지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2015.1
- 수록면
- 213 - 222 (10page)
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초록· 키워드
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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.
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참고문헌 (80)
참고문헌 신청
Stone JH. / 2012 / IgG4-related disease / N Engl J Med 366:539~551
Hamano H. / 2001 / High serum IgG4 concentrations in patients with sclerosing pancreatitis / N Engl J Med 344:732~738
Kamisawa T. / 2003 / A new clinicopathological entity of IgG4-related autoimmune disease / J Gastroenterol 38:982~984
Stone JH. / 2012 / Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations / Arthritis Rheum 64:3061~3067
Uchida K. / 2012 / Prevalence of IgG4-related disease in Japan based on nationwide survey in 2009 / Int J Rheumatol 2012:358371
Hamano H. / 2001 / High serum IgG4 concentrations in patients with sclerosing pancreatitis / N Engl J Med 344:732~738
Kamisawa T. / 2003 / A new clinicopathological entity of IgG4-related autoimmune disease / J Gastroenterol 38:982~984
Stone JH. / 2012 / Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations / Arthritis Rheum 64:3061~3067
Uchida K. / 2012 / Prevalence of IgG4-related disease in Japan based on nationwide survey in 2009 / Int J Rheumatol 2012:358371
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