IgG4-related disease: an update on pathology and diagnostic criteria with a focus on salivary gland manifestations
Czarnywojtek A, Agaimy A, Pietrończyk K, Nixon IJ, Vander Poorten V, Mäkitie AA, Zafereo M, Florek E, Sawicka-Gutaj N, Ruchała M, Ferlito A (2024)
Publication Type: Journal article, Review article
Publication year: 2024
Journal
DOI: 10.1007/s00428-024-03757-0
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ disorder characterized by a highly variable clinical presentation depending on the affected organ/s, extent of tumefactive fibroinflammatory lesions, and associated functional impairment. The disease pursues a chronic, relapsing, often asymptomatic course and hence may pose a significant diagnostic challenge. Diagnostic delay can lead to progressive fibrosis and irreversible organ damage resulting into significant morbidity and even mortality. Given its broad clinical spectrum, physicians of all specialties may be the first clinicians facing this diagnostic challenge. Outside the pancreatobiliary system, the head and neck represents the major site of IgG4-RD with variable organ-specific diffuse or mass-forming lesions. In up to 75% of cases, elevated serum IgG4 levels are observed, but this figure possibly underestimates the fraction of seronegative cases, as the disease manifestations may present metachronously with significant intervals. Together with negative serology, this can lead to misdiagnosis of seronegative cases. A standardized nomenclature and diagnostic criteria for IgG4-RD were established in 2012 and revised in 2020 facilitating scientific research and expanding the range of diseases associated with IgG4 abnormalities. In addition to orbital pseudotumor, dacryoadenitis, Riedel thyroiditis, sinonasal manifestations, and rare miscellaneous conditions, IgG4-related sialadenitis is one of the most frequent presentations in the head and neck region. However, controversy still exists regarding the relationship between sialadenitis and IgG4-RD. This review focuses on the clinicopathological features of IgG4-related sialadenitis and its contemporary diagnostic criteria.
Authors with CRIS profile
Involved external institutions
Poznan University of Medical Sciences / Uniwersytet Medyczny im. Karola Marcinkowskiego w Poznaniu
Poland (PL)
NHS Lothian
United Kingdom (GB)
Katholieke Universiteit Leuven (KUL) / Catholic University of Leuven
Belgium (BE)
University of Turku / Turun yliopisto
Finland (FI)
University of Texas MD Anderson Cancer Center
United States (USA) (US)
International Head and Neck Scientific Group
Germany (DE)
Poland (PL)
NHS Lothian
United Kingdom (GB)
Katholieke Universiteit Leuven (KUL) / Catholic University of Leuven
Belgium (BE)
University of Turku / Turun yliopisto
Finland (FI)
University of Texas MD Anderson Cancer Center
United States (USA) (US)
International Head and Neck Scientific Group
Germany (DE)
How to cite
APA:
Czarnywojtek, A., Agaimy, A., Pietrończyk, K., Nixon, I.J., Vander Poorten, V., Mäkitie, A.A.,... Ferlito, A. (2024). IgG4-related disease: an update on pathology and diagnostic criteria with a focus on salivary gland manifestations. Virchows Archiv. https://dx.doi.org/10.1007/s00428-024-03757-0
MLA:
Czarnywojtek, Agata, et al. "IgG4-related disease: an update on pathology and diagnostic criteria with a focus on salivary gland manifestations." Virchows Archiv (2024).
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