Imaging modalities in the diagnosis and monitoring of IgG4-Related disease: Utilization patterns and clinical implications
DOI:
https://doi.org/10.5281/zenodo.15069860Keywords:
Immunoglobulin G4-related disease, imaging, computed tomography, magnetic resonance imaging, positron emission tomographyAbstract
Objective: Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibroinflammatory disorder affecting multiple organ systems, often requiring imaging for diagnosis and monitoring. Despite its critical role, an optimal imaging strategy, including modality preference and follow-up intervals, remains undefined. This study evaluates the utilization patterns of different imaging modalities in IgG4-RD.
Materials and methods: This retrospective, single-center study included 18 patients diagnosed with IgG4-RD according to ACR/EULAR criteria between May 2020 and December 2024. Clinical, laboratory, histopathological, and imaging data were collected. Computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET-CT) were analyzed for diagnostic and follow-up trends. Patients were stratified based on the presence of acute kidney injury (AKI) at diagnosis.
Results: At diagnosis, CT (83.3%) was the most frequently used modality, followed by PET-CT (61.1%) and MRI (27.8%). Combined imaging was performed in 66.6% of cases initially but declined during follow-up. The choice of the first follow-up imaging differed between patients with and without AKI (p=0.072). Notably, follow-up imaging intervals were significantly shorter in AKI patients (30.5 vs. 49 months, p=0.049). Over time, PET-CT use decreased, while MRI became the preferred modality. Despite these variations, no standardized follow-up approach was observed.
Conclusion: Our findings indicate a shift from multimodal to single-modality imaging during follow-up, with variations based on AKI status. The lack of a standardized imaging protocol indicates the need for consensus guidelines to enhance disease monitoring and optimize patient outcomes.
References
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-51.
Jayachamarajapura Onkaramurthy N, Suresh SC, Theetha Kariyanna P, Jayarangaiah A, Prakash G, Raju B. IgG4 related disease and aortitis: an up-to-date review. Scand J Rheumatol. 2023;52(3):306-16.
Wallace ZS, Naden RP, Chari S, Choi H, Della-Torre E, Dicaire JF, et al. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol. 2020;72(1):7-19.
Zhang W, Stone JH. Management of IgG4-related disease. Lancet Rheumatol. 2019;1(1):e55-e65.
Sanchez-Oro R, Alonso-Munoz EM, Marti Romero L. Review of IgG4-related disease. Gastroenterol Hepatol. 2019;42(10):638-47.
Naik M, Hesni S, Tamimi A, Hameed M, Tomlinson J, Poo S, et al. Imaging manifestations of IgG4-related disease. Clin Radiol. 2023;78(8):555-64.
Kawano M, Saeki T. IgG4-related kidney disease--an update. Curr Opin Nephrol Hypertens. 2015;24(2):193-201.
Melenotte C, Seguier J, Ebbo M, Kaphan E, Bernit E, Saillier L, et al. Clinical presentation, treatment and outcome of IgG4-related pachymeningitis: From a national case registry and literature review. Semin Arthritis Rheum. 2019;49(3):430-7.
Ren H, Mori N, Sato S, Mugikura S, Masamune A, Takase K. American College of Rheumatology and the European League Against Rheumatism classification criteria for IgG4-related disease: an update for radiologists. Jpn J Radiol. 2022;40(9):876-93.
Negoto S, Tahara N, Nohara Y, Otsuka H, Tayama E. Utility of non-contrast MRI and FDG-PET/CT to assess inflammatory activity in IgG4-related abdominal aortic aneurysm. Eur Heart J Cardiovasc Imaging. 2024.
Takahashi H, Yamashita H, Morooka M, Kubota K, Takahashi Y, Kaneko H, et al. The utility of FDG-PET/CT and other imaging techniques in the evaluation of IgG4-related disease. Joint Bone Spine. 2014;81(4):331-6.
Nakatani K, Nakamoto Y, Togashi K. Utility of FDG PET/CT in IgG4-related systemic disease. Clin Radiol. 2012;67(4):297-305.
Cortes-Romera M, Sabate-Llobera A, Gamez-Cenzano C, Torras-Ambros J. [Usefulness of PET/CT in IgG4-related disease]. Med Clin (Barc). 2015;145(7):321-2.
Pucar D, Hinchcliff M. FDG PET vascular imaging in IgG4-RD: Potential and challenges. J Nucl Cardiol. 2022;29(6):2934-7.
Dai T, He YQ. CT and MRI features of multi-organ involvement in IgG4-related disease. Diagn Interv Imaging. 2023;104(5):261-2.
Zhang J, Chen H, Ma Y, Xiao Y, Niu N, Lin W, et al. Characterizing IgG4-related disease with (1)(8)F-FDG PET/CT: a prospective cohort study. Eur J Nucl Med Mol Imaging. 2014;41(8):1624-34.
Zhao Z, Wang Y, Guan Z, Jin J, Huang F, Zhu J. Utility of FDG-PET/CT in the diagnosis of IgG4-related diseases. Clin Exp Rheumatol. 2016;34(1):119-25.
Zheng Y, Elsayes KM, Waranch C, Abdelaziz A, Menias CO, Sandrasegaran K, et al. IgG4-related disease in the abdomen and pelvis: atypical findings, pitfalls, and mimics. Abdom Radiol (NY). 2020;45(8):2485-99.
Nikiphorou E, Galloway J, Fragoulis GE. Overview of IgG4-related aortitis and periaortitis. A decade since their first description. Autoimmun Rev. 2020;19(12):102694.
Fragoulis GE, Evangelatos G, Tektonidou MG. Vasculitis beyond aortitis in IgG4-related disease (IgG4-RD): case report and review of the literature. Clin Rheumatol. 2021;40(3):1167-73.
Lecler A, Sene T. MRI and ultrasonography are useful tools for a non-invasive diagnosis of IgG4-related disease. Ann Rheum Dis. 2022;81(3):e51.
Xu VYY, Bell M, Zahirieh A, Hsiao J, Higgins K, Ghorab Z, et al. A Challenging Diagnosis of IgG4-Related Disease When Understanding Limitations of Laboratory Testing Was Pivotal. Case Rep Med. 2017;2017:8748696.
Jolfayi AG, Salmanipour A, Heshmat-Ghahdarijani K, Meshgi S, Dastmardi M, Salehabadi G, et al. Imaging Findings in Cardiovascular Involvements of IgG4 Related Disease: A Systematic Review Study. Rheumatology (Oxford). 2025;64(3):943-51.
Czerniak S, Rao A, Mathur M. Multimodality pictorial review of IgG4-related disease in the abdomen and pelvis. Abdom Radiol (NY). 2023;48(10):3147-61.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 Journal of Clinical Trials and Experimental Investigations

This work is licensed under a Creative Commons Attribution 4.0 International License.
The journal is licensed under a Attribution4.0 International (CC BY 4.0).