ArticleThe Journal of rheumatology2023
The Positive Predictive Value of a Very High Serum IgG4 Concentration for the Diagnosis of IgG4-Related Disease.
Article in The Journal of rheumatology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 13 citations in OpenAlex.
- IgG4-related inflammatory pseudotumor presenting with refractory seizures: a diagnostic pitfall.Arquivos de neuro-psiquiatria · 2026Article
- IgG4-Related Hepatic Inflammatory Pseudotumor With Extensive Portal Vein Thrombosis Mimicking Hepatocellular Carcinoma.ACG case reports journal · 2026Article
- IgG4-related disease as an uncommon cause of myocarditis: a case report.European heart journal. Case reports · 2026Article
- Development of Nephritis and Abnormal Lung Shadow in a Patient with IgG4-related Hypertrophic Pachymeningitis with MPO-ANCA Positivity after Unplanned Interruption of Maintenance Glucocorticoid Therapy.Internal medicine (Tokyo, Japan) · 2026Article
- Toward audit-ready care in IgG4-related disease: redefining relapse, damage, and access in the biologic era.Rheumatology international · 2026Article
- IgG4 elevation dilemma at the crossroads of helminth infection and IgG4-related disease: insights from a case report of pediatric paragonimiasis with persistent pericardial effusion.Translational pediatrics · 2025Article
- IgG4-Related Disease: A Review of Persistent Challenges in the Pathogenesis, Diagnosis, and Approaches to Treatment.Medical science monitor : international medical journal of experimental and clinical research · 2025Review
- IgG4-Related Disease: Current and Future Insights into Pathological Diagnosis.International journal of molecular sciences · 2025Review
- Current and future advances in practice: IgG4-related disease.Rheumatology advances in practice · 2024Review
- Reshaping the Concept of Riedel's Thyroiditis into the Larger Frame of IgG4-Related Disease (Spectrum of IgG4-Related Thyroid Disease).Biomedicines · 2023Review
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
Abstract
objectiveSerum IgG4 concentrations are used to evaluate a diagnosis of IgG4-related disease (IgG4-RD), but the positive predictive value (PPV) of a very high IgG4 level is uncertain. This study evaluated the PPV of a very high IgG4 concentration for diagnosing IgG4-RD.
methodsThe data warehouses of 2 large academic healthcare systems were queried for IgG4 concentration test results. Cases with serum IgG4 concentrations > 5× the upper limit of normal (ULN) were included. Cases of IgG4-RD were determined using the American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) classification criteria. The PPV for IgG4-RD of an IgG4 concentration > 5× ULN was estimated. Other conditions associated with very high IgG4 concentrations and specific features of IgG4-RD cases were characterized.
resultsIgG4 concentrations were available in 32,206 cases. Of these, 3039 (9.4%) had elevated IgG4 concentrations, and a final cohort of 191 (0.6%) cases had IgG4 concentrations > 5× ULN (median age 66 yrs, 72% male). The PPV of an IgG4 concentration > 5× ULN for a diagnosis of IgG4-RD was 75.4% (95% CI 68.7-81.3). In the remaining cases, elevated IgG4 concentrations were observed among patients with malignancies, autoimmune diseases, and infections.
conclusionThe majority of cases with serum IgG4 concentrations > 5× ULN in this study had IgG4-RD. These data support the high weight placed on very high serum IgG4 concentrations in the ACR/EULAR classification criteria. However, 25% of cases with very high IgG4 concentrations had an alternative diagnosis, underscoring the importance of considering the broad differential of etiologies associated with an elevated IgG4 concentration when evaluating a patient.
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