226. Don't Ignore IgG4 - Approach to IgG4 Related Disease
Mar 3, 2024
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Exploring the immune-mediated nature of IgG4 related diseases, diagnostic approach, comprehensive workup required for diagnosis, treatment strategies including B cell targeted therapy, and classification criteria for IgG4-related disease
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Quick takeaways
IgG4 Related Disease is a systemic entity affecting multiple organs with immune-mediated responses involving B and T cells.
Diagnosis and management of IgG4 related disease require a comprehensive evaluation encompassing various tests, imaging studies, and therapeutic strategies.
Deep dives
Understanding IgG4 Related Disease
Immunoglobulin G4, or IgG4 related disease, was reclassified in 2003 as a systemic disease entity linking various conditions like autoimmune pancreatitis, sclerosing cholangitis, and thyroiditis. The disease involves immune-mediated multi-organ responses with B cells and T cells contributing to lymphoplasmacytic infiltrations and fibrosis. Treatment aims to induce disease remission, prevent relapse, and preserve organ function.
Diagnosis and Workup for IgG4 Related Disease
Diagnosis of IgG4 related disease requires an in-depth evaluation involving a combination of investigations due to its potential to affect various organs. Workup includes CBC, creatinine, serum IgG4 levels, and histopathological biopsy findings. Imaging studies such as chest X-ray and CT abdomen aid in evaluating organ involvement and assessing disease extent.
Treatment Options for IgG4 Related Disease
Therapeutic strategies for IgG4 related disease include glucocorticoid therapy for remission induction, maintenance therapy, and consideration of B-cell targeted therapies like Rituximab for severe or relapsing cases. Surgical interventions may be necessary in specific complications. Early diagnosis and appropriate treatment are crucial in managing the disease and preventing extensive organ fibrosis.
This week we take you through a disease that can truly affect just about every organ system - IgG4 Related Disease. We will walk you through the presentation, work up, and approach to treatment