anemia and, eventually, a rapid decline in renal function. Serologic testing revealed dual positivity for anti-proteinase 3 (PR3, c-ANCA) and anti-myeloperoxidase (MPO, p-ANCA). Renal biopsy confirmed pauci-immune glomerulonephritis. She was ultimately diagnosed with granulomatosis with polyangiitis (GPA). CONCLUSIONS This case highlights a potential post-infectious autoimmune phenomenon triggered by SARS-CoV-2 and underscores the importance of considering AAV in patients with unexplained systemic symptoms and renal dysfunction after COVID-19. Furthermore, the mechanism of AAV seroconversion and disease progression may be linked to the ability of SARS-CoV-2 to induce the formation of neutrophil extracellular traps (NETs) through both direct viral-neutrophil interactions and cytokine-mediated inflammation. Our findings contribute to the growing body of evidence linking viral infections to the emergence of autoimmune vasculitis.
Kidney Prognosis in ANCA-associated Vasculitis: The ANCLA Risk Score From a Latin American Historical Cohort.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a leading cause of rapidly progressive glomerulonephritis and a major contributor to end-stage kidney disease (ESKD). Prognostic tools remain limited, particularly in Latin American populations. We historically analyzed 164 adults with biopsy-proven pauci-immune necrotizing and/or crescentic glomerulonephritis consistent with AAV, diagnosed between 2011 and 2024 at a tertiary referral center in Colombia. Clinical and histopathologic variables obtained near the kidney biopsy (≤30 d) were analyzed using Cox proportional hazards models. The primary outcome was ESKD, defined as initiation of chronic dialysis or sustained estimated glomerular filtration rate (eGFR) <15 mL/min/1.73 m². Independent predictors were used to derive the ANCLA (ANCA in Latin America) Risk Score. Model performance was evaluated using the area under the ROC curve (AUC), Harrell C-index, and Kaplan-Meier analysis. During a median follow-up of 12.9 months, 63 patients (38.4%) progressed to ESKD. Independent predictors included younger age, lower eGFR, higher 24-hour proteinuria, and a lower percentage of normal glomeruli. The ANCLA model demonstrated strong discrimination (AUC: 0.82, 95% CI: 0.79-0.85; C-index 0.72). Risk quartiles showed distinct kidney survival, with 2-year survival of ~95% in the lowest group versus <20% in the highest (log-rank p < 0.001). In comparative exploratory analyses, ANCLA outperformed the Berden classification and the Renal Risk Score within our cohort. The ANCLA Risk Score integrates routine clinical and histopathologic data into a simple, accurate tool for predicting kidney outcomes in AAV, including ANCA-negative cases (17.1%). Its strong performance in a Latin American cohort supports its potential for early risk stratification and clinical decision-making. External and multicenter validation are warranted.
Anti-Glomerular Basement Membrane Disease without Linear IgG Deposits in an Elderly Patient with Metastatic Rectal Cancer: A Case Report.
Anti-glomerular basement membrane (anti-GBM) disease is classically diagnosed based on presence of rapidly progressive glomerulonephritis, circulating anti-GBM antibodies, and the hallmark finding of linear IgG deposition along the glomerular basement membrane on kidney biopsy. These deposits are considered a defining feature of the disease, aiding in its differentiation from other pauci-immune glomerulonephritis. However, rare cases lacking IgG deposits on kidney biopsies challenge this paradigm, complicating both diagnosis and management. We report the case of an 80-year-old male with metastatic rectal cancer, who presented with acute kidney injury, hematuria, and proteinuria. Serological testing confirmed elevated anti-GBM antibody levels. Surprisingly, renal biopsy revealed crescentic glomerulonephritis without characteristic linear IgG deposition, which raised diagnostic uncertainty. Despite the absence of this classic finding, the patient's clinical course was consistent with anti-GBM disease. Given his malignancy, treatment was tailored to balance immunosuppression with oncologic and infectious considerations. The patient received a modified immunosuppressive regimen, including plasma exchange and corticosteroids, but IV cyclophosphamide was withheld due to recurrent infections. Initially, from a nephrology standpoint, his clinical course was notable for partial renal recovery, resolution of hematuria, and improvement in both creatinine and proteinuria levels. However, he ultimately passed away due to severe infectious complications. This case underscores the diagnostic challenges and therapeutic decision-making in patients who present with features suggestive of anti-GBM disease, characterized by positive anti-GBM antibodies but absent immune deposits, especially when accompanied by malignancy, prior exposure to immunosuppressive therapy, and concurrent infections.
Pauci-immune glomerulonephritis: Post-COVID kidney disease continues to reappear, a case report.
We present a female in her mid-70s with COVID-19 infection who developed worsening renal function along with systemic symptoms over months. The patient was initially monitored for long COVID and was referred to the nephrologist after she was found to have further worsening of her renal function. She underwent a renal biopsy and findings on light microscopy, immunofluorescence, and electron microscopy were consistent with crescentic glomerulonephritis, pauci-immune type with chronicity, and acute interstitial nephritis. These findings and a positive anti-neutrophilic cytoplasmic antibody immunofluorescence assay (ANCA IFA) and myeloperoxidase antibody confirmed myeloperoxidase-ANCA-positive pauci-immune glomerulonephritis. The patient was started on treatment with corticosteroids and Rituximab, resulting in improvement in her symptoms and renal function.
Publicações recentes
PR3-ANCA-Associated Vasculitis in IgGκ MGUS: A Fatal Case of Rapidly Progressive Glomerulonephritis.
[Vasculitides and anti-GBM disease].
Comparative Evaluation of Five Prognostic Scoring Systems in Pauci-Immune Necrotizing and Crescentic Glomerulonephritis.
ANCA positive pauci-immune glomerulonephritis as presenting feature of mantle cell lymphoma: A case report and review of literature.
Diagnosis of Dual c- and p-ANCA Vasculitis Following SARS-CoV-2 Infection: A Case Report.
📚 EuropePMC84 artigos no totalmostrando 196
ANCA positive pauci-immune glomerulonephritis as presenting feature of mantle cell lymphoma: A case report and review of literature.
NefrologiaDiagnosis of Dual c- and p-ANCA Vasculitis Following SARS-CoV-2 Infection: A Case Report.
The American journal of case 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 diseasesAnti-Glomerular Basement Membrane Disease without Linear IgG Deposits in an Elderly Patient with Metastatic Rectal Cancer: A Case Report.
Biomedicine hubTemporal and Demographic Trends in Biopsy-Proven Kidney Disease Diagnoses in the Southwestern United States, 1993-2022.
Kidney medicineFocal Crescentic Pauci-Immune Glomerulonephritis in Systemic Lupus Erythematosus: A Case Report.
Glomerular diseasesANCA-negative Pauci-immune Vasculitis, an Often-missed Clinical Entity: A Case Report.
The Journal of the Association of Physicians of IndiaCardiogenic shock and crescentic pauci-immune glomerulonephritis associated with systemic lupus erythematosus and ANCA-vasculitis overlap: a case report.
BMC nephrologyFactors associated with pauci-immune glomerulonephritis in patients undergoing kidney biopsy with positive anti-neutrophil cytoplasmic antibody results.
Journal of nephrologyProteinase 3-Antineutrophil Cytoplasmic Antibody (PR3-ANCA)-Associated Vasculitis: A Case Report.
CureusDrivers of hospital costs in ANCA-associated vasculitis patients with long-term follow-up-a real-world cost analysis.
Clinical kidney journalAnti-neutrophil Cytoplasmic Antibody (ANCA)-Negative Pauci-Immune Crescentic Glomerulonephritis: A Case Series From a Tertiary Care Hospital in South India.
CureusFrom Dermatology to Nephrology: Unveiling Granulomatosis With Polyangiitis.
CureusBeyond Heart Failure: A Case of Missed Anti-neutrophil Cytoplasmic Antibody (ANCA)-Associated Glomerulonephritis.
CureusInterstitial Lung Disease as a Herald of P-ANCA Vasculitis: A Case of Evolving Multisystem Disease.
CureusUnraveling Acute Kidney Injury: An Intricate Case of Sepsis and Immune-Mediated Renal Damage.
CureusPauci-immune glomerulonephritis: Post-COVID kidney disease continues to reappear, a case report.
Science progressAntineutrophil Cytoplasmic Antibody-Negative Pauci-Immune Crescentic Glomerulonephritis in Systemic Lupus Erythematosus: A Case Report and Literature Review.
CureusAntineutrophil Cytoplasmic Antibody-Negative Pauci-Immune Glomerulonephritis: Still Many Unknowns.
Kidney international reportsFocusing on Older Adults With Antineutrophil Cytoplasmic Autoantibody-Negative Pauci-Immune Glomerulonephritis.
Kidney international reportsKidney Biopsy in 613 Elderly Brazilian Patients: Clinicopathological Correlations.
Glomerular diseasesClinical Presentation and Outcomes of Antineutrophil Cytoplasmic Autoantibody-Negative Pauci-Immune Glomerulonephritis.
Kidney international reportsCurrent Understanding of the Pathogenesis of ANCA-Associated Vasculitis and Novel Treatment Options Targeting Complement Activation.
Life (Basel, Switzerland)Crescentic Glomerulonephritis Possibly Caused by COVID-19 Infection.
Journal of clinical medicineA Case of Hydralazine-Induced ANCA Vasculitis/Lupus Overlap Syndrome Presenting as Persistent Bicytopenia.
Case reports in rheumatologyOverlap Syndrome in Late-Onset Systemic Lupus Erythematosus With Lupus Nephritis and MPO-ANCA Pauci-Immune Glomerulonephritis and Tuberculosis: An Uncommon Association.
Case reports in nephrologyBartonella quintana Endocarditis and Pauci-Immune Glomerulonephritis in Patient with Known Risk Factors, USA, 2024.
Emerging infectious diseasesMicroscopic polyangiitis With Pauci-Immune Glomerulonephritis Associated With Gastrointestinal Tuberculosis.
Case reports in nephrologyEvaluating Avacopan in the Treatment of ANCA-Associated Vasculitis: Design, Development and Positioning of Therapy.
Drug design, development and therapyBeyond the Glomerulus With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
CureusAnalysis of the Sensitivity and Specificity of Histopathological Findings for Diagnosing Lupus Nephritis.
Diagnostics (Basel, Switzerland)A Case of Rapidly Progressive Glomerulonephritis Associated With Metastatic Melanoma.
CureusHaemophagocytic Lymphohistiocytosis Triggered by Cytomegalovirus Reactivation in An Immunosuppressed Patient with Pauci-Immune Glomerulonephritis.
European journal of case reports in internal medicineOutcomes of Covid-19 Vaccine-Associated Glomerular Diseases (CVAGD) - A Case Series from India.
Indian journal of nephrologyEvaluating Renal Disease in Pediatric-Onset Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Disease Course, Outcomes, and Predictors of Outcome.
Arthritis & rheumatology (Hoboken, N.J.)Rapidly Progressive Glomerulonephritis.
Advances in kidney disease and healthANCA-Negative Pauci-Immune Glomerulonephritis: A Review.
Glomerular diseasesThe clinical course of SARS-CoV-2 infection in patients with glomerular diseases and evaluation of the subsequent risk of relapse.
Frontiers in nephrologyHHV8-related Castleman disease presenting with nephrotic syndrome and pauci-immune glomerulonephritis.
Leukemia & lymphomaA Rare Case of ANCA-negative Rapidly Progressive Glomerulonephritis: A Case Report.
Journal of community hospital internal medicine perspectivesFemale With Hypertensive Emergency Later Found to Have ANCA-Associated Vasculitis: A Case Report.
CureusANCA-Negative Pauci-Immune Glomerulonephritis Associated with Bartonella Endocarditis.
Case reports in nephrologyEtiology, clinical profile, and outcomes of crescentic glomerulonephritis in children: a systematic review.
Pediatric nephrology (Berlin, Germany)Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis in a patient with Waldenstrom macroglobulinaemia.
Nephrology (Carlton, Vic.)Diagnostic accuracy of ANCA serology in ANCA-associated vasculitis with renal involvement.
Internal medicine journalDeciphering the association between biopsy-confirmed systemic small vessel vasculitis and Epstein-Barr virus-positive polymorphic B-cell lymphoproliferation.
Modern rheumatology case reportsRapidly Progressive Renal Failure in Type 2 Diabetic versus Non-diabetic Patients: Changing Trends, Natural History, and Outcome in an Indian Tertiary Care Center.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaMonocytes and Macrophages in Kidney Disease and Homeostasis.
International journal of molecular sciencesIdentification of inflammatory biomarkers in IgA nephropathy using the NanoString technology: a validation study in Caucasians.
Inflammation research : official journal of the European Histamine Research Society ... [et al.]Using an artificial intelligence tool incorporating natural language processing to identify patients with a diagnosis of ANCA-associated vasculitis in electronic health records.
Computers in biology and medicineGranulomatosis With Polyangiitis: The Complexity of Clinical Manifestations, Therapeutic Challenges, and Complications of a Severe Multisystemic Case.
CureusSubglottic Stenosis as a Presentation of Antineutrophil Cytoplasmic Antibody-Associated Glomerulonephritis.
CureusAcute interstitial nephritis caused by ANCA-associated vasculitis: a case based review.
Clinical rheumatologySARS-CoV-2 infection associated with antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (ANCA-GN): a systematic review and two case reports.
Journal of nephrology[Frequency of glomerular diseases in an analysis of 550 kidney biopsies].
Revista medica de ChileCase Report of MPO+ ANCA Vasculitis with Pauci-immune GN Associated with Invasive Ductal Carcinoma of the Breast.
Current rheumatology reviewsUnmasking Renal Disease in Systemic Lupus Erythematosus: Beyond Lupus Nephritis.
CureusNew-onset severe eosinophilic granulomatosis with polyangiitis following the third dose of mRNA COVID-19 vaccine: A case report.
Modern rheumatology case reportsIgA Nephropathy - Beyond the Renal Cortex.
Indian journal of nephrologyDiagnostic dilemma: drug-induced vasculitis versus systemic vasculitis.
BMJ case reportsUnraveling the Puzzle: A Case Report Questioning the Causal Relationship Between Subarachnoid Hemorrhage and Microscopic Polyangiitis.
CureusANCA Associated Glomerulonephritis Following SARS-CoV-2 Vaccination: A Case Series and Systematic Review.
VaccinesRapidly Progressive Glomerulonephritis: A COVID-19 Case Report.
Cureus'Granulomatosis with polyangiitis after Pfizer vaccination': a case report.
Therapeutic advances in rare diseaseIt Is Not Pneumocystis jiroveci (PCP), It Is Cyclophosphamide-Induced Pneumonitis.
Cureus[A Rare Cause for B Symptoms].
PraxisC-Reactive Protein Levels Are Associated with Complement C4 Deposits and Interstitial Arteritis in ANCA-Associated Renal Vasculitis.
International journal of molecular sciencesRapidly Progressive Pauci-Immune Glomerulonephritis with Aberrant Fibrinoid Necrosis Associated with Atezolizumab, an Immune Check Point Inhibitor: A Case Report and Review of Literature.
Antibodies (Basel, Switzerland)A Meta-Analysis and Cohort Study of Histopathologic and Clinical Outcomes in ANCA-Negative versus -Positive Vasculitis.
Kidney360Overlapping Syndromes: Drug-Induced Vasculitis From Hydralazine With Positive Antinuclear Antibodies.
Clinical medicine insights. Case reportsThe Cleveland Clinic Kidney Biopsy Epidemiological Project.
Kidney360Role of complement activation in anti-neutrophil cytoplasmic antibody-associated glomerulonephritis.
Frontiers in medicineImplication of platelets and complement C3 as link between innate immunity and tubulointerstitial injury in renal vasculitis with MPO-ANCA seropositivity.
Frontiers in immunologyGlomerular diseases after immune checkpoint inhibitors use: What do We know so far?
Renal failureAntineutrophil Cytoplasmic Antibody (ANCA)-Associated Renal Vasculitis Following COVID-19 Vaccination: A Case Report and Literature Review.
CureusPauci-immune glomerulonephritis with recurrent hematuria and normal renal function.
Pediatrics international : official journal of the Japan Pediatric SocietyCoexistence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgA nephropathy.
Immunologic researchDupilumab-Associated Eosinophilic Granulomatosis With Polyangiitis.
CureusInfective Endocarditis-Associated Pauci-Immune Glomerulonephritis in a Patient With Cryoglobulinemia.
CureusCytomegalovirus disease in immunosuppressed patients with necrotizing pauci-immune glomerulonephritis.
Medicina clinicaPropylthiouracil-Induced Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis with Renal and Lung Involvement.
Case reports in nephrology and dialysisAntineutrophil Cytoplasmic Antibody (ANCA)-Associated Renal Vasculitis After COVID-19 Infection: A Case Report.
CureusIdentifying relevant determinants of in-hospital time to diagnosis for ANCA-associated vasculitis patients.
Rheumatology advances in practiceAntineutrophil cytoplasmic antibodies in infective endocarditis: a case report and systematic review of the literature.
Clinical rheumatologyPeritubular Capillaritis in Native Kidney Biopsies.
American journal of clinical pathologyAcute glomerulonephritis.
Lancet (London, England)ANCA-associated vasculitis and IgG4-related disease overlap syndrome: a case report and literature review.
Immunologic researchWide variation in glucocorticoid dosing in paediatric ANCA-associated vasculitis with renal disease: a paediatric vasculitis initiative study.
Clinical and experimental rheumatologySpectrum of biopsy-proven kidney diseases in northwest China: a review of 30 years of experiences.
International urology and nephrologyGranulomatosis with polyangiitis: An atypical initial presentation.
Journal of translational autoimmunityNot Lupus Nephritis but a Rare Case of Drug-Induced Pauci-Immune Glomerulonephritis.
CureusAnti-neutrophil cytoplasmic antibody seropositivity in young adults aged up to 35 years: kidney histopathological findings and patient outcomes.
The Journal of international medical researchJournal Club Review of "Avacopan for the Treatment of ANCA-Associated Vasculitis".
ACR open rheumatologyNative kidney biopsies in older adults: disease spectrum, long-term kidney and patient survival and safety.
International urology and nephrology2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
Annals of the rheumatic diseases2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis.
Annals of the rheumatic diseasesEarly Renal Recovery after the First Flare of Pauci-Immune Glomerulonephritis.
American journal of nephrologyRecurrence or reactivation of SARS-CoV-2 infection after immunosuppressive therapy in patients with ANCA-associated vasculitis and COVID-19.
Clinical nephrology. Case studiesDe-novo Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Following the mRNA-1273 (Moderna) Vaccine for COVID-19.
CureusAnti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in Kidney Transplantation.
Medicina (Kaunas, Lithuania)Pauci-immune crescentic glomerulonephritis associated with metastatic rectal carcinoma.
The journal of the Royal College of Physicians of EdinburghFulminant Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis After 10 Years of Hydralazine Use.
CureusLow Complement C3 Levels at Diagnosis of ANCA-Associated Glomerulonephritis, a Specific Subset of Patients to Target With Anti-C5aR Therapy?: In response to: Hypocomplementemia at Diagnosis of Pauci-immune Glomerulonephritis Is Associated with Advanced Histopathological Activity Index and High Probability of Treatment Resistance (Lionaki et al., Kidney International Reports, June 2021, DOI: 10.1016/j.ekir.2021.05.043).
Kidney international reportsLow Serum C3 Pauci-Immune Glomerulonephritis: High Histopathological Activity and Lower Rates of Response to Standard Therapies.
Kidney international reportsSomething Out of Nothing: A Rare Case of Pulmonary Renal Syndrome With Pauci-Immune Glomerulonephritis and Diffuse Alveolar Hemorrhage With Negative Serologies.
CureusMethimazole-Induced ANCA Vasculitis: A Case Report.
Diagnostics (Basel, Switzerland)Hypocomplementemia at Diagnosis of Pauci-immune Glomerulonephritis Is Associated With Advanced Histopathological Activity Index and High Probability of Treatment Resistance.
Kidney international reportsSpectrum of biopsy-based renal disease in an elderly Turkish population.
Clinical nephrologyKidney transplantation outcomes in patients with IgA nephropathy and other glomerular and non-glomerular primary diseases in the new era of immunosuppression.
PloS oneAntineutrophil Cytoplasmic Autoantibody-Associated Vasculitis with Kidney Involvement in a Patient with AL Amyloidosis.
Case reports in nephrology and dialysisAcute glomerulonephritis in a hematopoietic blood stem cell donor.
Clinical nephrology. Case studiesA cohort study to investigate sex-specific differences in ANCA-associated glomerulonephritis outcomes.
Scientific reportsDoes withdrawal of immunosuppression in rheumatoid arthritis after SARS-CoV-2 infection increase the risk of vasculitis?
BMJ case reportsGlomerular Immune Deposition in MPO-ANCA Associated Glomerulonephritis Is Associated With Poor Renal Survival.
Frontiers in immunologyTwo faces of lupus nephritis? Answers.
Pediatric nephrology (Berlin, Germany)Rare renal pathologic manifestation of antineutrophil cytoplasmic antibodies associated vasculitis as suppurative interstitial nephritis: A case report and review of the literature.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaAn Unusual Presentation of Pauci-Immune Necrotizing Glomerulonephritis.
CureusA Systematic Review of Immune Checkpoint Inhibitor-Associated Glomerular Disease.
Kidney international reportsCocaine Consumption and Antineutrophil Cytoplasmic Antibody-associated Glomerulonephritis: A Case Report.
The American journal of forensic medicine and pathologyIncidence of glomerulonephritis in the western part of Switzerland over the last decade.
Swiss medical weeklyIncidence and Risk Factors of Venous Thromboembolic Events in Patients with ANCA-Glomerulonephritis: A Cohort Study from the Maine-Anjou Registry.
Journal of clinical medicineIntense immunostaining of heat shock protein 70 within renal interstitium associates with long-term renal survival in an ANCA-associated vasculitis cohort.
Cell stress & chaperonesHydralazine-Induced Dual Antineutrophil Cytoplasmic Antibody Positive Vasculitis and Nephritis.
CureusHydralazine-induced antineutrophil cytoplasmic antibody-associated vasculitis with pulmonary-renal syndrome: a case report.
Journal of medical case reportsSurveillance of a Renal Mass in a Patient With ANCA-Negative Pauci-immune Glomerulonephritis: A Case Report.
UrologyUnique case of ANCA-negative pauci-immune necrotizing glomerulonephritis with diffuse alveolar hemorrhage, potentially associated with midostaurin.
CEN case reportsSuccessful Treatment With Multitarget Therapy of Mycophenolate Mofetil and Tacrolimus for Cyclophosphamide-Resistant Antineutrophil Cytoplasmic Antibody-Associated Pauci-Immune Glomerulonephritis Developed Independently of Systemic Lupus Erythematosus.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesA Case of Granulomatosis with Polyangiitis: Consequences of Delayed Diagnosis in a Life-threatening Malady.
CureusAcute Respiratory Distress Syndrome Requiring Extracorporeal Membrane Oxygenation as the Initial Presentation of Anti-neutrophillic Cytoplasmic Auto-antibody Positive Vasculitis.
CureusAssociation between neutrophil-lymphocyte & platelet lymphocyte ratios with prognosis & mortality in rapidly progressive glomerulonephritis.
The Indian journal of medical researchThe role of mammalian target of rapamycin pathway in the pathogenesis of pauci-immune glomerulonephritis.
Renal failureSensitivity and Specificity of Pathologic Findings to Diagnose Lupus Nephritis.
Clinical journal of the American Society of Nephrology : CJASNDe novo pauci-immune glomerulonephritis in renal allografts.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncANCA vasculitis presenting with acute interstitial nephritis without glomerular involvement.
Clinical nephrology. Case studiesRenal Vasculitis and Pauci-immune Glomerulonephritis Associated With Immune Checkpoint Inhibitors.
American journal of kidney diseases : the official journal of the National Kidney FoundationPrognostic Value of Microscopic Hematuria after Induction of Remission in Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis.
American journal of nephrologyEosinophilic granulomatosis with polyangiitis in a continuous flow left ventricular assist device patient: a case report and review of literature.
Cardiovascular pathology : the official journal of the Society for Cardiovascular PathologyNephrotoxicity of immune checkpoint inhibitors beyond tubulointerstitial nephritis: single-center experience.
Journal for immunotherapy of cancerA rare case of Alport syndrome, atypical hemolytic uremic syndrome and Pauci-immune crescentic glomerulonephritis.
BMC nephrologyBacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis .
Clinical nephrologyHistopathological Classification-A Prognostic Tool for Rapidly Progressive Glomerulonephritis.
Medicina (Kaunas, Lithuania)Case report: de novo ANCA-associated vasculitis after kidney transplantation treated with rituximab and plasma exchange.
BMC nephrologyA novel glucocorticoid-free maintenance regimen for anti-neutrophil cytoplasm antibody-associated vasculitis.
Rheumatology (Oxford, England)Methimazole-Induced Pauci-Immune Glomerulonephritis and Anti-Phospholipid Syndrome: An Important Association to Be Aware of.
Journal of clinical medicine researchComplement your knowledge with a rare cause of pauci-immune glomerulonephritis.
Clinical rheumatologyPauci-immune Glomerulonephritis in Systemic Lupus Erythematosus (SLE).
CureusAnca-associated crescentic glomerulonephritis in a child with isolated renal involvement.
Jornal brasileiro de nefrologiaParaneoplastic Seronegative Pauci-Immune Glomerulonephritis Associated with Lung Adenocarcinoma Responds to Rituximab: A Case Report.
Case reports in oncologyPrognostic Factors in Anti-Neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis with Severe Glomerular Sclerosis: A National Registry-Based Cohort Study.
Pathology research internationalPulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis.
MedicinePatient Outcomes in Renal-Limited Antineutrophil Cytoplasmic Antibody Vasculitis With Inactive Histology.
Kidney international reportsImpact of Proteinase 3 versus Myeloperoxidase Positivity on Risk of End-Stage Renal Disease in ANCA-Associated Glomerulonephritis Stratified by Histological Classification: A Population-Based Cohort Study.
Disease markersHydralazine-associated adverse events: a report of two cases of hydralazine-induced ANCA vasculitis.
Jornal brasileiro de nefrologiaHydralazine-associated vasculitis: Overlapping features of drug-induced lupus and vasculitis.
Seminars in arthritis and rheumatismManagement of Elderly Patients with Rapidly Progressive Glomerulonephritis.
Blood purificationCancer in ANCA-Associated Glomerulonephritis: A Registry-Based Cohort Study.
International journal of nephrology[Renal manifestation in ANCA-associated vasculitis].
Deutsche medizinische Wochenschrift (1946)Two clinical cases of granulomatosis with polyangiitis with isolated otitis media and mastoiditis.
ReumatologiaCiliary metaplasia in a patient with antineutrophil cytoplasmic autoantibody-associated pauci-immune glomerulonephritis.
The Korean journal of internal medicineClinicopathological Characteristics and Outcomes of Diffuse Crescentic Glomerulonephritis - A Single Center Experience from Southern India.
Journal of clinical and diagnostic research : JCDRA case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance?
Clinical nephrology. Case studiesANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma.
Clinical nephrology. Case studiesAntineutrophil cytoplasmic antibody-positive pauci-immune glomerulonephritis associated with mantle cell lymphoma.
Clinical nephrology. Case studiesParaneoplastic pauci-immune glomerulonephritis in a patient with lung carcinoma.
Nefrologia : publicacion oficial de la Sociedad Espanola NefrologiaMMP2 and MMP9 associate with crescentic glomerulonephritis.
Clinical kidney journal[Extranodal NK-T-cell lymphoma, nasal type in granulomatosis with polyangiitis. A case report].
Revista medica del Instituto Mexicano del Seguro SocialEarly Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
Arthritis & rheumatology (Hoboken, N.J.)ANCA-associated pauci-immune glomerulonephritis: always pauci-immune?
Clinical and experimental rheumatologyFavorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: authors' response to comments.
Pediatric nephrology (Berlin, Germany)Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: impact of urine screening program.
Pediatric nephrology (Berlin, Germany)Bartonella Endocarditis and Pauci-Immune Glomerulonephritis: A Case Report and Review of the Literature.
Infectious diseases in clinical practice (Baltimore, Md.)Kidney disease in the elderly: biopsy based data from 14 renal centers in Poland.
BMC nephrologySystemic vasculitis associated with vemurafenib treatment: Case report and literature review.
MedicineAntineutrophil cytoplasmic antibody-associated glomerulonephritis with immunoglobulin deposition.
Clinical and experimental nephrologyThe Prevalence and Management of Pauci-Immune Glomerulonephritis and Vasculitis in Western Countries.
Kidney diseases (Basel, Switzerland)The Prevalence and Management of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in China.
Kidney diseases (Basel, Switzerland)Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience.
Indian journal of nephrologyPauci-immune glomerulonephritis in a captive chimpanzee (Pan troglodytes), and a review of spontaneous cases in animals.
Journal of medical primatologyBeneficial Effect of Rituximab in the Treatment of Esophageal Cancer-Associated Pauci-Immune Glomerulonephritis.
Kidney international reportsAnti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaPauci Immune crescentic glomerulonephritis in a patient with T-cell lymphoma and argyria.
BMC nephrologyCentral nervous system vasculitis in pediatric microscopic polyangiitis.
Acta reumatologica portuguesaEpidemiology of Glomerular Disease in Southern Arizona: Review of 10-Year Renal Biopsy Data.
MedicineHydralazine-induced pauci-immune glomerulonephritis: intriguing case series with misleading diagnoses.
Journal of community hospital internal medicine perspectivesCocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis.
Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicasAnti-neutrophil cytoplasmic antibody-associated pauci-immune glomerulonephritis in a patient with chronic lymphocytic leukaemia.
Hong Kong medical journal = Xianggang yi xue za zhiCollapsing glomerulopathy in a case of anti-neutrophil cytoplasmic antibody associated vasculitis.
Indian journal of nephrologyPauci-immune glomerulonephritis: the ANCA-negative side of the coin.
International journal of rheumatic diseases[Large vessel involvement in ANCA-associated vasculitis: Report of one case].
Revista medica de ChileAnti-Neutrophil Cytoplasmic Antibody (ANCA)-Negative Small Vessel Vasculitis: A Rare Cause of Pulmonary Renal Syndrome.
Internal medicine (Tokyo, Japan)Idiopathic lung fibrosis and anti myeloperoxidase glomerulonephritis: the tree that hides the forest.
BMC pulmonary medicineAntineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis with massive intestinal bleeding.
Kidney research and clinical practiceA historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis.
Clinical rheumatologyPresence of Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase Anti-Neutrophilic Cytoplasmic Antibodies in a Case of Rapidly Progressive Glomerulonephritis.
Frontiers in medicinePauci-immune glomerulonephritis: does negativity of anti-neutrophilic cytoplasmic antibodies matters?
International journal of rheumatic diseasesANCA positive crescentic glomerulonephritis outcome in a Central East European cohort: a retrospective study.
BMC nephrologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Glomerulonefrite pauci-imune.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Glomerulonefrite pauci-imune
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
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.
- ANCA positive pauci-immune glomerulonephritis as presenting feature of mantle cell lymphoma: A case report and review of literature.
- Diagnosis of Dual c- and p-ANCA Vasculitis Following SARS-CoV-2 Infection: A Case Report.
- Kidney Prognosis in ANCA-associated Vasculitis: The ANCLA Risk Score From a Latin American Historical Cohort.Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases· 2026· PMID 41537456mais citado
- Anti-Glomerular Basement Membrane Disease without Linear IgG Deposits in an Elderly Patient with Metastatic Rectal Cancer: A Case Report.
- Pauci-immune glomerulonephritis: Post-COVID kidney disease continues to reappear, a case report.
- PR3-ANCA-Associated Vasculitis in IgGκ MGUS: A Fatal Case of Rapidly Progressive Glomerulonephritis.
- [Vasculitides and anti-GBM disease].
- Comparative Evaluation of Five Prognostic Scoring Systems in Pauci-Immune Necrotizing and Crescentic Glomerulonephritis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:93126(Orphanet)
- MONDO:0019641(MONDO)
- GARD:19175(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55788763(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
