Processo inflamatório fibrótico crônico que afeta a glândula salivar. Os sinais e sintomas incluem inchaço firme e doloroso da glândula salivar, frequentemente associado à presença de cálculos nas glândulas salivares.
Introdução
O que você precisa saber de cara
Processo inflamatório fibrótico crônico que afeta a glândula salivar. Os sinais e sintomas incluem inchaço firme e doloroso da glândula salivar, frequentemente associado à presença de cálculos nas glândulas salivares.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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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
+ 14 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 25 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Doença da glândula submandibular IgG4-relacionada
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Inebilizumab treatment in Japanese patients with IgG4-related disease: a subgroup analysis of a randomized, double-blind, placebo-controlled, phase 3 trial (MITIGATE).
This subgroup analysis of the MITIGATE study aimed to evaluate whether the efficacy and safety of inebilizumab in Japanese patients with IgG4-related disease are consistent with those observed in the overall trial population. MITIGATE was a randomized, double-blind, placebo-controlled, phase 3 study conducted in 22 countries, and this subgroup analysis included Japanese patients enrolled in that trial. In total, 27 Japanese patients (inebilizumab, N = 20; placebo, N = 7) were included in this subgroup analysis. The organs most frequently affected were the submandibular gland (66.7%), lacrimal gland (59.3%), and pancreas (51.9%). Despite the discontinuation of glucocorticoids within 8 weeks of study initiation, there were no adjudication committee-determined flares requiring treatment in the inebilizumab group. Flares were observed in four patients (57.1%) in the placebo group, with a median time to first flare of 246.0 days. Inebilizumab reduced annualized flare rate, increased flare-free complete remission, and lowered glucocorticoid use. Safety profiles were similar between treatment groups; most treatment-emergent adverse events were non-serious. Inebilizumab reduced flare risk and glucocorticoid exposure and was well tolerated in Japanese patients with IgG4-related disease, consistent with overall MITIGATE results. NCT04540497.
Comparison of IgG4 related disease in patients aged≤25 years (pediatric, adolescent, and young adult) and those aged≥50 years.
To evaluate and compare the manifestations of IgG4-RD in patients≤25 years of age (pediatric, adolescents and young adults) and those≥50 years of age. A retrospective descriptive study of 64 patients diagnosed with IgG4-RD based on the Umehara 2011/2020 criteria and the ACR/EULAR 2019 classification criteria, covering the period from 2014 to 2023. The medical records of these two age groups were reviewed. Of the 64 patients, 7 were ≤25 years old (11%) and 35 were ≥50 years old (54.7%). Among patients≤25 years old, 6 were female, with a mean age at consultation of 21.3 years (± 5.06). Five (71%) had single-organ involvement, while two presented with multi-organ involvement. In the ≥50 years group, females also predominated (57%), with a mean age at consultation of 60 years (± 7.4) and more constitutional symptoms were observed, multi-organ involvement (p=0.009), asthenia (p=0.047), lymph nodes (p=0.047), submandibular gland involvement (p=0.009), lung involvement (p=0.013) and parotidomegaly (p=0.097). Ocular involvement was more frequent in those under 25 years of age; however, in those over 50 years of age, this involvement was mostly bilateral (p=0.011). There were no statistically significant differences in laboratory variables between both groups. There was a predominance of lymphoplasmacytic infiltrate in the majority of the biopsies performed. In cases of ocular involvement, histopathological analysis revealed lymphoplasmacytic infiltrate with no differences in the presence of storiform fibrosis, obliterative phlebitis, or eosinophilic infiltrate. However, the latter was more frequent in older patients. Reviewing the literature and describing our data on IgG4-RD in children is crucial, as pediatric physicians often overlook this condition. This fact, as in adult cases, could help prevent unnecessary surgeries and irreversible organ damage.
[Clinical analysis of 9 cases of IgG4-related disease with head and neck organ involvement as the initial symptom].
Objective: To summarize the clinical features and diagnostic/management experience of diagnosis and treatment of IgG4-related disease (IgG4-RD) presenting with initial head-and-neck organ involvement. Methods: A retrospective analysis was conducted of 9 IgG4-RD patients (7 males, 2 females; aged 54-65 years, with a median of 59 years) treated in department of Otorhinolaryngology Head and Neck Surgery of the Second Affiliated Hospital of Shanxi Medical University from May 2018 to January 2025. Descriptive statistics were used to analyze involved organs, clinical manifestations, imaging findings, histopathology, therapy and outcome. Results: The initial head-and-neck involvement comprised bilateral submandibular glands in 4 cases, nasal cavity/paranasal sinuses in 2, unilateral parotid gland in 1, larynx in 1, and both nasal septum and subglottis in 1. Serum IgG4 levels were elevated in all cases, the range was 2 380-17 000 mg/L. All pathological findings fulfilled the diagnostic criteria for IgG4-RD. All patients were treated with systemic corticosteroids. During 6-80 months of follow-up, one case of relapse occurred and was controlled by increasing steroid dose and prolonging treatment course. Conclusions: IgG4-RD is rare in clinic and their presentations are highly variable due to different organs involved. The head-and-neck region is one of the common initial sites. Obtaining a pathological diagnosis is particularly critical. Glucocorticoids remain the first-line therapy for IgG4-RD. 目的： 总结以头颈部器官受累为首发症状的IgG4相关疾病（IgG4-RD）的临床特点及诊疗经验。 方法： 回顾性分析2018年5月至2025年1月山西医科大学第二医院耳鼻咽喉头颈外科收治的9例IgG4-RD患者的临床资料，其中男7例，女2例，年龄54~65岁，中位年龄59岁。采用描述性统计学方法分析其受侵器官、临床表现、影像学结果、病理特征、治疗情况及随访结果。 结果： 首发受累的头颈部器官为：双侧颌下腺4例，鼻腔-鼻窦2例，单侧腮腺1例，喉1例，鼻中隔和声门下1例。全部病例血清IgG4水平均升高（范围为2 380~17 000 mg/L）。所有病理学结果均符合IgG4-RD诊断标准。所有患者均给予糖皮质激素治疗，随访6~80个月，1例患者复发，通过增加激素剂量和延长治疗疗程获得病情控制。 结论： IgG4-RD临床罕见，其临床表现因受累器官不同而复杂多样，获得病理学诊断尤为关键，糖皮质激素是治疗IgG4-RD的一线药物。.
Clinical phenotypes and prognosis of IgG4-RD patients with allergy history: A retrospective cohort study.
There is a high prevalence of allergic conditions in patients with IgG4-related disease (IgG4-RD), paralleled by shared dysregulation in immune responses. The aim of this study was to explore the clinical features and outcomes of IgG4-RD patients with allergy history. 215 IgG4-RD patients were retrospectively enrolled and clinical features were compared between IgG4-RD patients with and without allergy history. Subsequently, 148 patients who had been followed up for ≥ 1 year were further selected to evaluate the outcomes and risk factors of relapse by conventional COX and Bayesian Cox proportional hazards models. Among IgG4-RD patients, 81 (37.7%) had allergy history. IgG4-RD patients with allergy history were found to have lower proportion of males, younger age of disease onset, higher peripheral blood eosinophil, more proliferative subtype, less fibrotic subtype, higher incidence of lacrimal gland, parotid gland, submandibular gland involvement, sinusitis and lower incidence of retroperitoneal fibrosis, pancreas, biliary tree involvement. Allergy history was correlated with a shorter relapse-free survival time (HR 95% CI: 1.067-2.604, p = 0.02). More organs involved, higher IgG4-RD responder index (IgG4-RD RI), allergy history, lacrimal gland involvement, sinusitis, retroperitoneal fibrosis and Immunosuppressants (IMs) usage were associated with relapse. IgG4-RD patients with allergy history have distinctive phenotypes and prognosis. Therefore, differentiated treatment strategies and novel medications are required for them. Key Points • IgG4-RD patients with allergy history exhibit high prevalence of allergic conditions. • Conventional and Bayesian Cox regression models were used to analyze the risk factors of relapse. • Allergy history are correlated with distinctive phenotypes and shorter relapse-free survival time in IgG4-RD.
An Ultrasound-Based Machine Learning Model for Differentiating IgG4-Related Sialadenitis and Primary Sjögren's Syndrome.
To establish and validate an interpretable machine learning (ML) model based on ultrasound (US) scoring system for differentiating immunoglobulin G4-related sialadenitis (IgG4-RS) from primary Sjögren's syndrome (pSS). A total of 263 patients with IgG4-RS or pSS were allocated to training (n = 184) and validation (n = 79) cohorts. Class imbalance was addressed using the synthetic minority oversampling technique. Optimal features were selected via least absolute shrinkage and selection operator (LASSO) regression, with different predictive models constructed using logistic regression alongside eight ML algorithms. Model performance was assessed through multiple metrics, including the area under the curve (AUC), while feature contributions were quantified using the Shapley Additive explanations (SHAP) algorithm. LASSO regression identified eight key features: sex, dry mouth, dry eyes, parotid gland enlargement, submandibular gland enlargement, parotid gland ultrasound (PGUS) scores, submandibular gland ultrasound (SMGUS) scores, and submandibular gland vascularity (SMGV) scores. Among the developed models, the light gradient boosting machine model performed best, achieving an AUC of 0.955, accuracy of 93.7%, sensitivity of 96.5%, and specificity of 86.4% in the validation cohort; decision curve analysis confirmed its superior clinical utility over others. Females, low SMGUS scores, high PGUS scores, absence of submandibular gland enlargement, and low SMGV scores were identified as top five pSS predictors using the SHAP analysis. The interpretable ML model based on US scoring system offers an accurate, non-invasive tool to differentiate IgG4-RS from pSS.
Publicações recentes
Ver todas no PubMed📚 EuropePMCmostrando 161
Inebilizumab treatment in Japanese patients with IgG4-related disease: a subgroup analysis of a randomized, double-blind, placebo-controlled, phase 3 trial (MITIGATE).
Modern rheumatologyComparison of IgG4 related disease in patients aged≤25 years (pediatric, adolescent, and young adult) and those aged≥50 years.
Reumatologia clinica[Clinical analysis of 9 cases of IgG4-related disease with head and neck organ involvement as the initial symptom].
Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgeryFeatures of IgG4-related sclerosing mesenteritis: A Chinese cohort study and literature review.
Rheumatology and immunology researchClinical phenotypes and prognosis of IgG4-RD patients with allergy history: A retrospective cohort study.
Clinical rheumatologyAn Ultrasound-Based Machine Learning Model for Differentiating IgG4-Related Sialadenitis and Primary Sjögren's Syndrome.
International dental journalUltrasonographic features and clinical management of IgG4-related submandibular gland inflammation: a case report.
Journal of ultrasoundClinicopathological Pearls and Diagnostic Pitfalls in IgG4-Related Disease: Challenging Case Series and Literature Review.
Diagnostics (Basel, Switzerland)Atypical IgG4 related hypophysitis revealed by submandibular sialadenitis: a case report.
Endocrine regulationsRNA-Seq analysis of lymphocyte-specific gene expression patterns in IgG4-related disease: comparison of submandibular glands and peripheral blood.
Modern rheumatologyChallenges in Diagnosing and Differentiating IgG4-Related Disease From Sjögren's Disease: A Case Report and Literature Review.
CureusKuttner's Tumour: A Case Series and Narrative Review on Diagnosis, Management, and Outcomes.
Journal of clinical medicineClinical characterization of malignant lymphoma mimicking IgG4-related disease.
Clinical rheumatologyIgG4‑related disease with endobronchial nodules: A case report and literature review.
Medicine internationalSymmetrical polyarthritis in IgG4-related sialadenitis: a diagnostic challenge with seronegative rheumatoid arthritis.
BMC rheumatologyClinical profile of IgG4-related disease in Japan based on the rare disease data registry.
Immunological medicinePediatric IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) with acquired hemophilia A: A case report and review of literature.
International journal of immunopathology and pharmacologyAssessing the diagnostic and therapeutic value of submandibular gland elastography in patients with immunoglobulin G4-related sialadenitis.
Modern rheumatologyIgG4-related Disease Concomitant with Diffuse Large B-cell Lymphoma.
Internal medicine (Tokyo, Japan)Submandibular gland tissue RNAseq and spatial transcriptome analyses in IgG4-related disease.
Rheumatology (Oxford, England)Toll-like receptors 2 and 4, and bacterial proteins in IgG4-related sialadenitis, other types of chronic sialadenitis and sialolithiasis.
Journal of oral microbiologyChronic Sclerosing Sialadenitis of the Submandibular Gland and its Histopathological Spectrum in the IgG4-Related Disease: a Series of 17 Cases.
Head and neck pathologyViral DNA in submandibular gland tissue with an inflammatory disorder.
Journal of oral microbiologyIgG4-Related Membranous Nephropathy After COVID-19 Vaccination: A Case Report.
Cureus[Salivary gland disorder: consider IgG4-related disease].
Nederlands tijdschrift voor tandheelkundeChronic sclerosing sialadenitis of the bilateral submandibular glands in childhood - a diagnostic dilemma.
Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologieSingle-cell RNA sequencing of submandibular gland reveals collagen type XV-positive fibroblasts as a disease-characterizing cell population of IgG4-related disease.
Arthritis research & therapyImmunoglobulin G4 (IgG4)-related Lymphadenopathy in Submandibular Space Mimicking Submandibular Malignant Tumor: A Case Report.
Current medical imagingIgG4-related disease with massive pericardial effusion diagnosed clinically using FDG-PETCT: a case report.
Frontiers in immunologyAdvancements in diagnosing IgG4-related disease of the head and neck: Navigating diagnostic pitfalls.
Seminars in diagnostic pathologyA case of IgG4-related hypophysitis maintained remission of diabetes insipidus for over 3 months after completion of steroid treatment.
Endocrinology, diabetes & metabolism case reports[A case of duodenal ulcer as prominent manifestation of IgG4-related disease].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences[Medical visit status and clinical features in patients with IgG4 related disease].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesIgG4-Related Pericarditis Diagnosed by Accumulated Pericardial Effusion.
Case reports in cardiologyA case of atypical IgG4-related disease presenting hypereosinophilia, polyneuropathy, and liver dysfunction.
Modern rheumatology case reportsExtraction of characteristic serum microRNAs and prediction of target genes in IgG4-related dacryoadenitis and sialadenitis.
Modern rheumatologyImmunoglobulin G4-related Disease with Multiple Organs Involvement Depicted on FDG PET/CT: A Case Report and Literature Review.
Current medical imagingIgG4-Related Disease Involving the Paranasal Sinus Orbit: A Case Report.
Ear, nose, & throat journal[Analysis of clinicopathological characteristics of IgG4-related sialadenitis].
Zhonghua kou qiang yi xue za zhi = Zhonghua kouqiang yixue zazhi = Chinese journal of stomatologySingle-cell transcriptome analysis profiles cellular and molecular alterations in submandibular gland and blood in IgG4-related disease.
Annals of the rheumatic diseasesAutoimmune Polyglandular Syndrome Type 3 Complicated with IgG4-related Disease.
Internal medicine (Tokyo, Japan)IgG4-RD in a Unilateral Parotid Mass: A Rare Manifestation and Review of the Literature.
CureusAuntMinnie Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography Leads to Diagnosis of Immunoglobulin G4-Related Disease.
Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, IndiaSpontaneous regression of immunoglobulin G4-related dacryoadenitis and multiple organ involvement: A case report.
MedicineDifferentiation between IgG4-related Mikulicz disease and Sjögren's syndrome: A review case report and literature review.
MedicineId4 modulates salivary gland homeostasis and its expression is downregulated in IgG4-related disease via miR-486-5p.
Biochimica et biophysica acta. Molecular cell researchImmunoglobulin G4-related Disease with Marked Eosinophilia: A Case Report and Literature Review.
Internal medicine (Tokyo, Japan)[Establishment and application of new techniques for submandibular gland preservation].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesImmunological characteristics of IgG4-related Kuttner tumors.
MedicineIgG4-related disease as multiple head and neck swellings: supported by histopathology and immunohistochemistry.
Romanian journal of ophthalmologyComplemental Diagnosis of IgG4-Related Pancreaticobiliary Diseases by Multiple Hypoechoic Lesions in the Submandibular Glands.
Journal of clinical medicineInterleukin-13 promotes cellular senescence through inducing mitochondrial dysfunction in IgG4-related sialadenitis.
International journal of oral scienceIgG4-positive plasma cells in nonspecific sialadenitis and sialolithiasis.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncCentral Diabetes Insipidus Due to IgG4-related Hypophysitis That Required over One Year to Reach the Final Diagnosis Due to Symptoms Being Masked by Sialadenitis.
Internal medicine (Tokyo, Japan)Immunoglobulin G4-related disease involving multiple systems: A case report.
World journal of clinical casesClinical Characteristics and CD4+ T Cell Subsets in IgG4-Related Disease.
Frontiers in immunologyPrediction of new organ onset in recurrent immunoglobulin G4-related disease during 10 years of follow-up.
Journal of internal medicineSevere asthma as the initial clinical manifestation of IgG4-related disease: a retrospective clinical study.
BMC pulmonary medicineOral and maxillofacial manifestations of IgG4-related disease: A clinicopathological study.
Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral PathologyIgG4-related pseudotumours: a series of 12 cases and a review of the literature.
Pathology[Studies on newly recognized chronic sialadenitis].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesUsual Interstitial Pneumonia Pattern Interstitial Lung Disease Developed in a Patient with IgG4-related Chronic Sclerosing Sialadenitis.
Internal medicine (Tokyo, Japan)Diffuse Large B-Cell Lymphoma 18 Years After Bilateral Lacrimal Gland IgG4-Related Disease: Case Report and Literature Review.
Journal of investigative medicine high impact case reportsIgG4-related sialadenitis - a rare bilateral submandibular gland enlargement: case report.
Oral and maxillofacial surgeryIgG4-related chronic sclerosing sialadenitis in a child with recurrent parotitis: a case report.
BMC pediatricsUltrasonography of IgG4-related dacryoadenitis and sialadenitis: Imaging features and clinical usefulness.
Modern rheumatologyIgG4-related tubulointerstitial nephritis.
BMJ case reportsUltrasound and Sialogram Correlates to Parotid Immunoglobulin G4-Related Disease.
Ear, nose, & throat journalSuspected IgG4-related disease of the submandibular salivary gland and periorbital soft tissue in a man with latent tuberculosis.
BMJ case reportsReticular enhancement of the submandibular gland on contrast-enhanced magnetic resonance imaging in three cases with IgG4-related chronic sclerosing sialadenitis.
The neuroradiology journalClinical features and treatment efficacy for IgG4-related thyroiditis.
Orphanet journal of rare diseasesNatural developing process of immunoglobulin G4-related sialadenitis after submandibular gland excision: a retrospective cohort study.
Clinical rheumatologyIgG4-Related Disease and the Salivary Glands: A Review of Pathophysiology, Diagnosis, and Management.
Otolaryngologic clinics of North AmericaThe role of PD-1/PD-Ls in the pathogenesis of IgG4-related disease.
Rheumatology (Oxford, England)Clinical characteristics and outcome of IgG4-related disease with hypocomplementemia: a prospective cohort study.
Arthritis research & therapyIgG4-related Dacryoadenitis and Sialadenitis with Palatal Gland Swelling.
The Bulletin of Tokyo Dental CollegeA Hard Mass of the Right Submandibular Gland in an 83-Yr-Old Lady.
Acta cytologicaNot All Küttner Tumors Are IgG4-Related Disease (IgG4-RD).
Head and neck pathologyDifferential diagnosis of immunoglobulin G4-related sialadenitis and Kimura's disease of the salivary gland: a comparative case series.
International journal of oral and maxillofacial surgeryMRI Features in Submandibular Gland Chronic Sclerosing Sialadenitis: A Report of Three Cases and Imaging Findings.
Iranian journal of otorhinolaryngologyApplication of Lip Biopsy for the Histological Diagnosis of Immunoglobulin G4-Related Disease.
Ear, nose, & throat journalSerum IgE in the clinical features and disease outcomes of IgG4-related disease: a large retrospective cohort study.
Arthritis research & therapyUltrasound-guided core needle biopsy compared with open biopsy: a new diagnostic approach to salivary gland enlargement in Sjögren's syndrome?
Rheumatology (Oxford, England)IgG4-related fibrosing mediastinitis: clinical presentation, treatment efficacy and comparison with IgG4-RD without fibrosing mediastinitis.
Clinical and experimental rheumatologySialadenitis in a patient with ulcerative colitis and autoimmune pancreatitis type 2.
Pathology, research and practicePediatric Parotid Chronic Sclerosing Sialadenitis in an African-American Female: A Rare Case and Review of the Literature.
Cureus[Raynaud phenomenon with arterial thromboses and IgG4-related disease].
La Revue de medecine interneFive Cases of IgG4-related Disease with Nasal Mucosa and Sinus Involvement.
Internal medicine (Tokyo, Japan)A comparison of the features of fluorine-18 fluorodeoxyglucose-positron emission tomography (FDG-PET) between IgG4-related disease with bilateral hilar lymphadenopathy and sarcoidosis.
Nagoya journal of medical scienceIgG4-related Bronchial Gland Inflammation Proved by Transbronchial Cryobiopsy.
American journal of respiratory and critical care medicineIgG4-related pleural disease with aortitis and submandibular glands involvement successfully treated with corticosteroid: case-based review.
Rheumatology internationalDoes a Subset of Localized Chronic Fibrosing Vasculitis Represent Cutaneous Manifestation of IgG4-Related Disease/a Histologic Pattern of IgG4-Related Skin Disease? A Reappraisal of an Enigmatic Pathologic Entity.
The American Journal of dermatopathologyCytotoxic Tph-like cells are involved in persistent tissue damage in IgG4-related disease.
Modern rheumatologyMalignancies in Patients with IgG4-Related Diseases in Head and Neck Regions.
The Tohoku journal of experimental medicine[Application of ultrasonography scoring system in the assessment of IgG4-related sialadenitis].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences[Analysis of the clinical features and misdiagnosis reasons of 17 patients misdiagnosed with IgG4-related disease].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesClinical Utility of Diffusion-weighted Whole-body Magnetic Resonance Imaging with Background Body Signal Suppression for Assessing and Monitoring IgG4-related Disease.
Internal medicine (Tokyo, Japan)Sclerosing sialadenitis of the submandibular gland is rarely an immunoglobulin G4-related disease in the Finnish population.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncAn unusual presentation of chronic sclerosing sialadenitis of submandibular gland (Kuttner's tumour).
BMJ case reportsTNF-α Suppresses Autophagic Flux in Acinar Cells in IgG4-Related Sialadenitis.
Journal of dental researchSuccessful treatment with rituximab of IgG4-related disease coexisting with adult-onset asthma and periocular xanthogranuloma.
Rheumatology internationalClinical and biochemical characteristics of patients having general symptoms with increased serum IgG4.
Modern rheumatologyActivated M2 Macrophages Contribute to the Pathogenesis of IgG4-Related Disease via Toll-like Receptor 7/Interleukin-33 Signaling.
Arthritis & rheumatology (Hoboken, N.J.)Cutaneous and systemic IgG4-related disease: a review for dermatologists.
Dermatology online journal[A case of hypertrophic pachymeningitis associated with probable sarcoidosis with increased serum IgG4].
Rinsho shinkeigaku = Clinical neurologyRenal Pelvis Involvement of Immunoglobulin G4-Related Disease Mimicking Malignancy on 18F-FDG PET/CT.
Clinical nuclear medicineIgG4-Related Disease Presenting as Cervical Lymphadenopathy.
Journal of medical ultrasound[Clinicopathological characteristics and diagnosis of IgG4related sialadenitis].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesClinical features and mechanistic insights regarding IgG4-related dacryoadenitis and sialoadenitis: a review.
International journal of oral and maxillofacial surgeryUp-regulation of activation-induced cytidine deaminase and its strong expression in extra-germinal centres in IgG4-related disease.
Scientific reportsAbundant a proliferation-inducing ligand (APRIL)-producing macrophages contribute to plasma cell accumulation in immunoglobulin G4-related disease.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationMechanism of fibrogenesis in submandibular glands in patients with IgG4-RD.
Journal of molecular histologyClinical characteristics of a concurrent condition of IgG4-RD and Castleman's disease.
Clinical rheumatologyIgG4-related disease with skin, submaxillary and pulmonary involvement.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGThoracic Paravertebral Mass as an Infrequent Manifestation of IgG4-Related Disease.
Case reports in rheumatologyGlucocorticoid receptor expression in resident and hematopoietic cells in IgG4-related disease.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncTreatment of immunoglobulin G4-related sialadenitis: outcomes of glucocorticoid therapy combined with steroid-sparing agents.
Arthritis research & therapyIgG4-related cerebral pseudotumor with perineural spreading along branches of the trigeminal nerves causing compressive optic neuropathy: A case report.
MedicineIgG4-related Hypophysitis with Subtle Hypopituitarism in an Elderly Diabetic Patient: Is Treatment or Observation Preferable?
Internal medicine (Tokyo, Japan)Cutting Edge: A Critical Role of Lesional T Follicular Helper Cells in the Pathogenesis of IgG4-Related Disease.
Journal of immunology (Baltimore, Md. : 1950)Rosai-Dorfman Disease of the Lung Overlapping with IgG4-related Disease: The Difficulty in Its Differential Diagnosis.
Internal medicine (Tokyo, Japan)IgG4-related disease in an adolescent with radiologic-pathologic correlation.
Radiology case reports[Case of EGPA and Eosinophilic Chronic Rhinosinusitis Concomitant with IgG4 Related Disease].
Nihon Jibiinkoka Gakkai kaiho18F-FDG PET/CT Features of Chronic Sclerosing Sialadenitis Presenting as Localized IgG4-Related Disease.
Clinical nuclear medicine[Non-Hodgkin's lymphoma mimicking Mikulicz disease: a case report].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesDifferential diagnosis of IgG4-related sialadenitis, primary Sjögren syndrome, and chronic obstructive submandibular sialadenitis.
The British journal of oral & maxillofacial surgeryA case of IgG4-related tubulointerstitial nephritis and membranous glomerulonephritis during the clinical course of gastric cancer: Imaging features of IgG4-related kidney disease.
Modern rheumatologyKüttner Tumor: IgG4-Related Disease of the Submandibular Gland.
Head and neck pathologyGallium SPECT/CT in evaluation of IgG4-related disease: A case report and literature review.
MedicineIgG4-related Disease with Bone Marrow Involvement.
Internal medicine (Tokyo, Japan)Lesional CD4+ IFN-γ+ cytotoxic T lymphocytes in IgG4-related dacryoadenitis and sialoadenitis.
Annals of the rheumatic diseasesIgG4-related disease involving vital organs diagnosed with lip biopsy: A case report and literature review.
MedicineInvasive cervical cancer accompanied by IgG4-related disease.
The journal of obstetrics and gynaecology researchSporadic occurrence of non-diagnosed IgG4-related disease in lymphoma patients with a previous Sjögren's syndrome diagnosis.
Acta oncologica (Stockholm, Sweden)Overlap of IgG4-related Disease and Primary Biliary Cirrhosis Complicated with Autoimmune Thrombocytopenia.
Internal medicine (Tokyo, Japan)IgG4-Related Sialoadenitis with a Skin Lesion and Multiple Mononeuropathies Suggesting Coexistent Cryoglobulinemic Vasculitis.
Internal medicine (Tokyo, Japan)Clinicopathological analysis of salivary gland tissue from patients with IgG4-related disease.
Acta oto-laryngologicaClonal expansion of CD4(+) cytotoxic T lymphocytes in patients with IgG4-related disease.
The Journal of allergy and clinical immunologyInterferon-gamma increased epithelial barrier function via upregulating claudin-7 expression in human submandibular gland duct epithelium.
Journal of molecular histologyUtility of FDG PET/CT for Differential Diagnosis of Patients Clinically Suspected of IgG4-Related Disease.
Clinical nuclear medicineDNA Microarray Analysis of Submandibular Glands in IgG4-Related Disease Indicates a Role for MARCO and Other Innate Immune-Related Proteins.
MedicineCT features and pathologic characteristics of IgG4-related systemic disease of submandibular gland.
International journal of clinical and experimental pathologyIgG4-related disease in asbestos-related pleural disease.
Respirology case reports[IgG4-related salivary gland lesions].
Terapevticheskii arkhivIgG4-related disease in the head and neck region: report of two cases and review of the literature.
Polish journal of pathology : official journal of the Polish Society of PathologistsDiffuse large B-cell lymphoma of the lacrimal sac arising from a patient with IgG4-related disease.
Modern rheumatologyIgG4-Related Disease: Treatment and Insight Into Pathophysiology: Video of the Lecture by John Stone MD, MPH.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesIgG4-related disease: A relatively new concept for clinicians.
European journal of internal medicineChronic sclerosing sialadenitis of the submandibular gland: an entity of IgG4-related sclerosing disease.
International journal of clinical and experimental pathologyImmunoglobulin G4-related autoimmune pancreatitis and sialadenitis: A case report.
World journal of gastroenterologyEffectiveness of imaging modalities for screening IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) and for differentiating it from Sjögren's syndrome (SS), with an emphasis on sonography.
Arthritis research & therapyHypocomplementemic urticarial vasculitis arising in a patient with immunoglobulin G4-related disease.
International journal of dermatologyA case of mantle cell lymphoma presenting as IgG4-related dacryoadenitis and sialoadenitis, so-called Mikulicz's disease.
World journal of surgical oncologyChronic Sclerosing Sialadenitis of the Submandibular Gland as the Initial Symptom of IgG4-Related Disease: A Case Report.
The Tohoku journal of experimental medicineIgG4-related disease in idiopathic sclerosing orbital inflammation.
The British journal of ophthalmologySonographic evaluation of the treatment response in patients with immunoglobulin G4-related disease of the submandibular glands.
Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in MedicineA case of marginal zone B cell lymphoma mimicking IgG4-related dacryoadenitis and sialoadenitis.
World journal of surgical oncologyImmunoglobulin G4 related chronic sclerosing sialadenitis.
The Journal of laryngology and otologyA case of adenoid cystic carcinoma associated with IgG4-related disease.
International journal of surgery case reports[The value of ultrasound in diagnosis of IgG4-related sialadenitis].
Shanghai kou qiang yi xue = Shanghai journal of stomatologyInterleukin 13-positive mast cells are increased in immunoglobulin G4-related sialadenitis.
Scientific reportsAssociações
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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.
- Inebilizumab treatment in Japanese patients with IgG4-related disease: a subgroup analysis of a randomized, double-blind, placebo-controlled, phase 3 trial (MITIGATE).
- Comparison of IgG4 related disease in patients aged≤25 years (pediatric, adolescent, and young adult) and those aged≥50 years.
- [Clinical analysis of 9 cases of IgG4-related disease with head and neck organ involvement as the initial symptom].Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery· 2026· PMID 41588294mais citado
- Clinical phenotypes and prognosis of IgG4-RD patients with allergy history: A retrospective cohort study.
- An Ultrasound-Based Machine Learning Model for Differentiating IgG4-Related Sialadenitis and Primary Sjögren's Syndrome.
- A Narrative Review of Salivary Gland Extracellular Matrix and Sjögren's Syndrome: Research Status and Future Prospects.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:449432(Orphanet)
- MONDO:0018674(MONDO)
- GARD:21884(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55788252(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.
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