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Updated: Apr 5, 2026
07:45
An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy
Published on: October 21, 2014
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IgG4-related disease
Kanae Kubo1, Kazuhiko Yamamoto1
1Department of Allergy and Rheumatology, The University of Tokyo Hospital, Tokyo, Japan.
International Journal of Rheumatic Diseases
|August 11, 2015
Summary
Immunoglobulin G4-related disease (IgG4-RD) is a complex fibro-inflammatory condition. Glucocorticoid therapy effectively manages IgG4-RD, improving clinical and pathological outcomes.
Area of Science:
- Rheumatology and Immunology
- Pathology
- Internal Medicine
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibro-inflammatory condition with unknown causes.
- It is characterized by specific histopathological findings: dense lymphoplasmacytic infiltrates, IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis.
- IgG4-RD encompasses diverse conditions like Mikulicz disease, autoimmune pancreatitis, and retroperitoneal fibrosis.
Purpose of the Study:
- To review the current understanding of Immunoglobulin G4-related disease (IgG4-RD).
- To summarize recent advancements in IgG4-RD pathogenesis, clinical manifestations, and therapeutic strategies.
Main Methods:
- Literature review of recent evidence on IgG4-RD.
- Synthesis of information regarding diagnostic criteria, histopathology, and clinical presentations.
- Analysis of treatment outcomes, particularly focusing on glucocorticoid therapy.
Main Results:
- IgG4-RD presents with inflammatory swelling or tumefactive lesions.
- Glucocorticoid therapy demonstrates efficacy in resolving clinical and pathological abnormalities.
- Significant progress has been made in understanding the disease since its international recognition in 2011.
Conclusions:
- IgG4-RD is a distinct clinicopathological entity requiring comprehensive understanding.
- Effective management is achievable with glucocorticoid treatment, leading to improved organ function.
- Ongoing research continues to elucidate the complexities of IgG4-RD pathogenesis and treatment.
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