Learning objectives
To review spectrum of typical and atypical imaging findings of IgG4-related diseases in the body on various modalities with emphasis on MRI and CT / PET-CT findings.
To highlight the imaging clues for correct diagnosis of IgG4-related diseases in various organ systems with particular focus on abdominal manifestations.
To discuss the differential diagnosis in affected organs and to highlight the relevant histopathological findings.
Background
IgG4-related disease is a systemic disease that can affect virtually any organ system in the body, most commonly the pancreas and biliary system. It usually affects patients older than 50 years of age and has a definite male preponderance with a male: female ratio of 2.9 to 3.7 [1].
Although histo-pathology as well as immuno-histochemistry (IHC) and raised serum IgG4 levels are required for the definite diagnosis, imaging plays an important role to raise the diagnostic possibility and map the entire extent of the disease....
Findings and procedure details
We present 10 cases of confirmed IgG4-related diseases involving pancreas, hepatobiliary system, gall-bladder, retroperitoneum, mesentery, kidneys and head-neck. Key typical and atypical MRI, CT & CT-PET findings will be highlighted.
The typical imaging features encountered were pancreatic mass, sausage shaped pancreas, capsule like rim / halo sign, multifocal & long segmental strictures of biliary system (most commonly of lower CBD), acalculous cholecystitis, retroperitoneal fibrosis, masses in mesentery and anterior pararenal space, inflammatory pseudotumor of nasal groove and buccal space mass.
Pancreas[Fig 1][Fig 2][Fig 3][Fig 4]:...
Conclusion
Radiologists should be familiar with the spectrum of its imaging & clinical features in various organ systems to avoid a delay in the correct diagnosis.
Knowledge of the system-wise pattern of presentation of IgG4 related diseases on imaging helps in making accurate and timely diagnosis.
A low threshold should be kept to suggest confirmatory investigations to prevent unnecessary surgery as IgG4 disease can simulate malignancy.
The disease shows a good response to steroid treatment making prompt diagnosis important and to prevent progression.
Personal information and conflict of interest
R. Jyani:
Nothing to disclose
S. K. Puri:
Nothing to disclose
A. K. Chaturvedi:
Nothing to disclose
J. Khoda:
Nothing to disclose
M. KM:
Nothing to disclose
References
Tang CS, Sivarasan N, Griffin N. Abdominal manifestations of IgG4-related disease: a pictorial review. Insights into imaging. 2018 Aug;9(4):437-48.
Martínez-de-Alegría A, Baleato-González S, García-Figueiras R, Bermúdez-Naveira A, Abdulkader-Nallib I, Díaz-Peromingo JA, Villalba-Martín C. IgG4-related disease from head to toe. Radiographics. 2015 Nov;35(7):2007-25.
Hedgire SS, McDermott S, Borczuk D, Elmi A, Saini S, Harisinghani MG. The spectrum of IgG4-related disease in the abdomen and pelvis. American Journal of Roentgenology. 2013 Jul;201(1):14-22.
Jandee S, Boonsri P. Atypical Manifestations of IgG4-Related Disease as Multiple Liver Abscesses with Subcapsular...