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Inicio Medicina Clínica (English Edition) IgG4-related disease: Effectiveness evaluation through Umehara–Okazaki 2011 an...
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Vol. 163. Issue 5.
Pages 217-223 (September 2024)
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Vol. 163. Issue 5.
Pages 217-223 (September 2024)
Original article
IgG4-related disease: Effectiveness evaluation through Umehara–Okazaki 2011 and ACR/EULAR 2019 diagnostic criteria
Enfermedad relacionada con IgG4: evaluación de la eficacia mediante los criterios diagnósticos de Umehara-Okazaki 2011 y ACR/EULAR 2019
Pablo Martínez Calabuiga,b,
Corresponding author
pablomc96@hotmail.es

Corresponding author.
, Jorge Juan Fragío Gila,b, Roxana González Mazaríoa,b, Fernando López Gutiérrezc, Javier Loricera Garcíac,d, Ricardo Blanco Alonsoc,d, Cristina Campos Fernándeza,b
a Servicio de Reumatología y Metabolismo Óseo, Consorcio Hospital General Universitario, Valencia, Spain
b Escuela de Doctorado, Universidad Católica de Valencia San Vicente Mártir (UCV), Valencia, Spain
c Servicio de Reumatología, Hospital Marqués de Valdecilla, Santander, Spain
d IDIVAL, Spain
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Figures (2)
Tables (4)
Table 1. The Umehara–Okazaki 2011 comprehensive diagnostic criteria for IgG4-related disease.10
Table 2. Adapted criteria from The 2019 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for IgG4-related disease (IgG4-RD).3
Table 3. Main characteristics of the IgG4-related disease clinical diagnosis and Highly diagnosis of IgG4-related disease.
Table 4. Comparison of the Umehara–Okazaki 201110 and ACR/EULAR 20193 criteria.
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Abstract
Background

IgG4-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory condition with an unclear etiology and pathophysiology, potentially affecting multiple organs. It presents with common clinical, radiological, and serological characteristics. This study aims to compare the latest two IgG4-RD classification and diagnostic criteria: Umehara–Okazaki 2011 and ACR/EULAR 2019.

Material and methods

In a retrospective cross-sectional study conducted across two centers from January 2010 to July 2023, we included patients suspected of having IgG4-RD from various hospital departments. Patients finally diagnosed with other pathologies were excluded. The remaining suspected IgG4-RD cases were evaluated using both Umehara–Okazaki 2011 and ACR/EULAR 2019 criteria.

Results

Out of 34 patients with a clinical diagnosis of IgG4-RD, the Umehara–Okazaki 2011 classified 20 patients: 5 as definitive, 7 as probable, and 8 as possible cases. Applying the ACR/EULAR 2019 criteria to the same cohort resulted in the diagnosis of 9 patients. Notably, retroperitoneal fibrosis and aortitis were the most prevalent form of presentation, accounting for 25% and 22.2% of cases classified under the 2011 and 2019 criteria, respectively.

Discussion

The more recent and stringent ACR/EULAR 2019 criteria focus on histopathology, various forms of presentation, and analytical data, allow for a more accurate classification of patients.

Keywords:
IgG4-RD
Classification criteria
Resumen
Fundamento

La enfermedad relacionada con la IgG4 (ER-IgG4) es una enfermedad fibroinflamatoria inmunomediada sistémica rara, con una etiología y fisiopatología desconocida, que puede afectar a múltiples órganos. Se presenta con características clínicas, radiológicas y serológicas comunes. Este estudio tiene como objetivo comparar los dos últimos criterios de clasificación y diagnóstico de IgG4-RD: Umehara-Okazaki 2011 y ACR/EULAR 2019.

Material y método

Estudio transversal retrospectivo realizado en dos centros desde enero de 2010 hasta julio de 2023, donde incluimos pacientes sospechosos de tener ER-IgG4 de varios departamentos hospitalarios. Se excluyeron los pacientes que finalmente fueron diagnosticados de otras patologías. Los casos sospechosos de ER-IgG4 restantes fueron evaluados utilizando tanto los criterios de Umehara-Okazaki 2011 como los de ACR/EULAR 2019.

Resultados

De los 34 pacientes con un diagnóstico clínico de ER-IgG4, los criterios de Umehara-Okazaki 2011 clasificaban a 20 pacientes: 5 como casos definitivos, 7 como probables y 8 como posibles. La aplicación de los criterios ACR/EULAR 2019 a la misma cohorte resultó en el diagnóstico de 9 pacientes. La fibrosis retroperitoneal y la aortitis fueron las formas más prevalentes de presentación, representando el 25% y el 22.2% de los casos clasificados bajo los criterios de 2011 y 2019, respectivamente.

Conclusiones

Los recientes criterios ACR/EULAR 2019, que se centran en la histopatología, diversas formas de presentación y datos analíticos, permiten una clasificación más precisa de los pacientes.

Palabras clave:
ER-IgG4
Criterios de clasificación

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