Immunoglobulin G4-Related Disease
Journal of Rheumatic Diseases
; : 213-222, 2015.
Article
在 Ko
| WPRIM
| ID: wpr-10585
Responsible library:
WPRO
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.
Key words
全文:
1
索引:
WPRIM
主要主题:
Pathology
/
Plasma Cells
/
Azathioprine
/
Fibrosis
/
Immunoglobulin G
/
Immunoglobulins
/
T-Lymphocytes
/
Methotrexate
/
Cytokines
/
T-Lymphocytes, Helper-Inducer
研究类型:
Diagnostic_studies
限制:
Humans
语言:
Ko
期刊:
Journal of Rheumatic Diseases
年:
2015
类型:
Article