The Positive Predictive Value of a Very High Serum IgG4 Concentration for the Diagnosis of IgG4-Related Disease.
J Rheumatol
; 50(3): 408-412, 2023 03.
Article
em En
| MEDLINE
| ID: mdl-36319016
ABSTRACT
OBJECTIVE:
Serum 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.METHODS:
The 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.RESULTS:
IgG4 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.CONCLUSION:
The 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.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Doenças Autoimunes
/
Doença Relacionada a Imunoglobulina G4
/
Neoplasias
Tipo de estudo:
Diagnostic_studies
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Prognostic_studies
/
Risk_factors_studies
Limite:
Aged
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Female
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Humans
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Male
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article