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Glomerulonefrite pauci-imune
ORPHA:93126CID-10 · N05.7CID-11 · MF84DOENÇA RARA

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.

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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.

Publicações científicas
303 artigos
Último publicado: 2026 Mar 27

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adolescent
+ adult, childhood, elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: N05.7
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫘
Rins
12 sintomas
🫁
Pulmão
6 sintomas
🫃
Digestivo
3 sintomas
🩸
Sangue
2 sintomas
🧬
Pele e cabelo
1 sintomas
👂
Ouvidos
1 sintomas

+ 9 sintomas em outras categorias

Características mais comuns

100%prev.
Glomerulonefrite
90%prev.
Vasculite de pequenos vasos
Muito frequente (99-80%)
90%prev.
Insuficiência renal
Muito frequente (99-80%)
90%prev.
Positividade para anticorpos antineutrófilos citoplasmáticos
Muito frequente (99-80%)
90%prev.
Taxa de filtração glomerular diminuída
Muito frequente (99-80%)
90%prev.
Concentração elevada de creatinina circulante
Muito frequente (99-80%)
37sintomas
Muito frequente (8)
Frequente (5)
Ocasional (16)
Muito raro (8)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 37 características clínicas mais associadas, ordenadas por frequência.

GlomerulonefriteGlomerulonephritis
Muito frequente100%
Vasculite de pequenos vasosSmall vessel vasculitis
Muito frequente (99-80%)90%
Insuficiência renalRenal insufficiency
Muito frequente (99-80%)90%
Positividade para anticorpos antineutrófilos citoplasmáticosCytoplasmic antineutrophil antibody positivity
Muito frequente (99-80%)90%
Taxa de filtração glomerular diminuídaDecreased glomerular filtration rate
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico303PubMed
Últimos 10 anos200publicações
Pico202230 papers
Linha do tempo
2026Hoje · 2026🧪 2019Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Nenhum gene associado encontrado

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Diagnóstico

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Tratamento e manejo

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Onde tratar no SUS

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Publicações mais relevantes

Timeline de publicações
212 papers (10 anos)
#1

ANCA positive pauci-immune glomerulonephritis as presenting feature of mantle cell lymphoma: A case report and review of literature.

Nefrologia2026 Jan

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.

#2

Diagnosis of Dual c- and p-ANCA Vasculitis Following SARS-CoV-2 Infection: A Case Report.

The American journal of case reports2026 Jan 19

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.

#3

Kidney Prognosis in ANCA-associated Vasculitis: The ANCLA Risk Score From a Latin American Historical Cohort.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases2026 Mar 01

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.

#4

Anti-Glomerular Basement Membrane Disease without Linear IgG Deposits in an Elderly Patient with Metastatic Rectal Cancer: A Case Report.

Biomedicine hub2026

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.

#5

Pauci-immune glomerulonephritis: Post-COVID kidney disease continues to reappear, a case report.

Science progress2025

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

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📚 EuropePMC84 artigos no totalmostrando 196

2026

ANCA positive pauci-immune glomerulonephritis as presenting feature of mantle cell lymphoma: A case report and review of literature.

Nefrologia
2026

Diagnosis of Dual c- and p-ANCA Vasculitis Following SARS-CoV-2 Infection: A Case Report.

The American journal of case reports
2026

Kidney Prognosis in ANCA-associated Vasculitis: The ANCLA Risk Score From a Latin American Historical Cohort.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2026

Anti-Glomerular Basement Membrane Disease without Linear IgG Deposits in an Elderly Patient with Metastatic Rectal Cancer: A Case Report.

Biomedicine hub
2025

Temporal and Demographic Trends in Biopsy-Proven Kidney Disease Diagnoses in the Southwestern United States, 1993-2022.

Kidney medicine
2025

Focal Crescentic Pauci-Immune Glomerulonephritis in Systemic Lupus Erythematosus: A Case Report.

Glomerular diseases
2025

ANCA-negative Pauci-immune Vasculitis, an Often-missed Clinical Entity: A Case Report.

The Journal of the Association of Physicians of India
2025

Cardiogenic shock and crescentic pauci-immune glomerulonephritis associated with systemic lupus erythematosus and ANCA-vasculitis overlap: a case report.

BMC nephrology
2025

Factors associated with pauci-immune glomerulonephritis in patients undergoing kidney biopsy with positive anti-neutrophil cytoplasmic antibody results.

Journal of nephrology
2025

Proteinase 3-Antineutrophil Cytoplasmic Antibody (PR3-ANCA)-Associated Vasculitis: A Case Report.

Cureus
2025

Drivers of hospital costs in ANCA-associated vasculitis patients with long-term follow-up-a real-world cost analysis.

Clinical kidney journal
2025

Anti-neutrophil Cytoplasmic Antibody (ANCA)-Negative Pauci-Immune Crescentic Glomerulonephritis: A Case Series From a Tertiary Care Hospital in South India.

Cureus
2025

From Dermatology to Nephrology: Unveiling Granulomatosis With Polyangiitis.

Cureus
2025

Beyond Heart Failure: A Case of Missed Anti-neutrophil Cytoplasmic Antibody (ANCA)-Associated Glomerulonephritis.

Cureus
2025

Interstitial Lung Disease as a Herald of P-ANCA Vasculitis: A Case of Evolving Multisystem Disease.

Cureus
2025

Unraveling Acute Kidney Injury: An Intricate Case of Sepsis and Immune-Mediated Renal Damage.

Cureus
2025

Pauci-immune glomerulonephritis: Post-COVID kidney disease continues to reappear, a case report.

Science progress
2025

Antineutrophil Cytoplasmic Antibody-Negative Pauci-Immune Crescentic Glomerulonephritis in Systemic Lupus Erythematosus: A Case Report and Literature Review.

Cureus
2025

Antineutrophil Cytoplasmic Antibody-Negative Pauci-Immune Glomerulonephritis: Still Many Unknowns.

Kidney international reports
2025

Focusing on Older Adults With Antineutrophil Cytoplasmic Autoantibody-Negative Pauci-Immune Glomerulonephritis.

Kidney international reports
2025

Kidney Biopsy in 613 Elderly Brazilian Patients: Clinicopathological Correlations.

Glomerular diseases
2025

Clinical Presentation and Outcomes of Antineutrophil Cytoplasmic Autoantibody-Negative Pauci-Immune Glomerulonephritis.

Kidney international reports
2025

Current Understanding of the Pathogenesis of ANCA-Associated Vasculitis and Novel Treatment Options Targeting Complement Activation.

Life (Basel, Switzerland)
2025

Crescentic Glomerulonephritis Possibly Caused by COVID-19 Infection.

Journal of clinical medicine
2025

A Case of Hydralazine-Induced ANCA Vasculitis/Lupus Overlap Syndrome Presenting as Persistent Bicytopenia.

Case reports in rheumatology
2025

Overlap Syndrome in Late-Onset Systemic Lupus Erythematosus With Lupus Nephritis and MPO-ANCA Pauci-Immune Glomerulonephritis and Tuberculosis: An Uncommon Association.

Case reports in nephrology
2025

Bartonella quintana Endocarditis and Pauci-Immune Glomerulonephritis in Patient with Known Risk Factors, USA, 2024.

Emerging infectious diseases
2025

Microscopic polyangiitis With Pauci-Immune Glomerulonephritis Associated With Gastrointestinal Tuberculosis.

Case reports in nephrology
2025

Evaluating Avacopan in the Treatment of ANCA-Associated Vasculitis: Design, Development and Positioning of Therapy.

Drug design, development and therapy
2024

Beyond the Glomerulus With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Cureus
2024

Analysis of the Sensitivity and Specificity of Histopathological Findings for Diagnosing Lupus Nephritis.

Diagnostics (Basel, Switzerland)
2024

A Case of Rapidly Progressive Glomerulonephritis Associated With Metastatic Melanoma.

Cureus
2024

Haemophagocytic Lymphohistiocytosis Triggered by Cytomegalovirus Reactivation in An Immunosuppressed Patient with Pauci-Immune Glomerulonephritis.

European journal of case reports in internal medicine
2024

Outcomes of Covid-19 Vaccine-Associated Glomerular Diseases (CVAGD) - A Case Series from India.

Indian journal of nephrology
2025

Evaluating Renal Disease in Pediatric-Onset Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Disease Course, Outcomes, and Predictors of Outcome.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2024

Rapidly Progressive Glomerulonephritis.

Advances in kidney disease and health
2024

ANCA-Negative Pauci-Immune Glomerulonephritis: A Review.

Glomerular diseases
2024

The clinical course of SARS-CoV-2 infection in patients with glomerular diseases and evaluation of the subsequent risk of relapse.

Frontiers in nephrology
2025

HHV8-related Castleman disease presenting with nephrotic syndrome and pauci-immune glomerulonephritis.

Leukemia &amp; lymphoma
2024

A Rare Case of ANCA-negative Rapidly Progressive Glomerulonephritis: A Case Report.

Journal of community hospital internal medicine perspectives
2024

Female With Hypertensive Emergency Later Found to Have ANCA-Associated Vasculitis: A Case Report.

Cureus
2024

ANCA-Negative Pauci-Immune Glomerulonephritis Associated with Bartonella Endocarditis.

Case reports in nephrology
2025

Etiology, clinical profile, and outcomes of crescentic glomerulonephritis in children: a systematic review.

Pediatric nephrology (Berlin, Germany)
2024

Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis in a patient with Waldenstrom macroglobulinaemia.

Nephrology (Carlton, Vic.)
2024

Diagnostic accuracy of ANCA serology in ANCA-associated vasculitis with renal involvement.

Internal medicine journal
2025

Deciphering the association between biopsy-confirmed systemic small vessel vasculitis and Epstein-Barr virus-positive polymorphic B-cell lymphoproliferation.

Modern rheumatology case reports
2023

Rapidly 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 Arabia
2024

Monocytes and Macrophages in Kidney Disease and Homeostasis.

International journal of molecular sciences
2024

Identification 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.]
2024

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 medicine
2023

Granulomatosis With Polyangiitis: The Complexity of Clinical Manifestations, Therapeutic Challenges, and Complications of a Severe Multisystemic Case.

Cureus
2023

Subglottic Stenosis as a Presentation of Antineutrophil Cytoplasmic Antibody-Associated Glomerulonephritis.

Cureus
2024

Acute interstitial nephritis caused by ANCA-associated vasculitis: a case based review.

Clinical rheumatology
2024

SARS-CoV-2 infection associated with antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (ANCA-GN): a systematic review and two case reports.

Journal of nephrology
2023

[Frequency of glomerular diseases in an analysis of 550 kidney biopsies].

Revista medica de Chile
2024

Case Report of MPO+ ANCA Vasculitis with Pauci-immune GN Associated with Invasive Ductal Carcinoma of the Breast.

Current rheumatology reviews
2023

Unmasking Renal Disease in Systemic Lupus Erythematosus: Beyond Lupus Nephritis.

Cureus
2023

New-onset severe eosinophilic granulomatosis with polyangiitis following the third dose of mRNA COVID-19 vaccine: A case report.

Modern rheumatology case reports
2023

IgA Nephropathy - Beyond the Renal Cortex.

Indian journal of nephrology
2023

Diagnostic dilemma: drug-induced vasculitis versus systemic vasculitis.

BMJ case reports
2023

Unraveling the Puzzle: A Case Report Questioning the Causal Relationship Between Subarachnoid Hemorrhage and Microscopic Polyangiitis.

Cureus
2023

ANCA Associated Glomerulonephritis Following SARS-CoV-2 Vaccination: A Case Series and Systematic Review.

Vaccines
2023

Rapidly Progressive Glomerulonephritis: A COVID-19 Case Report.

Cureus
2022

'Granulomatosis with polyangiitis after Pfizer vaccination': a case report.

Therapeutic advances in rare disease
2023

It Is Not Pneumocystis jiroveci (PCP), It Is Cyclophosphamide-Induced Pneumonitis.

Cureus
2023

[A Rare Cause for B Symptoms].

Praxis
2023

C-Reactive Protein Levels Are Associated with Complement C4 Deposits and Interstitial Arteritis in ANCA-Associated Renal Vasculitis.

International journal of molecular sciences
2023

Rapidly 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)
2023

A Meta-Analysis and Cohort Study of Histopathologic and Clinical Outcomes in ANCA-Negative versus -Positive Vasculitis.

Kidney360
2023

Overlapping Syndromes: Drug-Induced Vasculitis From Hydralazine With Positive Antinuclear Antibodies.

Clinical medicine insights. Case reports
2022

The Cleveland Clinic Kidney Biopsy Epidemiological Project.

Kidney360
2022

Role of complement activation in anti-neutrophil cytoplasmic antibody-associated glomerulonephritis.

Frontiers in medicine
2022

Implication of platelets and complement C3 as link between innate immunity and tubulointerstitial injury in renal vasculitis with MPO-ANCA seropositivity.

Frontiers in immunology
2022

Glomerular diseases after immune checkpoint inhibitors use: What do We know so far?

Renal failure
2022

Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Renal Vasculitis Following COVID-19 Vaccination: A Case Report and Literature Review.

Cureus
2022

Pauci-immune glomerulonephritis with recurrent hematuria and normal renal function.

Pediatrics international : official journal of the Japan Pediatric Society
2023

Coexistence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgA nephropathy.

Immunologic research
2022

Dupilumab-Associated Eosinophilic Granulomatosis With Polyangiitis.

Cureus
2022

Infective Endocarditis-Associated Pauci-Immune Glomerulonephritis in a Patient With Cryoglobulinemia.

Cureus
2023

Cytomegalovirus disease in immunosuppressed patients with necrotizing pauci-immune glomerulonephritis.

Medicina clinica
2022

Propylthiouracil-Induced Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis with Renal and Lung Involvement.

Case reports in nephrology and dialysis
2022

Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Renal Vasculitis After COVID-19 Infection: A Case Report.

Cureus
2022

Identifying relevant determinants of in-hospital time to diagnosis for ANCA-associated vasculitis patients.

Rheumatology advances in practice
2022

Antineutrophil cytoplasmic antibodies in infective endocarditis: a case report and systematic review of the literature.

Clinical rheumatology
2022

Peritubular Capillaritis in Native Kidney Biopsies.

American journal of clinical pathology
2022

Acute glomerulonephritis.

Lancet (London, England)
2022

ANCA-associated vasculitis and IgG4-related disease overlap syndrome: a case report and literature review.

Immunologic research
2022

Wide variation in glucocorticoid dosing in paediatric ANCA-associated vasculitis with renal disease: a paediatric vasculitis initiative study.

Clinical and experimental rheumatology
2022

Spectrum of biopsy-proven kidney diseases in northwest China: a review of 30 years of experiences.

International urology and nephrology
2022

Granulomatosis with polyangiitis: An atypical initial presentation.

Journal of translational autoimmunity
2022

Not Lupus Nephritis but a Rare Case of Drug-Induced Pauci-Immune Glomerulonephritis.

Cureus
2022

Anti-neutrophil cytoplasmic antibody seropositivity in young adults aged up to 35 years: kidney histopathological findings and patient outcomes.

The Journal of international medical research
2022

Journal Club Review of "Avacopan for the Treatment of ANCA-Associated Vasculitis".

ACR open rheumatology
2022

Native kidney biopsies in older adults: disease spectrum, long-term kidney and patient survival and safety.

International urology and nephrology
2022

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.

Annals of the rheumatic diseases
2022

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis.

Annals of the rheumatic diseases
2022

Early Renal Recovery after the First Flare of Pauci-Immune Glomerulonephritis.

American journal of nephrology
2022

Recurrence or reactivation of SARS-CoV-2 infection after immunosuppressive therapy in patients with ANCA-associated vasculitis and COVID-19.

Clinical nephrology. Case studies
2021

De-novo Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Following the mRNA-1273 (Moderna) Vaccine for COVID-19.

Cureus
2021

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in Kidney Transplantation.

Medicina (Kaunas, Lithuania)
2021

Pauci-immune crescentic glomerulonephritis associated with metastatic rectal carcinoma.

The journal of the Royal College of Physicians of Edinburgh
2021

Fulminant Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis After 10 Years of Hydralazine Use.

Cureus
2021

Low 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 reports
2021

Low Serum C3 Pauci-Immune Glomerulonephritis: High Histopathological Activity and Lower Rates of Response to Standard Therapies.

Kidney international reports
2021

Something Out of Nothing: A Rare Case of Pulmonary Renal Syndrome With Pauci-Immune Glomerulonephritis and Diffuse Alveolar Hemorrhage With Negative Serologies.

Cureus
2021

Methimazole-Induced ANCA Vasculitis: A Case Report.

Diagnostics (Basel, Switzerland)
2021

Hypocomplementemia at Diagnosis of Pauci-immune Glomerulonephritis Is Associated With Advanced Histopathological Activity Index and High Probability of Treatment Resistance.

Kidney international reports
2022

Spectrum of biopsy-based renal disease in an elderly Turkish population.

Clinical nephrology
2021

Kidney transplantation outcomes in patients with IgA nephropathy and other glomerular and non-glomerular primary diseases in the new era of immunosuppression.

PloS one
2021

Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis with Kidney Involvement in a Patient with AL Amyloidosis.

Case reports in nephrology and dialysis
2021

Acute glomerulonephritis in a hematopoietic blood stem cell donor.

Clinical nephrology. Case studies
2021

A cohort study to investigate sex-specific differences in ANCA-associated glomerulonephritis outcomes.

Scientific reports
2021

Does withdrawal of immunosuppression in rheumatoid arthritis after SARS-CoV-2 infection increase the risk of vasculitis?

BMJ case reports
2021

Glomerular Immune Deposition in MPO-ANCA Associated Glomerulonephritis Is Associated With Poor Renal Survival.

Frontiers in immunology
2021

Two faces of lupus nephritis? Answers.

Pediatric nephrology (Berlin, Germany)
2020

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 Arabia
2020

An Unusual Presentation of Pauci-Immune Necrotizing Glomerulonephritis.

Cureus
2021

A Systematic Review of Immune Checkpoint Inhibitor-Associated Glomerular Disease.

Kidney international reports
2021

Cocaine Consumption and Antineutrophil Cytoplasmic Antibody-associated Glomerulonephritis: A Case Report.

The American journal of forensic medicine and pathology
2020

Incidence of glomerulonephritis in the western part of Switzerland over the last decade.

Swiss medical weekly
2020

Incidence and Risk Factors of Venous Thromboembolic Events in Patients with ANCA-Glomerulonephritis: A Cohort Study from the Maine-Anjou Registry.

Journal of clinical medicine
2021

Intense immunostaining of heat shock protein 70 within renal interstitium associates with long-term renal survival in an ANCA-associated vasculitis cohort.

Cell stress &amp; chaperones
2020

Hydralazine-Induced Dual Antineutrophil Cytoplasmic Antibody Positive Vasculitis and Nephritis.

Cureus
2020

Hydralazine-induced antineutrophil cytoplasmic antibody-associated vasculitis with pulmonary-renal syndrome: a case report.

Journal of medical case reports
2020

Surveillance of a Renal Mass in a Patient With ANCA-Negative Pauci-immune Glomerulonephritis: A Case Report.

Urology
2020

Unique case of ANCA-negative pauci-immune necrotizing glomerulonephritis with diffuse alveolar hemorrhage, potentially associated with midostaurin.

CEN case reports
2021

Successful 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 &amp; musculoskeletal diseases
2019

A Case of Granulomatosis with Polyangiitis: Consequences of Delayed Diagnosis in a Life-threatening Malady.

Cureus
2019

Acute Respiratory Distress Syndrome Requiring Extracorporeal Membrane Oxygenation as the Initial Presentation of Anti-neutrophillic Cytoplasmic Auto-antibody Positive Vasculitis.

Cureus
2019

Association between neutrophil-lymphocyte & platelet lymphocyte ratios with prognosis & mortality in rapidly progressive glomerulonephritis.

The Indian journal of medical research
2019

The role of mammalian target of rapamycin pathway in the pathogenesis of pauci-immune glomerulonephritis.

Renal failure
2019

Sensitivity and Specificity of Pathologic Findings to Diagnose Lupus Nephritis.

Clinical journal of the American Society of Nephrology : CJASN
2020

De novo pauci-immune glomerulonephritis in renal allografts.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2019

ANCA vasculitis presenting with acute interstitial nephritis without glomerular involvement.

Clinical nephrology. Case studies
2019

Renal Vasculitis and Pauci-immune Glomerulonephritis Associated With Immune Checkpoint Inhibitors.

American journal of kidney diseases : the official journal of the National Kidney Foundation
2019

Prognostic Value of Microscopic Hematuria after Induction of Remission in Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis.

American journal of nephrology
2019

Eosinophilic 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 Pathology
2019

Nephrotoxicity of immune checkpoint inhibitors beyond tubulointerstitial nephritis: single-center experience.

Journal for immunotherapy of cancer
2018

A rare case of Alport syndrome, atypical hemolytic uremic syndrome and Pauci-immune crescentic glomerulonephritis.

BMC nephrology
2018

Bacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis
.

Clinical nephrology
2018

Histopathological Classification-A Prognostic Tool for Rapidly Progressive Glomerulonephritis.

Medicina (Kaunas, Lithuania)
2018

Case report: de novo ANCA-associated vasculitis after kidney transplantation treated with rituximab and plasma exchange.

BMC nephrology
2019

A novel glucocorticoid-free maintenance regimen for anti-neutrophil cytoplasm antibody-associated vasculitis.

Rheumatology (Oxford, England)
2018

Methimazole-Induced Pauci-Immune Glomerulonephritis and Anti-Phospholipid Syndrome: An Important Association to Be Aware of.

Journal of clinical medicine research
2018

Complement your knowledge with a rare cause of pauci-immune glomerulonephritis.

Clinical rheumatology
2018

Pauci-immune Glomerulonephritis in Systemic Lupus Erythematosus (SLE).

Cureus
2019

Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement.

Jornal brasileiro de nefrologia
2018

Paraneoplastic Seronegative Pauci-Immune Glomerulonephritis Associated with Lung Adenocarcinoma Responds to Rituximab: A Case Report.

Case reports in oncology
2018

Prognostic Factors in Anti-Neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis with Severe Glomerular Sclerosis: A National Registry-Based Cohort Study.

Pathology research international
2018

Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis.

Medicine
2018

Patient Outcomes in Renal-Limited Antineutrophil Cytoplasmic Antibody Vasculitis With Inactive Histology.

Kidney international reports
2018

Impact 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 markers
2018

Hydralazine-associated adverse events: a report of two cases of hydralazine-induced ANCA vasculitis.

Jornal brasileiro de nefrologia
2018

Hydralazine-associated vasculitis: Overlapping features of drug-induced lupus and vasculitis.

Seminars in arthritis and rheumatism
2018

Management of Elderly Patients with Rapidly Progressive Glomerulonephritis.

Blood purification
2017

Cancer in ANCA-Associated Glomerulonephritis: A Registry-Based Cohort Study.

International journal of nephrology
2018

[Renal manifestation in ANCA-associated vasculitis].

Deutsche medizinische Wochenschrift (1946)
2017

Two clinical cases of granulomatosis with polyangiitis with isolated otitis media and mastoiditis.

Reumatologia
2019

Ciliary metaplasia in a patient with antineutrophil cytoplasmic autoantibody-associated pauci-immune glomerulonephritis.

The Korean journal of internal medicine
2017

Clinicopathological Characteristics and Outcomes of Diffuse Crescentic Glomerulonephritis - A Single Center Experience from Southern India.

Journal of clinical and diagnostic research : JCDR
2017

A case report of paraproteinemia-associated pauci-immune glomerulonephritis - a new form of monoclonal gammopathy of renal significance?

Clinical nephrology. Case studies
2017

ANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma.

Clinical nephrology. Case studies
2017

Antineutrophil cytoplasmic antibody-positive pauci-immune glomerulonephritis associated with mantle cell lymphoma.

Clinical nephrology. Case studies
2017

Paraneoplastic pauci-immune glomerulonephritis in a patient with lung carcinoma.

Nefrologia : publicacion oficial de la Sociedad Espanola Nefrologia
2017

MMP2 and MMP9 associate with crescentic glomerulonephritis.

Clinical kidney journal
2017

[Extranodal NK-T-cell lymphoma, nasal type in granulomatosis with polyangiitis. A case report].

Revista medica del Instituto Mexicano del Seguro Social
2017

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2017

ANCA-associated pauci-immune glomerulonephritis: always pauci-immune?

Clinical and experimental rheumatology
2017

Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: authors' response to comments.

Pediatric nephrology (Berlin, Germany)
2017

Favorable renal outcome in Japanese children with ANCA-associated pauci-immune glomerulonephritis: impact of urine screening program.

Pediatric nephrology (Berlin, Germany)
2016

Bartonella Endocarditis and Pauci-Immune Glomerulonephritis: A Case Report and Review of the Literature.

Infectious diseases in clinical practice (Baltimore, Md.)
2016

Kidney disease in the elderly: biopsy based data from 14 renal centers in Poland.

BMC nephrology
2016

Systemic vasculitis associated with vemurafenib treatment: Case report and literature review.

Medicine
2017

Antineutrophil cytoplasmic antibody-associated glomerulonephritis with immunoglobulin deposition.

Clinical and experimental nephrology
2016

The Prevalence and Management of Pauci-Immune Glomerulonephritis and Vasculitis in Western Countries.

Kidney diseases (Basel, Switzerland)
2016

The Prevalence and Management of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in China.

Kidney diseases (Basel, Switzerland)
2016

Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience.

Indian journal of nephrology
2016

Pauci-immune glomerulonephritis in a captive chimpanzee (Pan troglodytes), and a review of spontaneous cases in animals.

Journal of medical primatology
2016

Beneficial Effect of Rituximab in the Treatment of Esophageal Cancer-Associated Pauci-Immune Glomerulonephritis.

Kidney international reports
2016

Anti-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 Arabia
2016

Pauci Immune crescentic glomerulonephritis in a patient with T-cell lymphoma and argyria.

BMC nephrology
2016

Central nervous system vasculitis in pediatric microscopic polyangiitis.

Acta reumatologica portuguesa
2016

Epidemiology of Glomerular Disease in Southern Arizona: Review of 10-Year Renal Biopsy Data.

Medicine
2016

Hydralazine-induced pauci-immune glomerulonephritis: intriguing case series with misleading diagnoses.

Journal of community hospital internal medicine perspectives
2016

Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis.

Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas
2016

Anti-neutrophil cytoplasmic antibody-associated pauci-immune glomerulonephritis in a patient with chronic lymphocytic leukaemia.

Hong Kong medical journal = Xianggang yi xue za zhi
2016

Collapsing glomerulopathy in a case of anti-neutrophil cytoplasmic antibody associated vasculitis.

Indian journal of nephrology
2016

Pauci-immune glomerulonephritis: the ANCA-negative side of the coin.

International journal of rheumatic diseases
2015

[Large vessel involvement in ANCA-associated vasculitis: Report of one case].

Revista medica de Chile
2015

Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Negative Small Vessel Vasculitis: A Rare Cause of Pulmonary Renal Syndrome.

Internal medicine (Tokyo, Japan)
2015

Idiopathic lung fibrosis and anti myeloperoxidase glomerulonephritis: the tree that hides the forest.

BMC pulmonary medicine
2015

Antineutrophil cytoplasmic antibody-negative pauci-immune glomerulonephritis with massive intestinal bleeding.

Kidney research and clinical practice
2016

A historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis.

Clinical rheumatology
2015

Presence of Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase Anti-Neutrophilic Cytoplasmic Antibodies in a Case of Rapidly Progressive Glomerulonephritis.

Frontiers in medicine
2016

Pauci-immune glomerulonephritis: does negativity of anti-neutrophilic cytoplasmic antibodies matters?

International journal of rheumatic diseases
2015

ANCA positive crescentic glomerulonephritis outcome in a Central East European cohort: a retrospective study.

BMC nephrology

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Comunidades

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Doenças relacionadas

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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.

  1. ANCA positive pauci-immune glomerulonephritis as presenting feature of mantle cell lymphoma: A case report and review of literature.
    Nefrologia· 2026· PMID 41654351mais citado
  2. Diagnosis of Dual c- and p-ANCA Vasculitis Following SARS-CoV-2 Infection: A Case Report.
    The American journal of case reports· 2026· PMID 41549496mais citado
  3. Kidney Prognosis in ANCA-associated Vasculitis: The ANCLA Risk Score From a Latin American Historical Cohort.
    Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases· 2026· PMID 41537456mais citado
  4. Anti-Glomerular Basement Membrane Disease without Linear IgG Deposits in an Elderly Patient with Metastatic Rectal Cancer: A Case Report.
    Biomedicine hub· 2026· PMID 41446802mais citado
  5. Pauci-immune glomerulonephritis: Post-COVID kidney disease continues to reappear, a case report.
    Science progress· 2025· PMID 40760815mais citado
  6. PR3-ANCA-Associated Vasculitis in IgGκ MGUS: A Fatal Case of Rapidly Progressive Glomerulonephritis.
    J Clin Med· 2026· PMID 41976855recente
  7. [Vasculitides and anti-GBM disease].
    Inn Med (Heidelb)· 2026· PMID 41927844recente
  8. Comparative Evaluation of Five Prognostic Scoring Systems in Pauci-Immune Necrotizing and Crescentic Glomerulonephritis.
    Nephron· 2026· PMID 41719207recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:93126(Orphanet)
  2. MONDO:0019641(MONDO)
  3. GARD:19175(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. 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

Glomerulonefrite pauci-imune
Compêndio · Raras BR

Glomerulonefrite pauci-imune

ORPHA:93126 · MONDO:0019641
Prevalência
Unknown
Herança
Not applicable
CID-10
N05.7 · Síndrome nefrítica não especificada - glomerulonefrite difusa em crescente
CID-11
Início
Adolescent, Adult, Childhood, Elderly
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0343196
EuropePMC
Wikidata
Papers 10a
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