Raras
Buscar doenças, sintomas, genes...
Doença renal IgG4-relacionada
ORPHA:449395CID-10 · N11.8CID-11 · 4A43.0DOENÇA RARA

Imunidade humoral é a parte da imunidade que é mediada por macromoléculas, que se encontram nos fluidos extracelulares e podem ser anticorpos, proteínas do sistema complemento ou certos péptidos antimicrobianos. A imunidade humoral é assim designada porque depende de substâncias que se encontram nos humores do corpo.

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Introdução

O que você precisa saber de cara

📋

Doença renal IgG4-relacionada é uma condição inflamatória crônica que afeta os rins, frequentemente associada a outras manifestações sistêmicas como pancreatite e linfadenite. Caracteriza-se por infiltração de plasmócitos IgG4+ nos órgãos afetados, podendo levar à insuficiência renal progressiva.

Publicações científicas
163 artigos
Último publicado: 2026 Apr

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
Adult
+ elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: N11.8
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Entender a doença

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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
🩸
Sangue
4 sintomas
🫃
Digestivo
3 sintomas
📏
Crescimento
3 sintomas
🫁
Pulmão
2 sintomas
❤️
Coração
2 sintomas

+ 30 sintomas em outras categorias

Características mais comuns

90%prev.
Aumento do nível circulante de IgG
Muito frequente (99-80%)
90%prev.
Aumento do nível circulante de IgG4
Muito frequente (99-80%)
90%prev.
Nefrite túbulo-intersticial
Muito frequente (99-80%)
55%prev.
Fator reumatoide positivo
Frequente (79-30%)
55%prev.
Depósitos de imunoglobulinas intersticiais renais
Frequente (79-30%)
55%prev.
Deficiência de complemento
Frequente (79-30%)
59sintomas
Muito frequente (3)
Frequente (13)
Ocasional (39)
Muito raro (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 59 características clínicas mais associadas, ordenadas por frequência.

Aumento do nível circulante de IgGIncreased circulating IgG level
Muito frequente (99-80%)90%
Aumento do nível circulante de IgG4Increased circulating IgG4 level
Muito frequente (99-80%)90%
Nefrite túbulo-intersticialTubulointerstitial nephritis
Muito frequente (99-80%)90%
Fator reumatoide positivoRheumatoid factor positive
Frequente (79-30%)55%
Depósitos de imunoglobulinas intersticiais renaisRenal interstitial immunoglobulin deposits
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico163PubMed
Últimos 10 anos138publicações
Pico201517 papers
Linha do tempo
2026Hoje · 2026📈 2015Ano de pico🧪 2024Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
1Fase 13
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 3 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Doença renal IgG4-relacionada

🗺️

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

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3 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

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Publicações mais relevantes

Timeline de publicações
139 papers (10 anos)
#1

The Effectiveness of Lesion Detection for Immunoglobulin G4-Related Kidney Diseases by Diffusion-Weighted Imaging.

Nephron2026

The aim of the study was to compare the efficacy of magnetic resonance imaging (MRI) with that of plain or contrast-enhanced computed tomography (CT) in the detection of renal parenchymal and pelvic lesions of immunoglobulin G4-related kidney disease (IgG4-RKD). Patients with IgG4-RKD and controls, who performed plain, contrast-enhanced CT and MRI around the kidney region in our hospital, were enrolled. The diagnosis of IgG4-RKD was made by definite cases of IgG4-RKD diagnostic criteria in 2020. Five blinded observers independently assessed image datasets by confidence scores to assess diagnostic accuracy, sensitivity, specificity, areas under the receiver operating characteristic curve (AUROC), and Cronbach's alpha coefficient. A total of 31 patients were included in the study. Fourteen (45.2%) had IgG4-RKD. Five patients with IgG4-RKD had parenchymal lesions, 5 had renal pelvic lesions, and 4 had both. In the parenchymal lesions, there was no significant difference in diagnostic performance between contrast-enhanced CT and diffusion-weighted imaging (DWI)-b800. The AUROC and sensitivity were higher in DWI-b800 than in plain CT (p < 0.05). Cronbach's alpha coefficient was 0.44 for plain CT and over 0.80 for contrast-enhanced CT and DWI-b800. In the pelvic lesions, there were fewer differences in the performance among each sequence. Cronbach's alpha coefficient was over 0.80 for plain CT, contrast-enhanced CT, and DWI-b800. Plain MRI, especially in DWI-b800, can effectively detect renal parenchymal lesions in IgG4-RKD. In cases where the use of a contrast agent of CT is difficult, DWI-b800 can be an alternative for the screening of IgG4-RKD.

#2

Urinary β2-microglobulin as a Potential Biomarker for Early Treatment Selection: A Patient with Severe IgG4-related Tubulointerstitial Nephritis.

Internal medicine (Tokyo, Japan)2026 Feb 01

This case report describes an 80-year-old man with severe immunoglobulin G4-related tubulointerstitial nephritis (IgG4-TIN), characterized by storiform fibrosis with diffuse lymphocytic and plasma cell infiltration observed on a renal biopsy. Steroid pulse therapy administered immediately after confirming a remarkable increase in urinary β2-microglobulin (100,948 μg/L) along with no evidence of malignancy significantly improved the renal function and reduced the urinary β2-microglobulin levels. This study highlights the potential utility of urinary β2-microglobulin as a biomarker for early treatment selection in severe IgG4-TIN and emphasizes the need for timely intervention to prevent irreversible kidney damage.

#3

Clinical features and outcomes of immunoglobulin G4-related kidney disease and immunoglobulin G4-related retroperitoneal fibrosis in Korea.

Kidney research and clinical practice2026 Jan

Immunoglobulin G4 (IgG4)-related disease (RD) is a newly recognized disease, and a few epidemiologic studies about this disorder have been published. This research aimed to demonstrate the clinical features and outcomes of IgG4-related kidney disease (RKD) and IgG4-related retroperitoneal fibrosis (RPF) compared to other organs' involvement. Patients who were diagnosed with IgG4-RD from January 2009 to July 2019 at three medical institutions in South Korea were included. They were classified into three groups: RKD, RPF, and Others groups. The differences in symptoms, laboratory, histological and radiological findings, treatment, and outcomes among the three groups were evaluated. Of 94 patients, 13 (13.8%) and 22 patients (23.4%) were classified into the RKD and RPF groups, respectively. There were older (p = 0.004) and more asymptomatic patients (p = 0.02) in the RKD and RPF groups. In the RKD group, hypocomplementemia (p = 0.003) and eosinophilia (p = 0.001) were more frequently identified. In logistic regression analysis, hypocomplementemia (odds ratio [OR], 14.04; 95% confidence interval [CI], 1.38-142.95) and decreased renal function at the time of diagnosis (OR, 0.95; 95% CI, 0.91-0.98) were associated with renal involvement. Older age (OR, 1.05; 95% CI, 1.00-1.11), male (OR, 6.11; 95% CI, 1.41-26.61), and higher serum IgG4 levels (OR, 1.00; 95% CI, 1.00-1.00) were associated with retroperitoneal involvement. The treatment duration was longer in the RKD and RPF groups (p = 0.01) with glucocorticoids. Renal and retroperitoneal involvement in IgG4-RD presented clinical features that distinguish it from other organs' involvement, such as incidental diagnosis, hypocomplementemia, eosinophilia, and the need for a longer duration of maintenance treatment.

#4

Ultrasonographic features of IgG4-related kidney disease: diagnostic value and correlation with clinical-pathological findings.

Frontiers in medicine2025

To systematically characterize the ultrasonographic features of biopsy-proven IgG4-related kidney disease (IgG4-RKD) and to evaluate their correlation with key clinicopathological parameters. In this retrospective study, the ultrasonographic, clinical, laboratory, and pathological data of 15 patients with biopsy-confirmed IgG4-RKD were analyzed. Key sonographic features, including renal size, parenchymal echotexture, focal lesions, and resistive index, were evaluated and correlated with clinicopathological findings. The cohort consisted of predominantly older males (86.7%) with frequent multi-organ involvement (60.0%). The most common ultrasonographic pattern included bilateral involvement (93.3%), increased or heterogeneous echogenicity (73.3%/60.0%), multiple hypoechoic areas (46.7%), and enlargement of at least one kidney (>12 cm) in 40.0% of patients. Increased renal length showed a significant positive correlation with serum IgG4 levels (r = 0.63, 95% confidence interval (CI) [0.17, 0.86], p < 0.05), and parenchymal thickness showed a significant negative correlation with serum C3 levels (r = -0.58, 95% CI [-0.84, -0.10], p < 0.05). Furthermore, parenchymal thickness was moderately correlated with serum creatinine (r = 0.51, n = 15, 95% CI [0.00, 0.81], p < 0.05). Ultrasound also effectively monitored therapeutic response, with 83.3% of followed patients showing structural improvement. Ultrasonography in IgG4-RKD reveals a characteristic pattern of findings that not only serve a descriptive role but also quantitatively correlate with serological disease activity and renal dysfunction. Therefore, ultrasound is a valuable, non-invasive tool for initial assessment, guiding diagnosis, and monitoring therapeutic response in the clinical management of IgG4-RKD.

#5

IgG4-related kidney disease: from renal histopathology and immunopathogenesis to novel pharmacological interventions.

Expert review of clinical immunology2025 Nov

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a rare and chronic fibroinflammatory condition hallmarked by tumefactive lesions that can affect nearly any organ of the body and lead to fibrotic organ destruction. Parenchymal and non-parenchymal affection of the kidney and urogenital tract are subsumed under the umbrella term IgG4-related kidney disease (IgG4-RKD), which is a severe and quite common organ manifestation in IgG4-RD. The immunopathogenesis in IgG4-RD is depicted by a complex interplay of distinct B- and T-cell subsets, excessive antibody production, a unique cytokine environment and the development of exuberant fibrosis. Scientific advancements over the last two decades have fostered to explore a broad repertoire of pharmacological interventions starting from B-cell depleting agents and extending to modulators of T-cell co-stimulation. The aim of this review is to a) provide an overview of the current knowledge on IgG4-RKD with an emphasis on the unique properties of renal histopathology and immunopathogenesis and b) overview novel pharmacological interventions targeting B cells, T cells, and beyond. Speculating on a potential scenario that dominates IgG4-RD's treatment reality in 5 years, the advent of integrative treatment strategies combining both B- and T-cell targeting agents is conceivable.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC81 artigos no totalmostrando 136

2025

Ultrasonographic features of IgG4-related kidney disease: diagnostic value and correlation with clinical-pathological findings.

Frontiers in medicine
2025

IgG4-related kidney disease: from renal histopathology and immunopathogenesis to novel pharmacological interventions.

Expert review of clinical immunology
2026

The Effectiveness of Lesion Detection for Immunoglobulin G4-Related Kidney Diseases by Diffusion-Weighted Imaging.

Nephron
2025

Concurrent IgG4-related membranous nephropathy and proliferative diabetic kidney disease: a diagnostic dilemma and therapeutic strategy in long-standing diabetes.

BMC nephrology
2026

Urinary β2-microglobulin as a Potential Biomarker for Early Treatment Selection: A Patient with Severe IgG4-related Tubulointerstitial Nephritis.

Internal medicine (Tokyo, Japan)
2025

Case Report: Two cases of IgG4-related kidney disease with monoclonal gammopathy.

Frontiers in immunology
2025

Clinicopathological findings of tubulointerstitial nephritis: a cross-sectional analysis of the Japan Renal Biopsy Registry (J-RBR).

Clinical and experimental nephrology
2025

Collapsing Focal Segmental Glomerulosclerosis With Concurrent IgG4 Nephropathy.

Cureus
2026

Clinical features and outcomes of immunoglobulin G4-related kidney disease and immunoglobulin G4-related retroperitoneal fibrosis in Korea.

Kidney research and clinical practice
2025

An Atypical Cystic Renal Mass in a Patient with IgG4-Related Kidney Disease.

Internal medicine (Tokyo, Japan)
2025

IgG4-Related Kidney Disease.

Clinical journal of the American Society of Nephrology : CJASN
2024

Coexistence of immunoglobulin G4-related kidney disease and acute hematogenous disseminated pulmonary tuberculosis: a case report.

Frontiers in immunology
2025

Progression to end-stage renal disease due to IgG4-related nephritis: a case report.

Oxford medical case reports
2025

A Challenging Case of IgG4-Related Kidney Disease Accompanied With Positive Serologic Tests of Lupus Erythematosus.

Nephrology (Carlton, Vic.)
2024

An Unusual Case of Overlapping Immunoglobulin G4-Related Disease and Systemic Lupus Erythematosus.

Cureus
2025

Diffuse large B-cell lymphoma with rapid kidney enlargement after induction of hemodialysis in a patient with IgG4-related disease.

CEN case reports
2025

A Case of IgG4-Related Disease Manifesting as Extensive Abdominal Periarteritis and Membranous Nephropathy, Successfully Controlled with Low-Dose Steroid Therapy without Relapse or Complications.

Nephron
2025

Relationship between MRI findings and renal histopathology in IgG4-related tubulointerstitial nephritis.

Modern rheumatology
2024

Clinicopathologic Features of IgG4-Related Kidney Disease.

Kidney international reports
2024

IgG4-related disease complicated by PLA2R-associated membranous nephropathy: A case report.

Open life sciences
2024

Renal Manifestations of IgG4-Related Disease: A Concise Review.

International journal of nephrology
2025

Tubulointerstitial nephritis with storiform fibrosis in a patient with angioimmunoblastic T-cell lymphoma.

CEN case reports
2024

A case of immunoglobulin G4-related kidney disease manifesting after dipeptidyl peptidase-4 inhibitor treatment.

CEN case reports
2024

Immunoglobulin G4-related disease presenting with nephrotic syndrome due to minimal change disease: a case report.

Journal of medical case reports
2024

IgG4-Related Membranous Nephropathy After COVID-19 Vaccination: A Case Report.

Cureus
2024

IgG4-Related Kidney Disease: A Diagnostic Conundrum Successfully Treated With Steroids and Rituximab.

Cureus
2024

Improved Renal Function in Initial Treatment Improves Patient Survival, Renal Outcomes, and Glucocorticoid-Related Complications in IgG4-Related Kidney Disease in Japan.

Kidney international reports
2024

IgG4-related kidney disease: Clinicopathologic features, differential diagnosis, and mimics.

Seminars in diagnostic pathology
2023

A pictorial review of genitourinary infections and inflammations.

Clinical imaging
2023

Case Report: IgG4-related kidney disease complicated by interstitial pneumonia.

F1000Research
2023

IgG4-Related Membranous Nephropathy with Acute Nephrotic Syndrome During Successful Steroid Maintenance Treatment for Type 1 Autoimmune Pancreatitis.

The American journal of case reports
2023

Clinical and Prognostic Factors in Patients with IgG4-Related Kidney Disease.

Clinical journal of the American Society of Nephrology : CJASN
2023

A case of IgG4-related interstitial nephritis with ureteral obstruction: case report and literature review.

BMC urology
2023

Progression to End-Stage Renal Disease Due to IgG4-Related Nephritis Refractory to Rituximab.

Cureus
2023

IgG4-related kidney disease complicated with retroperitoneal fibrosis: A case report.

World journal of clinical cases
2023

[When a rear-end collision turns out to be a revelation: a case of IgG4 related kidney desease].

Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia
2022

A Case of IgG4-Related Disease and Membranous Nephropathy Associated with Thrombospondin Type-1 Domain-Containing 7A.

Glomerular diseases
2023

Importance of Awareness and Careful Follow-Up of Suspected IgG4-Related Periaortitis.

International heart journal
2023

Immunoglobulin G4-related Tubulointerstitial Nephritis with Simultaneous Resolution of Plasma Cell Infiltration and Fibrosis after Steroid Treatment.

Internal medicine (Tokyo, Japan)
2022

Immunoglobulin G4-Related Kidney Disease.

Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia
2022

Diagnostic Dilemma: Cardiorenal Syndrome As an Unusual Presentation of IgG4-Related Disease.

Indian journal of nephrology
2023

Comparison of clinicopathological features between patients with and without hypocomplementemia in IgG4-related kidney disease.

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
2023

Renal biopsy diagnosis of IgG4-related kidney disease with minor hematuria and mild renal dysfunction: lessons for the clinical nephrologist.

Journal of nephrology
2023

Recent advances in IgG4-related kidney disease.

Modern rheumatology
2022

Case Report: Atypical Manifestations Associated With FOXP3 Mutations. The "Fil Rouge" of Treg Between IPEX Features and Other Clinical Entities?

Frontiers in immunology
2022

IgG4-Related Kidney Disease Associated With End-Stage Kidney Disease, Renal Pseudotumor, and Renal Vein Thrombosis.

Cureus
2022

Nationwide epidemiological survey of immunoglobulin G4-related disease with malignancy in Japan.

Journal of gastroenterology and hepatology
2022

A Case of M-Type Phospholipase A2 Receptor-Associated Membranous Nephropathy With IgG4-Positive Cells Infiltration in the Interstitium.

Clinical medicine insights. Case reports
2022

Concurrent IgG4-Related disease and clear cell renal cell carcinoma.

Urology case reports
2021

[Clinicopathological characteristics and prognosis of IgG4-related kidney disease].

Zhonghua yi xue za zhi
2021

IgG4 Related Kidney Disease with Extra-Renal Involvement Demonstrated on FDG PET/CT.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2021

IgG4 tubulointerstitial nephritis - An uncommon enemy!

Indian journal of pathology &amp; microbiology
2021

A Case of IgG4-Related Kidney Disease Developing While on Steroid Treatment for Autoimmune IgG4 Pancreatitis.

Journal of investigative medicine high impact case reports
2021

Vasculitic neuropathy associated with IgG4-related kidney disease: A case report and literature review.

Clinical nephrology
2021

Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case-control study based on 450 patients.

Scientific reports
2021

Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight.

Frontiers in medicine
2021

IgG4-related kidney disease: Pathogenesis, diagnosis, and treatment.

Clinical nephrology
2021

Masquerader in oncology: IgG4-related kidney disease presenting as right upper ureteric mass.

Indian journal of cancer
2021

IgG4-related kidney disease with systemic Epstein-Barr virus infection: A case report.

Clinical nephrology
2021

Validation of the 2019 ACR/EULAR criteria for IgG4-related disease in a Japanese kidney disease cohort: a multicentre retrospective study by the IgG4-related kidney disease working group of the Japanese Society of Nephrology.

Annals of the rheumatic diseases
2021

Tertiary lymphoid tissue in early-stage IgG4-related tubulointerstitial nephritis incidentally detected with a tumor lesion of the ureteropelvic junction: a case report.

BMC nephrology
2021

IgG4-related nephritis and interstitial pulmonary disease complicated by invasive pulmonary fungal infection: a case report.

BMC nephrology
2020

[Computed Tomography Imaging Features of IgG4-related Nephropathy].

Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae
2021

Validation of the diagnostic criteria for IgG4-related kidney disease (IgG4-RKD) 2011, and proposal of a new 2020 version.

Clinical and experimental nephrology
2020

A case of eosinophilic granulomatosis with polyangiitis as a mimicker of IgG4-related disease.

Modern rheumatology case reports
2020

Idiopathic membranous nephropathy in a patient diagnosed with IgG4-related disease: A case report.

Medicine
2020

Clinicopathological features of IgG4-related kidney disease
.

Clinical nephrology
2020

Risks and characteristics of pancreatic cancer and pancreatic relapse in autoimmune pancreatitis patients.

Journal of gastroenterology and hepatology
2020

Low-density lipoprotein apheresis for PLA2R-related membranous glomerulonephritis accompanied by IgG4-related tubulointerstitial nephritis.

CEN case reports
2020

IgG4-related kidney disease: experience from a Mexican cohort.

Clinical rheumatology
2020

Clinical and imaging features of IgG4-related kidney disease.

Abdominal radiology (New York)
2021

Hypocomplementemia is related to elevated serum levels of IgG subclasses other than IgG4 in IgG4-related kidney disease.

Modern rheumatology
2020

An overlap of antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis and IgG4-related kidney disease.

Clinica chimica acta; international journal of clinical chemistry
2019

Acute Tubulointerstitial Nephritis as a Sole Manifestation of Immunoglobulin G4-Related Disease.

Journal of medical cases
2019

[A Case of Laparoscopic Resection of Cecal Carcinoma Complicated with IgG4-Related Disease].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2019

Progressive Renal Dysfunction due to IgG4-Related Kidney Disease Refractory to Steroid Therapy: A Case Report.

Case reports in nephrology and dialysis
2019

IgG4-related disease in the Japanese population: a genome-wide association study.

The Lancet. Rheumatology
2019

Imaging findings of IgG4-related kidney disease without extrarenal organ involvement: A case report.

Medicine
2019

IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review.

BMC nephrology
2019

IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia.

Case reports in nephrology and dialysis
2019

Clinical characteristics of autoimmune pancreatitis with IgG4 related kidney disease.

Advances in medical sciences
2019

Kidney Involvement in Patients with Type 1 Autoimmune Pancreatitis.

Journal of clinical medicine
2019

Simultaneous occurrence of IgG4-related Tubulointerstitial nephritis and colon adenocarcinoma with hepatic metastasis: a case report and literature review.

BMC nephrology
2019

A case of immunoglobulin G4-related inflammatory pseudotumor mimicking renal cell carcinoma.

Abdominal radiology (New York)
2019

Clinical Manifestations and Long-term Outcomes of IgG4-Related Kidney and Retroperitoneal Involvement in a United Kingdom IgG4-Related Disease Cohort.

Kidney international reports
2019

Immunoglobulin G4-Related Disease Arising from the Bladder Wall.

Urologia internationalis
2019

IgG4-related kidney disease and retroperitoneal fibrosis: An update.

Modern rheumatology
2018

An IgA1-lambda-type monoclonal immunoglobulin deposition disease associated with membranous features in a patient with IgG4-related kidney disease: a case report.

BMC nephrology
2019

Antineutrophil cytoplasmic antibody and/or antiglomerular basement membrane antibody associated crescentic glomerulonephritis in combination with IgG4-related tubulointerstitial nephritis.

Clinical and experimental rheumatology
2018

Immunoglobulin G4-related Kidney Disease Associated With Autoimmune Hemolytic Anemia.

Iranian journal of kidney diseases
2019

A case of IgG4-related kidney disease with predominantly unilateral renal atrophy.

CEN case reports
2018

Role of complement system in patients with biopsy-proven immunoglobulin G4-related kidney disease.

Human pathology
2018

Spectrum of IgG4-related Kidney Disease at a Tertiary Care Center.

Indian journal of nephrology
2018

Perirenal capsule and scrotal involvement in immunoglobulin G4-related kidney disease: case-based review.

Rheumatology international
2018

IgG4-related kidney disease: the effects of a Rituximab-based immunosuppressive therapy.

Oncotarget
2018

A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis.

Internal medicine (Tokyo, Japan)
2018

[IgG4-related kidney disease: Urologist trap?].

Progres en urologie : journal de l'Association francaise d'urologie et de la Societe francaise d'urologie
2017

Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment.

Chronic diseases and translational medicine
2017

Crescentic Glomerulonephritis With Immunoglobulin G4-Related Disease.

The American journal of the medical sciences
2017

IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass.

Case reports in surgery
2017

IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis.

The American journal of case reports
2017

IgG4-related membranous glomerulonephritis and generalized lymphadenopathy without pancreatitis: a case report.

BMC nephrology
2017

Bilateral renal hilar tumors in IgG4-related disease.

Clinical and experimental nephrology
2017

[A CASE OF IgG4-RELATED DISEASE WITH THICKENING OF THE RENAL PELVIS AT LEFT RENAL HILUM DIAGNOSED BY OBTURATOR LYMPH NODE DISSECTION].

Nihon Hinyokika Gakkai zasshi. The japanese journal of urology
2016

Factors related to renal cortical atrophy development after glucocorticoid therapy in IgG4-related kidney disease: a retrospective multicenter study.

Arthritis research &amp; therapy
2019

A case of IgG4-related tubulointerstitial nephritis and membranous glomerulonephritis during the clinical course of gastric cancer: Imaging features of IgG4-related kidney disease.

Modern rheumatology
2016

Coexistence of Acute Crescent Glomerulonephritis and IgG4-Related Kidney Disease.

Case reports in nephrology and dialysis
2016

IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis.

Seminars in liver disease
2016

Recognizing IgG4-related tubulointerstitial nephritis.

Canadian journal of kidney health and disease
2016

Distribution and components of interstitial inflammation and fibrosis in IgG4-related kidney disease: analysis of autopsy specimens.

Human pathology
2016

IgG4-Related Kidney Disease in a Patient With History of Breast Cancer: Findings on 18F-FDG PET/CT.

Clinical nuclear medicine
2017

Estimation of the number of histological diagnosis for IgG4-related kidney disease referred to the data obtained from the Japan Renal Biopsy Registry (J-RBR) questionnaire and cases reported in the Japanese Society of Nephrology Meetings.

Clinical and experimental nephrology
2016

IgG4-related nephropathy.

Journal of nephrology
2016

[IgG4-related kidney disease: what the nephrologist needs to know].

Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia
2016

IgG4-related Kidney Disease Mimicking Malignant Ureter Tumor: Case Report and Literature Review.

Medicine
2015

IgG4-related tubulointerstitial nephritis associated with only lymphadenopathy and without elevated serum IgG4 or renal imaging abnormalities: a case report and literature review.

International journal of clinical and experimental medicine
2015

A case of IgG4-related kidney disease complicated by eosinophilic lung disease.

CEN case reports
2015

Membranoproliferative glomerulonephritis with predominant IgG2 and IgG3 deposition in a patient with IgG4-related disease.

BMC nephrology
2018

IgG4-related stomach muscle lesion with a renal pseudotumor and multiple renal rim-like lesions: A rare manifestation of IgG4-related disease.

Modern rheumatology
2015

Immunoglobulin G4-Related Kidney Disease: A Comprehensive Pictorial Review of the Imaging Spectrum, Mimickers, and Clinicopathological Characteristics.

Korean journal of radiology
2015

IgG4-related kidney disease--A review.

Pathology, research and practice
2015

Immunoglobulin G4-related Kidney Disease as a Cause of Acute Renal Insufficiency.

Iranian journal of kidney diseases
2015

[112th Scientific Meeting of the Japanese Society of Internal Medicine: Symposium: Recent Progress in IgG4-related Disease: IgG4-related Kidney Disease・IgG4-related Retroperitoneal Fibrosis].

Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine
2016

Recovery of renal function after glucocorticoid therapy for IgG4-related kidney disease with renal dysfunction.

Clinical and experimental nephrology
2015

Organ Correlation in IgG4-Related Diseases.

Journal of Korean medical science
2015

An Overlapping Case of Lupus Nephritis and IgG4-Related Kidney Disease.

Journal of clinical medicine research
2015

IgG4-related kidney disease from the renal pelvis that mimicked urothelial carcinoma: a case report.

BMC urology
2015

IgG4-related Kidney Disease in Which the Urinalysis, Kidney Function and Imaging Findings Were Normal.

Internal medicine (Tokyo, Japan)
2015

A Case of IgG4-Related Hypophysitis Presented with Hypopituitarism and Diabetes Insipidus.

Clinical medicine insights. Case reports
2014

Distinct cytokine mRNA expression pattern in immunoglobulin G4-related kidney disease associated with renal cell carcinoma.

Clinical kidney journal
2015

Acute Interstitial Nephritis and Membranous Nephropathy in the Context of IgG4-Related Disease.

Case reports in nephrology and dialysis
2015

Immunoglobulin G4-related tubulointerstitial nephritis associated with interstitial pulmonary disease: Report of a case with review of literature.

Indian journal of nephrology
2015

[IgG4-related kidney disease. Diagnosis and treatment].

Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunology
2015

Ultrastructural studies of IgG4-related kidney disease.

Internal medicine (Tokyo, Japan)
2017

A case developing minimal change disease during the course of IgG4-related disease.

Modern rheumatology
2015

IgG4-related kidney disease--an update.

Current opinion in nephrology and hypertension

Associações

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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. The Effectiveness of Lesion Detection for Immunoglobulin G4-Related Kidney Diseases by Diffusion-Weighted Imaging.
    Nephron· 2026· PMID 40815121mais citado
  2. Urinary &#x3b2;2-microglobulin as a Potential Biomarker for Early Treatment Selection: A Patient with Severe IgG4-related Tubulointerstitial Nephritis.
    Internal medicine (Tokyo, Japan)· 2026· PMID 40670096mais citado
  3. Clinical features and outcomes of immunoglobulin G4-related kidney disease and immunoglobulin G4-related retroperitoneal fibrosis in Korea.
    Kidney research and clinical practice· 2026· PMID 40211870mais citado
  4. Ultrasonographic features of IgG4-related kidney disease: diagnostic value and correlation with clinical-pathological findings.
    Frontiers in medicine· 2025· PMID 41480534mais citado
  5. IgG4-related kidney disease: from renal histopathology and immunopathogenesis to novel pharmacological interventions.
    Expert review of clinical immunology· 2025· PMID 41082275mais citado
  6. IgG4-Related Kidney Disease With Hypocomplementemia and Circulating Immune Complex Containing IgG3 Presenting as Rapidly Progressive Glomerulonephritis: A Case Report.
    Kidney Med· 2026· PMID 41908616recente
  7. Concurrent IgG4-related membranous nephropathy and proliferative diabetic kidney disease: a diagnostic dilemma and therapeutic strategy in long-standing diabetes.
    BMC Nephrol· 2025· PMID 40722065recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:449395(Orphanet)
  2. MONDO:0018671(MONDO)
  3. GARD:21882(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55788248(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

Doença renal IgG4-relacionada
Compêndio · Raras BR

Doença renal IgG4-relacionada

ORPHA:449395 · MONDO:0018671
Prevalência
Unknown
Herança
Not applicable
CID-10
N11.8 · Outras nefrites túbulo-intersticiais crônicas
CID-11
Início
Adult, Elderly
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C5392056
EuropePMC
Wikidata
Papers 10a
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