213 Received:July 9, 2015, Revised:July 11, 2015, Accepted:July 13, 2015 Corresponding to:Jun-Ki Min, Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul 06591, Korea. E-mail:[email protected]pISSN: 2093-940X, eISSN: 2233-4718 Copyright ⓒ 2015 by The Korean College of Rheumatology. All rights reserved. This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited. Review Article Journal of Rheumatic Diseases Vol. 22, No. 4, August, 2015 http://dx.doi.org/10.4078/jrd.2015.22.4.213 Immunoglobulin G4 연관 질환 문수진ㆍ민준기 가톨릭대학교 의과대학 내과학교실 류마티스내과 Immunoglobulin G4-Related Disease Su-Jin Moon, Jun-Ki Min Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul, Korea Immunoglobulin G4-related disease (IgG4-RD) is an emerging immune-mediated fibro-inflammatory disorder which can in- volve 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 elu- sive, 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, par- ticularly histopathological findings. Glucocorticoid is the first-line therapy for patients with multiple organ dysfunction and clin- ical symptoms. Drugs such as azathioprine, mycophenolate mofetil, methotrexate, and cyclophosphamide can be used as ste- roid-sparing agents. Rituximab is reported to be an effective therapy for treatment of IgG4-RD, even without concomitant gluco- corticoid therapy. This review summarizes current concepts on pathophysiology, clinical manifestations, and treatment of IgG4-RD. (J Rheum Dis 2015;22:213-222) Key Words. Immunoglobulin G4-related disease, Physiopathology, Clinical manifestations, Therapy 서 론 Immunoglobulin G4 연관 질환(immunoglobulin G4 re- lated disease, IgG4-RD)은 혈청 IgG4 상승, IgG4 양성 형 질세포 및 림프구 침윤, 섬유화를 특징으로 하는 질환이다 [1]. IgG4 연관 질환은 비교적 최근에 알려진 질환으로 2001년 Hamano 등[2]은 자가면역췌장염 환자 혈청에서 IgG4가 상승되어 있으며 IgG4 양성 형질세포가 많이 침윤 되었음을 관찰하고 경화성 췌장염으로 보고하였고, 2003 년 Kamisawa 등[3]은 이 질환을 IgG4 연관 자가면역질환 으로 명명하였다. 2012년 국제 다학제 연구 모임(Inter- National Multidisciplinary Study Group)에서 IgG4 연관 질환이라는 명칭이 제시되어 현재 사용되고 있으며 다양 한 장기에서 IgG4 연관 질환이 보고되고 있다[4]. IgG4 연 관 질환의 발병기전은 아직 정확히 규명되지 않았지만, type 2 helper T (Th2) 세포와 조절 T (regulatory T, Treg) 세포로부터 나오는 사이토카인이 질병 발생에 관련된다고 알려진 자가면역질환의 일종이다[1]. 대부분의 IgG4 연관 질환은 스테로이드에 좋은 치료 반응을 보이지만 스테로 이드 사용량을 줄이면서 재발하거나, 스테로이드에 대한 반응이 충분치 않을 경우에는 azathioprine, cyclosporine, methotrexate와 같은 면역억제제의 사용이 효과적일 수 있다[1]. 특히 MIkulicz 병처럼 일부 IgG4 연관 질환은 류 마티스질환과 비슷한 임상 증상을 나타내는 경우가 있으
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Immunoglobulin G4 연관 질환 - KoreaMed · 2015-09-03 · Immunoglobulin G4-Related Disease 215 Figure 1. immunoglobulin G (IgG)4 Fab-arm exchange makes bispecifi c antibodies.
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Corresponding to:Jun-Ki Min, Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul 06591, Korea. E-mail:[email protected]
pISSN: 2093-940X, eISSN: 2233-4718Copyright ⓒ 2015 by The Korean College of Rheumatology. All rights reserved.This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.
Su-Jin Moon, Jun-Ki MinDivision of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul, Korea
Immunoglobulin G4-related disease (IgG4-RD) is an emerging immune-mediated fibro-inflammatory disorder which can in-volve 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 elu-sive, 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, par-ticularly histopathological findings. Glucocorticoid is the first-line therapy for patients with multiple organ dysfunction and clin-ical symptoms. Drugs such as azathioprine, mycophenolate mofetil, methotrexate, and cyclophosphamide can be used as ste-roid-sparing agents. Rituximab is reported to be an effective therapy for treatment of IgG4-RD, even without concomitant gluco-corticoid therapy. This review summarizes current concepts on pathophysiology, clinical manifestations, and treatment of IgG4-RD. (J Rheum Dis 2015;22:213-222)
binding oligomerization-like receptor2 (NOD2)를 활성화
시키면 BAFF를 생성하고 BAFF에 의해 B 세포가 IgG4를
만들게 된다. Watanabe 등[17]은 IgG4 연 제1형 자가면
역췌장염 환자의 액에서 얻은 단핵세포가 정상인에 비
해 NOD2를 통한 IgG4를 더 많이 생산한다고 주장하 다.
4) 호산구
조직 내 호산구 침윤은 IgG4 연 질환의 특징 인 조직
소견이며 IgG4 연 질환 환자의 약 30%정도에서 호산구
증가증이 찰된다[1]. 활성화된 호산구는 호산구량 이온
단백(eosinophil cationic protein)을 통한 콜라겐 수축,
TGF-β, IL-1β 생성을 통한 섬유화 유도, T 세포로의 항
원 제시 증가를 통해 IgG 연 질환 발병에 여하는 것으
로 알려져 있다[18,19].
5) 호염구
호염구는 Th2 반응에서 항원제시세포 역할을 한다. 호염
구 toll-유사 수용체(toll like receptor)를 자극하면 BAFF,
IL-13이 합성되고, 이는 B 세포에 작용하여 IgG4를 생성
Immunoglobulin G4-Related Disease
www.jrd.or.kr 215
Figure 1. immunoglobulin G (IgG)4 Fab-arm exchange makes bispecific antibodies. The heavy chains of IgG are bound to eachother by interchain disulfide bridge. As the disulfide bonds between heavy chains of IgG4 are unstable, IgG4 easily forms intrachaindisulfide bonds in the hinge region. Intrachain disulfide bond of IgG4 is linked by noncovalent interaction. When the non-con-valent bonds dissociate, half of one IgG4 molecule (a heavy chain-light chain pair) and half of another IgG4 molecule exchangerandomly, forming a Fab-arm exchange. Through such processes, the IgG4 molecule becomes bispecific by acquiring two Fab-arms with different epitope specificity. This bispecific IgG4 molecule, however, loses its ability to form immune complexes as the molecules cannot crosslink antigens.
(antibody-dependent cellular cytotoxicity), 보체를 통한
조직 손상과 같은 면역반응을 일으키는 능력이 다른 IgG
아형보다 떨어진다(Table 1)[21]. 이러한 이유들로 IgG4
는 면역반응을 유발하거나 악화시키기보다는 오히려 완화
시키는 역할을 할 것으로 생각되어 왔다. 이러한 주장은
IgG4가 용 유도를 통한 알러지 질환 치료를 한 경우에
생성되고 IgG4가 차단 항체로 작용하여 비만 세포 표면에
있는 IgE에 항원이 붙는 것을 억제함으로써 알러지 질병
발생을 억제할 수 있다는 연구 결과를 뒷받침해 다[22].
Nouri-Aria 등[23]은 조 T 세포에 의해 만들어지는
IL-10이 IgG4 생성을 유도하고 IgG4가 면역반응을 억제하
는 작용을 나타낼 수 있다고 보고한 바 있다. 그럼에도 불
구하고 IgG4가 질병의 발생에 여함을 시사하는 다음과
같은 소견들이 있다. 청 IgG4와 질병 활성도 사이에서
찰되는 상 계[1], IgG4 연 질환 환자의 약 50%∼
70%에서 보체 감소[24], 제1형 자가면역췌장염, IgG4 연
세뇨 간질신염 기 막에서 C3, IgG4 침착[25,26],
IgG4가 성구 표면에 있는 Fc-gamma 수용체 활성화를
Su-Jin Moon and Jun-Ki Min
216 J Rheum Dis Vol. 22, No. 4, August, 2015
Figure 2. Histological and immunohistochemical findings of biopsy specimens of the lymph node (A) and submandibular gland(B∼E). (A) The germinal centersare predominantly composed of small lymphocytes, centrocytes, centroblasts, and numerous ma-ture plasma cells (H&E, ×200). (B) The storiform pattern of fibrosis is present, indicating dense fibrosis within which lymphocytes,plasma cells, and occasional eosinophils are embedded (H&E, ×100). (C) Veins occluded by inflammatory infiltrate composed oflymphocytes and plasma cells are noted (arrowheads) (H&E, ×200). (D, E) The IgG4+/IgG+ plasma cell ratio is estimated at 90%.D: Immunoglobulin (Ig) G-immunohistochemical stain, E: IgG4-immunohistochemical stain; ×100.
No potential conflict of interest relevant to this article
was reported.
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