Raras
Buscar doenças, sintomas, genes...
Doença oftalmológica IgG4-relacionada
ORPHA:449563CID-10 · H05.1CID-11 · 4A43.0DOENÇA RARA

Uma doença inflamatória que tem a ver com a IgG4, um tipo de anticorpo, e que afeta o olho.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Uma doença inflamatória que tem a ver com a IgG4, um tipo de anticorpo, e que afeta o olho.

Pesquisas ativas
1 ensaio
3 total registrados no ClinicalTrials.gov
Publicações científicas
170 artigos
Último publicado: 2026 Apr 13

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: H05.1
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Partes do corpo afetadas

👁️
Olhos
7 sintomas
🫘
Rins
6 sintomas
🩸
Sangue
6 sintomas
📏
Crescimento
3 sintomas
🫁
Pulmão
2 sintomas
🫃
Digestivo
2 sintomas

+ 32 sintomas em outras categorias

Características mais comuns

55%prev.
Sialadenite
Frequente (79-30%)
55%prev.
Eosinofilia
Frequente (79-30%)
55%prev.
Pancreatite
Frequente (79-30%)
55%prev.
Anormalidade do nervo infraorbital
Frequente (79-30%)
55%prev.
Dacriocistite
Frequente (79-30%)
55%prev.
Aumento do nível circulante de IgG4
Frequente (79-30%)
65sintomas
Frequente (16)
Ocasional (12)
Muito raro (14)
Sem dados (23)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 65 características clínicas mais associadas, ordenadas por frequência.

SialadeniteSialadenitis
Frequente (79-30%)55%
EosinofiliaEosinophilia
Frequente (79-30%)55%
PancreatitePancreatitis
Frequente (79-30%)55%
Anormalidade do nervo infraorbitalAbnormality of infra-orbital nerve
Frequente (79-30%)55%
DacriocistiteDacryocystitis
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico170PubMed
Últimos 10 anos159publicações
Pico202532 papers
Linha do tempo
2026Hoje · 2026🧪 2006Primeiro ensaio clínico📈 2025Ano de pico
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.
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 2 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Doença oftalmológica 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

Pesquisa e ensaios clínicos

3 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
162 papers (10 anos)
#1

Single-cell atlas of B cell heterogeneity in lacrimal IgG4-related disorders: Unraveling the transition from inflammatory exhaustion to clonal malignancy.

The FEBS journal2026 Mar 19

This study analyzed B-cell heterogeneity in lacrimal gland tissues from 4 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 2 patients with IgG4-positive MALT lymphoma using single-cell transcriptome sequencing (scRNA-seq). The results revealed that while the B-cell differentiation trajectories have similarities between the two diseases, critical differences were evident. IgG4-ROD was predominantly composed of naïve B cells, with memory B cells mainly being the central memory type. These cells were enriched in hormone/innate immunity pathways, and the plasma cells exhibited features of functional exhaustion. Conversely, MALT lymphoma was dominated by memory B cells, particularly enriched in terminally differentiated subtypes, with aberrant activation of oncogenic pathways (BCR/NF-κB). In MALT lymphoma, naïve B cells showed upregulated expression of immunoglobulin genes (IGHG3/IGHG4) and abnormal activation of EBV/BCR/T-cell differentiation pathways, despite having suppressed basal metabolism. Germinal center-like B cells in MALT lymphoma revealed upregulated gene expression enriched in T-cell activation and PD-1/PD-L1 pathways. Plasma cells in MALT lymphoma displayed monoclonal expansion (high expression of IGHG2/IGHG3/IGHG4) and enhanced antibody secretion. Therefore, MALT lymphoma is characterized by abnormal activation and malignant transformation of B cells, whereas IgG4-ROD manifests as functional exhaustion and metabolic suppression. The differentiation state of memory B cells may represent a critical juncture for malignant transformation.

#2

Application of artificial intelligence in differentiating IgG4-related ophthalmic disease and orbital MALT lymphoma: a review of radiomics and deep learning advances.

Frontiers in immunology2026

The differentiation between Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and orbital lymphoma, particularly the mucosa-associated lymphoid tissue (MALT) subtype, presents a significant clinical challenge due to overlapping imaging features and similar presentations. Recent advances in artificial intelligence (AI), particularly radiomics and deep learning, have shown promising potential in enhancing diagnostic accuracy by extracting high-dimensional imaging features and constructing robust predictive models. This review systematically examines the current state of AI applications in distinguishing IgG4-ROD from orbital MALT lymphoma, highlighting key methodologies in image-based feature extraction, model development, and diagnostic performance evaluation. We explore various AI techniques applied to multimodal imaging data integration and discuss optimization strategies for deep learning architectures tailored to this clinical context. Additionally, the review addresses the practical challenges and limitations of translating AI-assisted diagnostic tools into routine clinical practice, including issues related to small sample sizes, retrospective single-center designs, data variability, interpretability, and the critical need for robust external validation. By synthesizing recent research findings, this review aims to provide a comprehensive overview of AI-driven diagnostic advances, critically assess current challenges, and propose future directions to improve the accuracy and reliability of orbital disease differentiation, ultimately supporting more precise clinical decision-making.

#3

Clinical significance of serum IgE levels in IgG4-related ophthalmic disease: a single-centre study.

Eye (London, England)2026 Feb 25

To explore the role of serum IgE in IgG4-related ophthalmic disease (IgG4-ROD) by comparing clinical features and prognosis of patients with elevated and normal serum IgE levels. This single-centre retrospective study included 54 IgG4-ROD patients diagnosed by histopathology from March 2022 to March 2024. Clinical features of the IgE+ group (serum IgE levels >165 IU/ml) and the IgE- group (serum IgE levels ≤ 165 IU/ml) were compared, and 50 patients were followed for at least 6 months to assess the prognosis. Statistical analyses included t tests, Mann-Whitney U, chi-square tests, Spearman correlation, Kaplan-Meier survival curves, and Cox regression. Among the 54 patients, both the IgE+ (n = 22) and IgE- groups (n = 32) were predominantly middle-aged women. Extraocular muscle involvement was more common in the IgE- group (P = 0.033), whereas bilateral ocular (P = 0.040) and sinus involvement (P = 0.046) were more frequent in the IgE+ group. Significant intergroup differences were observed in serum IgG4 (P = 0.004), C1q (P = 0.021), IL-10 levels (P = 0.030), IgG4/IgG (P = 0.022), and IgE/IgG4 ratios (P = 0.018). Serum IgE correlated positively with IgG4, IL-6, IL-10, and IL-12p70, and negatively with C1q and IgM (P < 0.05). The IgE+ group had a higher relapse risk (P = 0.038). Cox regression analysis identified serum IgE as an independent risk factor for relapse (P = 0.022). Elevated serum IgE in IgG4-ROD is associated with more severe disease and higher relapse risk, highlighting its potential as a prognostic marker.

#4

Autoimmune fibroinflammation in IgG4-related ophthalmic disease: TLR8-dependent signaling pathways and fibrotic remodeling revealed by proteomic profiling.

Frontiers in immunology2026

Immunoglobulin G4-related ophthalmic disease (IgG4-ROD) is an immune-mediated ocular condition characterized by tumefactive lesions with IgG4+ plasma cell infiltration, and commonly affecting the lacrimal gland, extraocular muscles, and trigeminal nerves.The precise pathogenesis of IgG4-ROD remains unclear. Elucidating its molecular mechanisms is crucial for the development of targeted molecular therapies. To investigate the molecular pathogenesis of IgG4-ROD, we conducted a case-controlled study involving 15 patients who presented at the Second Hospital of Jilin University between 2021 and 2022. In accordance with the comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD) established in 2011, participants were stratified into three distinct cohorts based on lacrimal gland histopathological findings: confirmed IgG4-ROD, suspected IgG4-ROD, and a control group. We then utilized 4D-Fast DIA technology to acquire proteomic profiles from the lacrimal gland biopsies, with rigorous bioinformatics methodologies employed to process and interpret these data, focusing on the differential protein expression patterns across the groups, aiming to identify signaling pathways that are significantly associated with IgG4-ROD. The comparative analysis of clinical characteristics, imaging features, and histopathological findings among the control group, patients with suspected IgG4-ROD, and diagnosed patients revealed a progressive trend towards more severe pathology. Principal component analysis (PCA) and Pearson correlation heatmaps indicated that the profiles of differentially expressed proteins in lacrimal gland samples from the suspected and diagnosed groups were highly similar, suggesting that the patients from these two groups may belong to the same population. Further analysis of protein expression changes across the three groups revealed significant enrichment in pathways related to asthma, Th1 and Th2 cell differentiation, and other relevant signaling pathways. Notably, Toll-like receptor (TLR), NF-κB, Wnt, and PI3K-AKT signaling pathways were prominently activated. In the diagnosed group, proteins such as MMP7, POSTN, and CD163 exhibited characteristic high-level expression. Furthermore, compared with the suspected group, the diagnosed group showed significant downregulation of proteins related to elastin fibers, indicating a more severe degree of fibrosis in the lacrimal gland tissues. Additionally, the diagnosed group exhibited a significant decrease in proteins associated with lacrimal secretion, suggesting impaired function of lacrimal gland. We also observed a notable upregulation of TLR-8-related proteins in both the suspected and diagnosed groups, implying that the TLR signaling pathway may be closely related to this disease. Proteomic analysis of lacrimal gland samples from the diagnosed, suspected, and control groups suggests that patients with suspected IgG4-ROD may be part of the same population as diagnosed patients. In the diagnosed group, the lacrimal gland tissue displayed more severe fibrosis and a significant loss of lacrimal secretion function. This study postulates that the TLR - 8/IRAK4/NF - κB pathway may contribute to the molecular pathogenesis of IgG4 - ROD by promoting tissue fibrosis. Proteins such as MMP7, POSTN, and CD163 could potentially serve as molecular markers for the early diagnosis and potential therapeutic targets of IgG4 - ROD. Based on these findings, proteomics offers significant advantages in the molecular diagnosis of IgG4 - ROD and should be regarded as a crucial tool in the diagnosis of this disease.

#5

Bilateral orbital masses caused by IgG4-expressing MALT lymphoma in the right side and IgG4-related ophthalmic disease in the left side: A case report.

Modern rheumatology case reports2026 Jan 06

A 60-year-old Japanese man developed a protruding right eye. He underwent a magnetic resonance imaging scan, which revealed a right orbital mass. The serum immunoglobulin G4 (IgG4) level was elevated, and IgG4+ plasma cells were observed in biopsy specimens of the mass. The result of biopsy and Southern blot analysis revealed that the mass was caused by IgG4+ extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. He received a total of 36 Gy of radiation therapy, and the mass disappeared. Five years later, he developed a protruding left eye. The magnetic resonance imaging scan at that time revealed a left orbital mass. Biopsy revealed findings of IgG4-related disease in the left orbital mass, but no findings of mucosa-associated lymphoid tissue lymphoma. He has been followed up without glucocorticoid treatment. Here, we report a patient who developed IgG4+ MALT lymphoma in the right orbital mass and IgG4-related ophthalmic disease in the left orbital mass. Because the treatment strategy for IgG4-related ophthalmic disease and malignant lymphoma is completely different, we emphasise the need for biopsy.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC102 artigos no totalmostrando 155

2026

Single-cell atlas of B cell heterogeneity in lacrimal IgG4-related disorders: Unraveling the transition from inflammatory exhaustion to clonal malignancy.

The FEBS journal
2026

Application of artificial intelligence in differentiating IgG4-related ophthalmic disease and orbital MALT lymphoma: a review of radiomics and deep learning advances.

Frontiers in immunology
2026

Clinical significance of serum IgE levels in IgG4-related ophthalmic disease: a single-centre study.

Eye (London, England)
2026

Autoimmune fibroinflammation in IgG4-related ophthalmic disease: TLR8-dependent signaling pathways and fibrotic remodeling revealed by proteomic profiling.

Frontiers in immunology
2026

Bilateral orbital masses caused by IgG4-expressing MALT lymphoma in the right side and IgG4-related ophthalmic disease in the left side: A case report.

Modern rheumatology case reports
2026

A patient-specific multidisciplinary approach to improve patient outcomes in IgG4-related ophthalmic disease.

Indian journal of ophthalmology
2026

Paradoxical Development of IgG4-Related Ophthalmic Disease During Dupilumab Therapy.

Seminars in ophthalmology
2026

Advancing the diagnosis of IgG4-related ophthalmic disease: a critical appraisal of current criteria.

Journal of clinical pathology
2025

Clinicopathological features differentiating Castleman Disease from IgG4-related ophthalmic disease, a 10 years retrospective study.

Indian journal of ophthalmology
2025

Case Report: IgG4-related ophthalmic disease presenting with unilateral proptosis.

Frontiers in immunology
2025

Comparison of volumetric responses to different corticosteroid administration methods in IgG4-related ophthalmic disease.

PloS one
2025

IgG4-Positive Conjunctival Tumor with Systemic Allergy: A Case Report.

Ocular immunology and inflammation
2025

Gut microbiome alterations in IgG4-related ophthalmic disease.

BMC microbiology
2025

Orbital and Adnexal IgG4-related disease: An insight into the clinical profile and management outcomes.

Indian journal of ophthalmology
2025

Applications of New Generation Sequencing (NGS) in Ocular Oncology.

International ophthalmology clinics
2025

Chronic Dacryocystitis With Elevated IgG4-Plasma Cells in a Patient With Rubinstein-Taybi Syndrome: An IgG4-Related Disease?

Ophthalmic plastic and reconstructive surgery
2025

A case of IgG4-related ophthalmic disease with bone destruction presenting as unilateral painful blepharitis.

International journal of ophthalmology
2025

Anterior scleritis with IgG4 lymphoplasmacytic infiltration: a case report.

Journal of ophthalmic inflammation and infection
2025

Gene expression cluster differences and molecular correlation with the STING pathway in orbital MALT lymphoma and orbital IgG4-related eye disease.

Discover oncology
2025

A Case in Which the Endoscopic Denker's Approach Was Useful in the Diagnosis of IgG4-Related Ophthalmic Disease.

Cureus
2025

IgG4-Related Ophthalmic Disease with Proptosis.

Balkan medical journal
2025

Labial salivary gland biopsy for diagnosing immunoglobulin G4-related ophthalmic disease with optic neuropathy: a case report.

Orbit (Amsterdam, Netherlands)
2025

Periocular Manifestations and Management of Immunoglobulin G4-Related Disease.

International ophthalmology clinics
2025

Perilesional dominance: radiomics of multiparametric MRI enhances differentiation of IgG4-Related ophthalmic disease and orbital MALT lymphoma.

BMC medical imaging
2025

Immunoglobulin G4-related ophthalmic disease with orbital deep hemangioma: A case report.

The Journal of international medical research
2025

Orbital inflammatory disease in a primarily black patient population.

Frontiers in ophthalmology
2025

Adult-Onset Asthma With Periocular Xanthogranuloma (AAPOX) Associated With IgG4-Related Disorder: A Case Report and Review of Current Literature.

Cureus
2025

A Case of IgG4-Related Disease Presenting With Concurrent Uveitis, Posterior Scleritis, Serous Retinal Detachment, and Choroidal Thickening Mimicking Intraocular Lymphoma.

Cureus
2025

SAPHO syndrome complicated by IgG4-related ophthalmic disease: a case report and literature review.

Frontiers in immunology
2025

Immunoglobulin G4-Related Ophthalmic Disease: A Nationwide Multicenter Study in Korea.

Ophthalmology
2025

IgG4-related ophthalmic disease presenting as posterior scleritis in a pediatric patient.

Journal of ophthalmic inflammation and infection
2025

Single-cell RNA-sequencing reveals cellular heterogeneity and immune microenvironment characteristics between ocular adnexal mucosa-associated lymphoid lymphoma and IgG4-related ophthalmic disease.

Frontiers in immunology
2025

Extensive Involvement of the Bilateral Trigeminal Nerve Branch as the Only Imaging Manifestation of IgG4‑Related Ophthalmic Disease.

Journal of the Belgian Society of Radiology
2025

Successful glucocorticoid treatment case for IgG4-related long-standing optic neuropathy.

Modern rheumatology case reports
2025

Similarities and differences of a proliferation-inducing ligand expression in lacrimal gland lesions of patients with IgG4-associated ophthalmic diseases and mucosa-associated lymphoid tissue lymphoma.

Frontiers in immunology
2025

Normative measurements of the frontal nerve by magnetic resonance imaging in an Australia cohort.

Surgical and radiologic anatomy : SRA
2025

Postoperative outcomes of biopsy versus debulking surgery for immunoglobulin G4-related ophthalmic disease: a retrospective comparative study.

Japanese journal of ophthalmology
2024

Comparison between Immunoglobulin G4-Related Eye Disease and Other Entities with Non-Immunoglobulin G4 Ocular Involvement.

European journal of rheumatology
2024

Advances in understanding and management of IgG4-related ophthalmic disease.

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.)
2024

Evaluation of the specificity of trigeminal nerve enlargement in the diagnosis of IgG4-related ophthalmic disease.

Japanese journal of ophthalmology
2024

IL-33/ST2 enhances MMP-12 expression by macrophages to mediate inflammatory and immune response in IgG4-Related Ophthalmic Disease.

Cytokine
2025

Normative Measurements of the Infraorbital Nerve by Magnetic Resonance Imaging in an Australia Cohort.

Ophthalmic plastic and reconstructive surgery
2025

Assessing IgG4-related ophthalmic disease and its mimics: a comparison of ACR/EULAR, organ-specific and revised comprehensive diagnostic criteria.

Journal of clinical pathology
2024

The 2023 revised diagnostic criteria for IgG4-related ophthalmic disease.

Japanese journal of ophthalmology
2024

Unraveling the Clinical Features and Outcomes of IgG4-Related Ophthalmic Disease.

Journal of clinical medicine
2024

Infraorbital nerve involvement as a pathognomonic sign of IgG4-related ophthalmic disease.

Joint bone spine
2024

Clinical characteristics and treatment outcomes of patients with IgG4-positive ocular adnexal marginal zone B-cell lymphoma.

Indian journal of ophthalmology
2024

Artificial intelligence-based differential diagnosis of orbital MALT lymphoma and IgG4 related ophthalmic disease using hematoxylin-eosin images.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2024

A case of IgG4-related ophthalmic disease after SARS-CoV-2 vaccination: case report and literature review.

Frontiers in immunology
2024

IgG4-related uveitis. A French cohort and literature review.

Seminars in arthritis and rheumatism
2024

Successful remission induction of IgG4-related ophthalmic disease by obinutuzumab therapy: a retrospective study of 8 patients.

Eye (London, England)
2025

IgG4-Related Ophthalmic Disease following COVID-19 Infection: Is it Associated or Incidental?

Klinische Monatsblatter fur Augenheilkunde
2023

Radiomics analysis of T1WI and T2WI magnetic resonance images to differentiate between IgG4-related ophthalmic disease and orbital MALT lymphoma.

BMC ophthalmology
2023

Efficacy and safety of intravenous glucocorticoid therapy for IgG4-related ophthalmic disease.

PloS one
2023

IgG4-related ophthalmic disease masquerading as ciliary body tumors and scleritis in both eyes: a case report.

BMC ophthalmology
2023

IgG4-related disease presenting with profound bilateral orbital and adnexal inflammation.

American journal of ophthalmology case reports
2022

Long-Term Follow-Up in IgG4-Related Ophthalmic Disease: Serum IgG4 Levels and Their Clinical Relevance.

Journal of personalized medicine
2022

[Cerebral venous sinus thrombosis presenting transient ischemic attack after recovery from COVID-19 with Graves' disease and IgG4-related ophthalmic disease: a case report].

Rinsho shinkeigaku = Clinical neurology
2023

Treatment outcomes and their determinants of IgG4-related ophthalmic disease: a territory-wide cohort study.

The British journal of ophthalmology
2024

Probable IGG4 related ophthalmic disease presenting with uveitis.

Orbit (Amsterdam, Netherlands)
2022

Local recurrence as extraocular muscle lymphoma after 6 years of chronic myositis: a case report.

BMC ophthalmology
2022

A Case of Immunoglobulin G4-Related Ophthalmic Disease With Unilateral Visual Field Impairment.

Cureus
2022

IgG4-related disease as multiple head and neck swellings: supported by histopathology and immunohistochemistry.

Romanian journal of ophthalmology
2022

Lacrimal Gland and Orbital Lesions in LatY136F Knock-in Mice, a Model for Human IgG4-Related Ophthalmic Disease.

Current eye research
2022

Apocrine Hidrocystoma with IgG4 Plasma Cell Infiltration Presenting as Recurrent Chalazion: A Case Report.

Medicina (Kaunas, Lithuania)
2022

IgG4-Related Ophthalmic Disease.

The New England journal of medicine
2023

Malignancies in Immunoglobulin G4-related ophthalmic disease.

European journal of ophthalmology
2022

Pathological count of IgG4-positive plasmacytes suggests extraophthalmic involvement and relapse in patients with IgG4-related ophthalmic disease: a retrospective study.

Arthritis research &amp; therapy
2022

IgG4-related hypertensive granulomatous anterior uveitis.

American journal of ophthalmology case reports
2022

Activation of Mast-Cell-Derived Chymase in the Lacrimal Glands of Patients with IgG4-Related Ophthalmic Disease.

International journal of molecular sciences
2022

The Role of Symptom Duration and Serologic Factors in the Relapse of IgG4-Related Ophthalmic Disease following Surgery: A Retrospective Cohort Study.

Disease markers
2022

Re: "Clinical Outcomes of IgG4-Related Ophthalmic Disease and Idiopathic Sclerosing Orbital Inflammation".

Ophthalmic plastic and reconstructive surgery
2022

Cases with IgG4-related ophthalmic disease with mass lesions surrounding the optic nerve.

American journal of ophthalmology case reports
2021

Long-Term Outcomes of IgG4-Related Ophthalmic Disease in a Chinese IgG4-Related Disease Cohort.

Frontiers in medicine
2021

Ophthalmic involvement disparities in clinical characteristics of IgG4-related disease: a retrospective study of 573 patients.

BMC ophthalmology
2023

IgG4-related ophthalmic disease in association with adult-onset asthma and periocular xanthogranuloma: a case report.

Orbit (Amsterdam, Netherlands)
2022

Clinical analysis of ocular adnexal mucosa-associated lymphoid tissue lymphoma with IgG4-related ophthalmic disease.

Orbit (Amsterdam, Netherlands)
2022

F-18 Fluorodeoxyglucose PET/CT as a Diagnostic Tool in Orbital Inflammatory Disorders.

Ocular immunology and inflammation
2021

Clinico-epidemiological analysis of 1000 cases of orbital tumors.

Japanese journal of ophthalmology
2021

IgG4-related disease of the orbit in an infant.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2021

Clinical features and symptoms of IgG4-related ophthalmic disease: a multicenter study.

Japanese journal of ophthalmology
2022

Low-dose 'boom-boom' radiotherapy for ocular lymphoma arising from IgG4-related ophthalmic disease: Case report and literature review.

European journal of ophthalmology
2022

IgG4-related disease in pediatric patients: a single-center experience.

Rheumatology international
2021

Upregulated Expression of Activation-Induced Cytidine Deaminase in Ocular Adnexal Marginal Zone Lymphoma with IgG4-Positive Cells.

International journal of molecular sciences
2021

[IgG4-related ophthalmic disease: three-case report].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2021

Clinical features and relapse risks of IgG4-related ophthalmic disease: a single-center experience in China.

Arthritis research &amp; therapy
2022

Clinical Outcomes of IgG4-Related Ophthalmic Disease and Idiopathic Sclerosing Orbital Inflammation.

Ophthalmic plastic and reconstructive surgery
2021

Morphologic Cross-Sectional Imaging Features of IgG4-Related Orbitopathy in Comparison to Ocular Adnexal Lymphoma.

Clinical ophthalmology (Auckland, N.Z.)
2021

Follow-up with serum IgG4-monitoring in 8 patients with IgG4-related disease diagnosed by a lacrimal gland mass.

Journal of clinical and experimental hematopathology : JCEH
2021

To Explore MR Imaging Radiomics for the Differentiation of Orbital Lymphoma and IgG4-Related Ophthalmic Disease.

BioMed research international
2021

Surgical management for IgG4-related ophthalmic disease by a transcranial biopsy combined with extraorbital decompression: illustrative case.

Journal of neurosurgery. Case lessons
2021

Differential Tissue Metabolic Signatures in IgG4-Related Ophthalmic Disease and Orbital Mucosa-Associated Lymphoid Tissue Lymphoma.

Investigative ophthalmology &amp; visual science
2020

Identification of Markers Predicting Clinical Course in Patients with IgG4-Related Ophthalmic Disease by Unbiased Clustering Analysis.

Journal of clinical medicine
2020

Radiologic imaging shows variable accuracy in diagnosing orbital inflammatory disease and assessing its activity.

Scientific reports
2020

[The imaging signs and significances of the enlargement of the infraorbital nerve and the frontal nerve in orbital lymphoproliferative diseases].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2020

Comprehensive Gene Analysis of IgG4-Related Ophthalmic Disease Using RNA Sequencing.

Journal of clinical medicine
2020

Image-Guided Intensity-Modulated Radiation Therapy for IgG4-Related Ophthalmic Disease.

Case reports in ophthalmological medicine
2020

Distinctive Tissue and Serum MicroRNA Profile of IgG4-Related Ophthalmic Disease and MALT Lymphoma.

Journal of clinical medicine
2021

A Case of IgG4-Related Ophthalmic Disease With Coronary Arteritis.

Ophthalmic plastic and reconstructive surgery
2021

Clinical features and outcomes of IgG4-related idiopathic orbital inflammatory disease: from a large southern China-based cohort.

Eye (London, England)
2020

Alteration of Cell Surface Markers CD38 and CD138 in Lymphoproliferative Disorders in the Ocular Adnexa.

Anticancer research
2020

Corticosteroid Withdrawal after Complete Resection of Recurrent IgG4-Related Ophthalmic Disease.

Neuro-ophthalmology (Aeolus Press)
2020

Bilateral IgG4-related Ophthalmic Disease With Diffuse Large B-cell Lymphoma of the Right Eye: A Case Report.

Ophthalmic plastic and reconstructive surgery
2020

Orbital inflammation and colitis in pediatric IgG4-related disease: A case report and review of the literature.

European journal of rheumatology
2019

[Clinical and pathological study on IgG4-related ophthalmic disease involving the lacrimal gland].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2019

The natural course of IgG4-related ophthalmic disease after debulking surgery: a single-centre retrospective study.

BMJ open ophthalmology
2019

IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis.

Case reports in ophthalmological medicine
2021

MULTIMODAL IMAGING OF IGG4-RELATED PAPILLITIS AND RETINAL DISEASE.

Retinal cases &amp; brief reports
2020

Long-term outcomes of ocular adnexal lesions in IgG4-related ophthalmic disease.

The British journal of ophthalmology
2019

Magnetic resonance imaging indicator of the causes of optic neuropathy in IgG4-related ophthalmic disease.

BMC medical imaging
2019

IgG4-related Ophthalmic Disease in Idiopathic Sclerosing and Non-Sclerosing Orbital Inflammation: A 25-Year Experience.

Current eye research
2020

IgG4-positive Cell Quantification Distinguishes Between Inflammatory and Noninflammatory Diseases of the Orbit.

Applied immunohistochemistry &amp; molecular morphology : AIMM
2019

Occult Nasolacrimal Duct Obstruction Secondary to IgG4-Related Ophthalmic Disease.

Ophthalmic plastic and reconstructive surgery
2018

Diagnosis of IgG4 - related ophthalmic disease in a group of patients with various lesions of the eye and orbits.

Terapevticheskii arkhiv
2019

IgG4-related disease in patients with idiopathic orbital inflammation syndrome: data from the French SIOI prospective cohort.

Acta ophthalmologica
2018

Purpura fulminans with Lemierre's syndrome caused by Gemella bergeri and Eikenella corrodens: a case report.

BMC infectious diseases
2018

A clinicopathological study on IgG4-related ophthalmic disease.

International journal of ophthalmology
2019

Distinguishing IgG4-Related Ophthalmic Disease From Graves Orbitopathy.

Ophthalmic plastic and reconstructive surgery
2019

A case of fatal osteolytic hypercalcemia complicated with IgG4-related ophthalmic disease leading to renal failure.

CEN case reports
2018

The Case of IgG4-Related Ophthalmic Disease with Perivascular Lesions of Superior Ophthalmic Vein Associated with Optic Nerve Disturbance.

Neuro-ophthalmology (Aeolus Press)
2018

The Case of IgG4-related Ophthalmic Disease Accompanied by Compressive Optic Neuropathy.

Neuro-ophthalmology (Aeolus Press)
2018

[Clinical analysis of patients with orbital nonspecific inflammatory response diseases whose CT or MR images show enlargement of the infraorbital nerves].

[Zhonghua yan ke za zhi] Chinese journal of ophthalmology
2018

IgG4-related ophthalmic disease involving extraocular muscles: case series.

BMC ophthalmology
2018

Longstanding IgG4-related Ophthalmic Disease Dramatically Improved after Steroid Therapy.

Internal medicine (Tokyo, Japan)
2018

Orbital progressive transformation of germinal centers as part of the spectrum of IgG4-related ophthalmic disease: Clinicopathologic features of three cases.

Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological Society
2018

Immunoglobulin G4-related ophthalmic disease.

Taiwan journal of ophthalmology
2018

Immunoglobulin G4-Related Disease: An Update.

Oman medical journal
2018

Infraorbital Nerve Involvement on Magnetic Resonance Imaging in Igg4-Related Ophthalmic Disease: A Highly Suggestive Sign.

Ophthalmology
2018

Immunoglobulin G4-related dacyroadenitis presenting as bilateral chorioretinal folds from severely enlarged lacrimal glands.

American journal of ophthalmology case reports
2018

Clinicoserological factors associated with response to steroid treatment and recurrence in patients with IgG4-related ophthalmic disease.

The British journal of ophthalmology
2018

The 2017 Doyne Lecture: the orbit as a window to systemic disease.

Eye (London, England)
2019

Clinical outcomes and pathological characteristics of immunoglobulin G4-related ophthalmic disease versus orbital inflammatory pseudotumor.

The Korean journal of internal medicine
2017

Frequency and distribution of CD4+CXCR5+ follicular B helper T cells within involved tissues in IgG4‑related ophthalmic disease.

Molecular medicine reports
2018

Risk factors for extraophthalmic involvement and treatment outcomes in patients with IgG4-related ophthalmic disease.

The British journal of ophthalmology
2018

Repeat Biopsy of IgG4-Related Ophthalmic Disease Identifies Superimposed Herpes Simplex Virus Infection: A Case Report.

Ocular immunology and inflammation
2017

Usefulness of Flow Cytometry in Diagnosis of IgG4-Related Ophthalmic Disease and Extranodal Marginal Zone B-Cell Lymphoma of the Ocular Adnexa.

Anticancer research
2017

IgG4-related disease in the eye and ocular adnexa.

Current opinion in ophthalmology
2017

Change of Serum IgG4 in Patients with Ocular Adnexal Marginal Zone B Cell Lymphoma Associated with IgG4-Related Ophthalmic Disease After Treatment.

Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics
2017

Immunophenotypic profiles for distinguishing orbital mucosa-associated lymphoid tissue lymphoma from benign lymphoproliferative tumors.

Japanese journal of ophthalmology
2019

IGG4-RELATED OPHTHALMIC DISEASE PRESENTING AS CHOROIDAL AND ORBITAL LESIONS.

Retinal cases &amp; brief reports
2017

Ophthalmic manifestations in IgG4-related disease: Clinical presentation and response to treatment in a French case-series.

Medicine
2016

Immunoglobulin G4-Related Ophthalmic Disease Involving the Sclera Misdiagnosed as Intraocular Tumor: Report of One Case.

Ocular oncology and pathology
2016

Orbital Involvement and Ocular Surface Changes in IgG4-Related Systemic Disease.

Cornea
2019

Ocular adnexal marginal zone lymphoma arising in a patient with IgG4-related ophthalmic disease.

Modern rheumatology
2017

Infraorbital nerve involvement on magnetic resonance imaging in European patients with IgG4-related ophthalmic disease: a specific sign.

European radiology
2016

[Diagnostic Criteria for IgG4-related Ophthalmic Disease].

Nippon Ganka Gakkai zasshi
2016

IgG4-related disease presenting as hypertrophic pachymeningitis and compressive optic neuropathy.

Joint bone spine
2016

Bilateral IgG4-related ophthalmic disease: a strong indication for systemic imaging.

The British journal of ophthalmology
2017

IgG4 Staining in Thyroid Eye Disease.

Ophthalmic plastic and reconstructive surgery
2016

Intraorbital corticosteroid injection for the treatment of IgG4-related ophthalmic disease.

The British journal of ophthalmology
2015

Immunoglobulin G4-related ophthalmic disease presenting as uveitis.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2015

IgG4-Related Ophthalmic Disease: Pooling of Published Cases and Literature Review.

Current allergy and asthma reports
2015

Genome-Wide Analysis of Ocular Adnexal Lymphoproliferative Disorders Using High-Resolution Single Nucleotide Polymorphism Array.

Investigative ophthalmology &amp; visual science
2015

IgG4-Related Ophthalmic Disease. Part II: Clinical Aspects.

Ophthalmic plastic and reconstructive surgery
2015

IgG4-related ophthalmic disease. Part I: background and pathology.

Ophthalmic plastic and reconstructive surgery

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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. Single-cell atlas of B cell heterogeneity in lacrimal IgG4-related disorders: Unraveling the transition from inflammatory exhaustion to clonal malignancy.
    The FEBS journal· 2026· PMID 41853890mais citado
  2. Application of artificial intelligence in differentiating IgG4-related ophthalmic disease and orbital MALT lymphoma: a review of radiomics and deep learning advances.
    Frontiers in immunology· 2026· PMID 41756276mais citado
  3. Clinical significance of serum IgE levels in IgG4-related ophthalmic disease: a single-centre study.
    Eye (London, England)· 2026· PMID 41741699mais citado
  4. Autoimmune fibroinflammation in IgG4-related ophthalmic disease: TLR8-dependent signaling pathways and fibrotic remodeling revealed by proteomic profiling.
    Frontiers in immunology· 2026· PMID 41685325mais citado
  5. Bilateral orbital masses caused by IgG4-expressing MALT lymphoma in the right side and IgG4-related ophthalmic disease in the left side: A case report.
    Modern rheumatology case reports· 2026· PMID 41609102mais citado
  6. Clinicopathological and Imaging Distinction Between Ocular Adnexal MALT Lymphoma and IgG4-Related Ophthalmic Disease.
    Am J Ophthalmol· 2026· PMID 41985855recente
  7. CT-guided biopsy with fine-needle via the anterior approach in the diagnosis of orbital lymphoma: a case series.
    Orbit· 2026· PMID 41949825recente
  8. Exploring the intersection: adult orbital xanthogranulomatous disease and IgG4-related disease - report of 3 cases and literature review.
    Orbit· 2026· PMID 41949590recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:449563(Orphanet)
  2. MONDO:0018675(MONDO)
  3. GARD:21885(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q25312948(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 oftalmológica IgG4-relacionada
Compêndio · Raras BR

Doença oftalmológica IgG4-relacionada

ORPHA:449563 · MONDO:0018675
Prevalência
Unknown
Herança
Not applicable
CID-10
H05.1 · Transtornos inflamatórios crônicos da órbita
CID-11
Ensaios
1 ativos
Início
Adult, Elderly
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C5681103
EuropePMC
Wikidata
Papers 10a
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