IgG4-related Disease from Head to Toe (2024)-M1-CMS06-2024

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Video Summary

Immunoglobulin G4 (IgG4)-related disease is a complex fibroinflammatory condition affecting multiple organs, often synchronously or asynchronously. It was recognized as a distinct disease entity less than 25 years ago. Commonly affected areas include the head and neck, where the salivary glands are often involved, although symptoms may be mild or absent. IgG4-related disease can mimic other conditions, such as allergies, Sjögren's syndrome, or malignancies. Diagnosis mainly requires histopathological confirmation, often through core biopsy. Imaging modalities like MRI and PET scans aid in identifying multisystem involvement but are not diagnostic alone. The disease typically responds well to corticosteroids, although recurrence rates are high, and the relapse can affect different organ systems. In the chest, IgG4-related disease can present with masses or lymphadenopathy, sometimes leading to misdiagnosis as sarcoidosis or lymphoma. Abdominal involvement frequently affects the pancreas, causing autoimmune pancreatitis, bile ducts, and kidneys. Unique imaging features include sausage-shaped pancreas or long-segment bile duct involvement. Given its multifocal nature, clinicians are encouraged to thoroughly check for IgG4-related disease across multiple organ systems and consider it in differential diagnoses, using PET scans for comprehensive assessment and confirming through biopsies.

Keywords

IgG4-related disease

fibroinflammatory condition

multiple organs

histopathological confirmation

corticosteroids

autoimmune pancreatitis

multisystem involvement

differential diagnoses

biopsies