ACR Meeting Abstracts

ACR Meeting Abstracts

  • Meetings
    • ACR Convergence 2024
    • ACR Convergence 2023
    • 2023 ACR/ARP PRSYM
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • ACR Meetings

Abstract Number: 1943

IgG4-related Disease: 2010-2022 Case Review and Comparative Evaluation of Diagnostic Criteria

Pablo Martínez Calabuig1, Jorge Juan Fragio1, Roxana Gonzalez Mazarío2, Amalia Rueda Cid3, Mireia Lucía Sanmartín Martínez1, Laura Salvador Maicas1, Laura Abenza Barberá1, María Cristina Sabater Abad1, Antonio Sierra Rivera1, Inmaculada Castelló Miralles1, Juan José Lerma Garrido1, Clara Molina Almela3, Isabel Balaguer Trull1 and Cristina Campos Fernández1, 1Hospital General Universitario Valencia, Valencia, Spain, 2Hospital de Sagunto, Valencia, Spain, 3Hospital General de Valencia, Valencia, Spain

Meeting: ACR Convergence 2023

Keywords: American College of Rheumatology Criteria, classification criteria, IgG4 Related Disease

  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print
Session Information

Date: Tuesday, November 14, 2023

Title: (1913–1944) Miscellaneous Rheumatic & Inflammatory Diseases Poster III

Session Type: Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: IgG4 immunoglobulin-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory process with an unclear etiology and pathophysiology with the capacity of affecting multiple organs. It exhibits common pathophysiological, serological, and clinical characteristics.
Objectives: To describe clinical presentation, evolution, and treatment heterogeneity among a series of IgG4-RD cases and to compare the accuracy of the last two most recent IgG4-RD classification and diagnostic criteria.

Methods: A single-center retrospective cross-sectional study was conducted, encompassing patients with a suspected diagnosis of IgG4-RD from various hospital departments (Rheumatology, Digestive Medicine, Internal Medicine and Pneumology). The study period lasted from January 2010 to August 2022. Exclusion criteria were applied to patients who were ultimately diagnosed with other pathologies, and for those patients who remained with a suspected diagnosis of IgG4-RD, the Umehara-Okazaki 2011 and ACR/EULAR 2019 criteria were subsequently employed.

Results: A total of 182 patients with elevated IgG4 and/or a suspected diagnosis of IgG4-RD were initially collected. After applying the exclusion criteria, 22 potential cases of IgG4-RD were described (Table 1). The Umehara and Okazaki 2011 diagnostic criteria were applied, resulting in the classification of 13 patients (mean age :60 years; 57% women); with 5 being classified as definitive disease, 3 as probable disease, and 5 as possible disease. One death was recorded in this group, which was related to heart failure. Furthermore, the ACR/EULAR 2019 classification criteria were applied to the same patient cohort (Table 1), resulting in the diagnosis of 7 patients (mean age:57 years; 71% women) with a mean follow-up of 5.3 years. In this group, 85.71% of patients had elevated IgG4 levels (mean: 176.3 mg/dL). Retroperitoneal fibrosis and aortitis were the most prevalent form of presentation for both groups (2011 and 2019 criteria) accounting for 38.5% and 28.6% respectively. Initially, 85.71% of the patients finally diagnosed with IgG4-RD received corticosteroids followed by other immunosuppressants such as Azathioprine, Methotrexate, Rituximab, surgical treatment, or maintenance doses of corticosteroids.

Conclusion: IgG4-RD is a recently recognized entity that exhibits significant heterogeneity in clinical, analytical, and histopathological presentation. Differences arise when utilizing different diagnostic criteria.

The most recent and stringent ACR/EULAR 2019 classification criteria allow for a more accurate classification of patients by emphasizing histopathology, different forms of presentation, and analytical data. Therefore, on many occasions, a multidisciplinary approach is often necessary.

Supporting image 1

Table 1. High diagnostic IgG4-RD group, Umehara and Okazaki 2011 and ACR/EULAR 2019 IgG4-RD criteria.

Supporting image 2

Image 1. Diagnostic sequence used with IgG4-RD patients.


Disclosures: P. Martínez Calabuig: None; J. Fragio: None; R. Gonzalez Mazarío: None; A. Rueda Cid: None; M. Sanmartín Martínez: None; L. Salvador Maicas: None; L. Abenza Barberá: None; M. Sabater Abad: None; A. Sierra Rivera: None; I. Castelló Miralles: None; J. Lerma Garrido: None; C. Molina Almela: None; I. Balaguer Trull: None; C. Campos Fernández: None.

To cite this abstract in AMA style:

Martínez Calabuig P, Fragio J, Gonzalez Mazarío R, Rueda Cid A, Sanmartín Martínez M, Salvador Maicas L, Abenza Barberá L, Sabater Abad M, Sierra Rivera A, Castelló Miralles I, Lerma Garrido J, Molina Almela C, Balaguer Trull I, Campos Fernández C. IgG4-related Disease: 2010-2022 Case Review and Comparative Evaluation of Diagnostic Criteria [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/igg4-related-disease-2010-2022-case-review-and-comparative-evaluation-of-diagnostic-criteria/. Accessed .
  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print

« Back to ACR Convergence 2023

ACR Meeting Abstracts - https://acrabstracts.org/abstract/igg4-related-disease-2010-2022-case-review-and-comparative-evaluation-of-diagnostic-criteria/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].

Wiley

  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences

© Copyright 2025 American College of Rheumatology