Immunoglobulin G4-Related Disease
Journal of Rheumatic Diseases
;
: 213-222, 2015.
Article
in Korean
| WPRIM
| ID: wpr-10585
ABSTRACT
Immunoglobulin G4-related disease (IgG4-RD) is an emerging immune-mediated fibro-inflammatory disorder which can involve any organ. The main characteristics of IgG4-RD are increased serum IgG4 concentration, abundant IgG4+ plasma cells in affected tissues, and painless swollen organs often without general symptoms. Typical pathology features of IgG4-RD are lymphoplasmacytic infiltration, dense storiform fibrosis, and obliterative pheblitis. The pathogenesis of IgG4-RD remains elusive, but involvement of excess production of type 2 T helper cells, regulatory T-cell cytokines, and B-cell activating factor in the development of IgG4-RD has been suggested. Diagnosis of IgG4-RD can be made on the basis of serological, imaging, particularly histopathological findings. Glucocorticoid is the first-line therapy for patients with multiple organ dysfunction and clinical symptoms. Drugs such as azathioprine, mycophenolate mofetil, methotrexate, and cyclophosphamide can be used as steroid-sparing agents. Rituximab is reported to be an effective therapy for treatment of IgG4-RD, even without concomitant glucocorticoid therapy. This review summarizes current concepts on pathophysiology, clinical manifestations, and treatment of IgG4-RD.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pathology
/
Plasma Cells
/
Azathioprine
/
Fibrosis
/
Immunoglobulin G
/
Immunoglobulins
/
T-Lymphocytes
/
Methotrexate
/
Cytokines
/
T-Lymphocytes, Helper-Inducer
Type of study:
Diagnostic study
Limits:
Humans
Language:
Korean
Journal:
Journal of Rheumatic Diseases
Year:
2015
Type:
Article
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