ArticleArthritis & rheumatology (Hoboken, N.J.)2015
IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients.
Article in Arthritis & rheumatology (Hoboken, N.J.), 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 302 papers, 5 of them syntheses that pooled it.
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Who cites it
302 citing papers in PubMed, 5 syntheses or guidelines pooled it, 644 citations in OpenAlex.
- Characterization of the neuromuscular manifestations in patients with IgG4-related disease: a systematic review.Immunologic research · 2025Pooled it
- Clinical characteristics and multimodal imaging insights of coronary involvement in immunoglobulin G4-related disease.Frontiers in immunology · 2025Pooled it
- Immunoglobulin G4-related coronary periarteritis: a systematic literature review with a case series.Clinical rheumatology · 2022Pooled it
- IgG4-related disease with multiorgan involvement: a case-based review.Rheumatology international · 2021Pooled it
- IgG4-Related Disease of the Skull and Skull Base-A Systematic Review and Report of Two Cases.World neurosurgery · 2021Pooled it
- An atypical case with recurrent episodes of pancreatitis: is eosinophilic pancreatitis a diagnosis or a mimic in the spectrum of IgG4-related diseases? A case-based review : Eosinophilic pancreatitis or IGG4-releated disease.Clinical journal of gastroenterology · 2026Article
- Estimated prevalence and distribution of causes of immune-mediated hypertrophic pachymeningitis in São Paulo, Brazil.Clinical rheumatology · 2026Article
- IgG4-related orbital disease - An update.Indian journal of ophthalmology · 2026Review
- Giant paratesticular immunoglobulin G4-related disease mimicking malignancy: A case report.Urology case reports · 2026Article
- Identification of Discriminatory Factors and Construction of a Nomogram for Differentiating IgG4-RD and DLBCL.Immunity, inflammation and disease · 2026Article
- Inflammatory Aortopathies in Rheumatic Diseases: A State-of-the-Art Review.Diagnostics (Basel, Switzerland) · 2026Review
- Immunoglobulin G4 sclerosing disease of the spine in a young patient: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Clinical characteristics of IgG4-related disease patients with different serum IgG4 levels: A single-center retrospective study.The Journal of international medical research · 2026Observational
- Coronary periarteritis as a rare cause of angina in a runner: hybrid PET/CT findings.European journal of nuclear medicine and molecular imaging · 2026Article
- IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis: An Update on Diagnosis and Treatment.Kidney international reports · 2026Review
- IgG4-related kidney disease presenting as severe renal failure: Diagnostic challenges of a multimodality imaging approach.Radiology case reports · 2026Article
- Article
- Proptosis Revealing a Neurological Form of IgG4-Related Disease: A Case Report and Literature Review.Cureus · 2026Article
- Isolated pulmonary and renal IgG4-related disease: a rare dual-organ presentation diagnosed via video-assisted thoracoscopic surgery.BMJ case reports · 2026Article
- Immunoglobulin G4-related Disease with Solitary Nasopharynx Involvement Mimicking Nasopharyngeal Tumor.Internal medicine (Tokyo, Japan) · 2026Article
242 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
Abstract
objectiveIgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect nearly any organ. Prior studies have focused on individual cases of IgG4-RD or small case series. This study was undertaken to report detailed clinical and laboratory findings in a larger group of patients with IgG4-RD whose diagnosis was established by strict clinicopathologic correlation.
methodsThe baseline features of 125 patients with biopsy-proven IgG4-RD were reviewed. The diagnosis was confirmed by pathologists' review, based on consensus diagnostic criteria and correlation with clinicopathologic features. Disease activity and damage were assessed using the IgG4-RD Responder Index (RI). Flow cytometry was used to assess levels of circulating plasmablasts.
resultsOf the 125 patients, 107 had active disease and 86 were not receiving treatment for IgG4-RD. Only 51% of the patients with active disease had elevated serum IgG4 concentrations. However, patients with active disease and elevated serum IgG4 concentrations were older, had a higher IgG4-RD RI score, a greater number of organs involved, lower complement levels, higher absolute eosinophil counts, and higher IgE levels compared to those with active disease but normal serum IgG4 concentrations (P < 0.01 for all comparisons). The correlation between IgG4+ plasmablast levels and the IgG4-RD RI of disease activity (Spearman's ρ = 0.45, P = 0.003) was stronger than the correlation between total plasmablast levels and the IgG4-RD RI. Seventy-six (61%) of the patients were male, but no significant differences according to sex were observed with regard to disease severity, organ involvement, or serum IgG4 concentrations. Treatment with glucocorticoids failed to produce sustained remission in 77% of patients.
conclusionNearly 50% of this patient cohort with biopsy-proven, clinically active IgG4-RD had normal serum IgG4 concentrations. Elevations in the serum IgG4 concentration appeared to identify a subset of patients with a more severe disease phenotype. In addition, the levels of IgG4+ plasmablasts correlated well with the extent of disease activity.
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