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Emerging therapy options for IgG4-related disease
Marco Lanzillotta1,2, Andreu Fernàndez-Codina3,4, Emma Culver5,6
1Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Expert Review of Clinical Immunology
|March 10, 2021
Summary
Immunoglobulin G4-related disease (IgG4-RD) requires ongoing treatment due to high relapse rates. Emerging therapies targeting B and T-lymphocyte activation show promise for personalized treatment strategies.
Area of Science:
- Immunology
- Rheumatology
- Pathophysiology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) awareness is growing globally.
- IgG4-RD requires repeated glucocorticoid courses for remission due to frequent relapses.
- Untreated IgG4-RD can lead to severe organ dysfunction, failure, and mortality.
Purpose of the Study:
- To review emerging treatment options for IgG4-RD.
- To correlate novel therapies with advancements in understanding disease pathophysiology.
- To explore personalized therapeutic strategies for IgG4-RD.
Main Methods:
- Literature review of English-language publications.
- Searches conducted on Pubmed and clinicaltrials.gov databases.
- Focus on emerging treatment options based on pathophysiology.
Main Results:
- Glucocorticoids are the current first-line induction therapy for IgG4-RD.
- Alternative immunosuppressive agents are needed to mitigate steroid toxicity and personalize treatment.
- Targeting B and T-lymphocyte activation is a promising therapeutic avenue.
Conclusions:
- Novel therapeutic targets are emerging from a better understanding of IgG4-RD pathogenesis.
- Personalized therapies are on the horizon for IgG4-RD management.
- Randomized controlled trials are essential to validate preliminary findings on targeted therapies.
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