Perspectives on current and emerging therapies for immunoglobulin G4-related disease.

Abstract

Understanding of the pathophysiology of immunoglobulin G4-related disease (IgG4-RD) over the last dozen years has opened the door to a variety of targeted treatment approaches. Glucocorticoids are an effective treatment for IgG4-RD if used at a sufficiently high dose, but disease flares are common during or after glucocorticoid tapers and these medications seldom lead to long-term, treatment-free remissions. Moreover, their long-term use in a disease that frequently affects middle-aged to elderly individuals and often causes major pancreatic damage leads to a narrow therapeutic index. Biological therapies offer the possibility of effective disease control with fewer treatment-associated side effects. Promising avenues of investigation include B-cell depletion, immunomodulation of B-cell subsets, interference with co-stimulation, Bruton's tyrosine kinase inhibition, and Signaling lymphocytic activation molecule F7-directed treatment.

• Authors: Yoshiya Tanaka, John H Stone
• Journal: Modern rheumatology (Mod Rheumatol) Vol. 33 Issue 2 Pg. 229-236 (Mar 02 2023) ISSN: 1439-7609 [Electronic] England
• PMID: 36408992 (Publication Type: Journal Article)
• Copyright: © Japan College of Rheumatology 2022. Published by Oxford University Press.

Chemical References

• Glucocorticoids

Topics

• Middle Aged
• Aged
• Humans
• Immunoglobulin G4-Related Disease (drug therapy)
• B-Lymphocytes
• B-Lymphocyte Subsets
• Treatment Outcome
• Remission Induction
• Glucocorticoids (therapeutic use, pharmacology)