혈중 호산구와 면역글로불린 E 증가를 동반한 면역글로불린 G4 관련안질환

임연주(Yeon Ju Lim),이수정(Soo Jung Lee) 대한검안학회 2021 Annals of optometry and contact lens Vol.20 No.3

목적: 혈중 호산구와 면역글로불린(immunoglobulin, Ig) E 증가를 보인 환자에서 외안근비대를 동반한 IgG4관련안질환이 진단된 증례를 보고하고자 한다. 증례요약: 56세 남자 환자가 5개월 전부터 발생한 전신 두드러기와 양안 눈꺼풀부종을 주소로 알레르기 내과에 내원하였다. 혈액검사에서 호산구 및 IgE가 1,309/uL, 1,793 IU/mL로 증가된 소견과 안와 컴퓨터단층촬영에서 양안 모든 외안근의 비대와 양안의 경한안구돌출이 관찰되어 안과로 의뢰되었다. 정렬은 모든 방향에서 정위였고, 양안 외전 -1이었다. 외안근의 조직검사에서 림프구 침윤과 섬유화를 보였고, IgG4+/IgG+ 형질세포의 비율이 40% 이상이어서 IgG4관련안질환으로 진단하였다. 혈청 IgG4도 1,710 mg/dL로 증가된 소견을 보였다. 고용량 스테로이드 정맥주사를 시행하였고 2개월 후 눈꺼풀부종은 호전된 모습을 보였다. 3개월 후 호산구 및 IgE와 IgG4는 모두 감소되었다. 결론: IgG4관련안질환은 호산구와 IgE 상승을 동반할 수 있으며 이러한 소견은 IgG4관련안질환을 진단하는 데 도움이 될 수 있다. Purpose: To report a case with peripheral eosinophilia and elevated immunoglobulin (Ig) E levels, subsequently diagnosed as IgG4-related ophthalmic disease involving the extraocular muscles. Case summary: A 56-year-old male visited the allergy department presenting with systemic urticaria and bilateral eyelid swelling that began 5 months prior. Laboratory examinations showed elevated levels of serum eosinophil and IgE, 1,309 IU/uL and 1,793 IU/mL, respectively. Orbital computed tomography revealed that all extraocular muscles and the bilateral exophthalmos were enlarged, and the patient was referred to the ophthalmology department. Eye alignment was orthophoric for all gaze directions, and limited abduction (-1) was noted in both eyes. An incisional biopsy of the extraocular muscles was conducted. Histopathological findings showed lymphoid aggregates, diffuse fibrosis, and an increased IgG4+/IgG+ plasma cell ratio of 40%, which led to the diagnosis of IgG4-related ophthalmic disease. An elevated IgG4 serum level (1,710 mg/dL) was also noted. The patient received high-dose intravenous steroids and eyelid swelling improved after two months. Levels of serum eosinophil, IgE, and IgG4 all decreased after three months. Conclusions: IgG4-related ophthalmic disease may be accompanied by eosinophilia and elevated IgE. These findings may facilitate future diagnoses of this disease.

비강내 악성종물로 오인된 부비동의 Immunoglobulin G4 연관 경화성 질환 1예

정주용,이재훈 대한비과학회 2017 Journal of rhinology Vol.24 No.1

Immunoglobulin G4 (IgG4)-related sclerosing disease (RSD) is a chronic inflammatory disorder characterized by elevated serum IgG4 levels as well as an abundant infiltration of IgG4-positive plasmacytes. Involvement of the paranasal sinus is very rare, so it is difficult to make an accurate diagnosis. Hence, various differential diagnoses, which typically include elevated serum IgG4 levels and the infiltration of IgG4-postive cells into tissues, need to be excluded, especially for malignant diseases and mimicking disorders. Systemic corticosteroids are generally effective at inducing IgG4-RSD remission. Recently, a 43-yearold woman presented with a mass originating from the ethmoid sinus, suggesting malignancy. The patient underwent biopsy of the lesion. The pathologic specimen revealed the magnitude of IgG4-positive plasma cell infiltration, which was consistent with IgG4-RSD. In addition, serum IgG4 levels were markedly increased. After glucocorticoid treatment, the serum IgG4 levels decreased and the symptoms improved. We present a rare case of IgG4-RSD with isolated involvement of the paranasal sinuses.

Case Report : Mikulicz`s Disease with Progressively Transformed Germinal Centers-type Immunoglobulin G4-related Lymphadenopathy Mimicking Sjogren`s Syndrome

( Hye Ji Kim ),( Jean A Kim ),( Jun Ki Min ) 대한류마티스학회 2015 대한류마티스학회지 Vol.22 No.6

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease, and lymphadenopathy is frequently observed in these patients. Among the 5 subtypes of IgG4-related lymphadenopathy, progressively transformed germinal centers (PTGC)-type IgG4-related lymphadenopathy possesses a unique characteristic that differentiates it from the other 4 subtypes. Here, we report on a rare case of PTGC-type IgG4-related lymphadenopathy accompanying Mikulicz`s disease. A 39-year-old female complained of a left cervical mass and bilateral upper eyelid hypertrophy. The serum level of IgG4 was elevated, and computed tomography showed enlargement of the bilateral lacrimal and submandibular glands and left cervical lymph node. Excisional biopsy of a submandibular gland and cervical lymph node was performed, and the histopathologic findings revealed Mikulicz`s disease accompanied by PTGC-type IgG4-related lymphadenopathy. After treatment of the patient with oral prednisolone and azathioprine, the patient`s appearance improved. To the best of our knowledge, no case of PTGC-type IgG4-related lymphadenopathy has been previously reported in Korea. (J Rheum Dis 2015;22:395-400)

Immunoglobulin G4-related hypertrophic pachymeningitis with an isolated scalp mass mimicking a brain tumor: a case report and literature review

( Jina Yeo ),( Gi Taek Yee ),( Jaedeok Seo ),( Mi Ryoung Seo ),( Han Joo Baek ),( Hyo-jin Choi ) 대한류마티스학회 2024 대한류마티스학회지 Vol.31 No.1

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder associated with fibroinflammatory conditions that can affect multiple organs. Hallmark histopathological findings of IgG4-RD include lymphocytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. However, little is known about central nervous system involvement of IgG4-RD. Hypertrophic pachymeningitis (HP) has recently been reported as a manifestation of IgG4-RD, which may have previously been demonstrated in a significant percentage of idiopathic cases. Herein, we report a rare case of a 63-year-old male who presented with a scalp mass that mimicked a brain tumor. He was diagnosed with IgG4-related HP (IgG4-RP) after surgery. This case suggests that awareness of a possibility of IgG4-RP in patients with isolated scalp masses, even in the absence of systemic symptoms, is crucial. A combination of careful history taking, evaluation of serum IgG4-levels and imaging as an initial work-up, followed by tissue biopsy, is important for the differential diagnosis of IgG4-RP, malignancy, and other infectious diseases.

면역글로불린 G4 연관질환의 개요

문성훈 ( Sung Hoon Moon ),송태준 ( Tae Jun Song ),김명환 ( Myung Hwan Kim ) 대한췌장담도학회 2015 대한췌담도학회지 Vol.20 No.3

Immunoglobulin G4 (IgG4)-related disease is a newly named fibroinflammatory condition characterized by tumefactive lesions that contain dense lymphoplasmacytic infiltrates rich in IgG4-positive cells. Elevated serum IgG4 concentrations also often accompany IgG4-related disease. Many medical conditions that were long viewed as diseases confined to single organs (e.g., Mikulicz disease, type 1 autoimmune pancreatitis, Riedel’s thyroiditis, multifocal fibrosclerosis, inflammatory pseudotumor, mediastinal fibrosis, retroperitoneal fibrosis, and etc.) are now designated as part of the spectrum of IgG4-related disease. The preferred nomenclature suggested by a committee of international experts uses the prefix “IgG4-related-” for individual organ involvement, regardless of the organ system affected. One exception is type 1 autoimmune pancreatitis (IgG4-related pancreatitis). Comprehensive diagnostic criteria for IgG4-related disease and organ-specific diagnostic criteria (e.g., IgG4-related dacryoadenitis and sialadenitis, type 1 autoimmune pancreatitis, IgG4-related kidney disease and IgG4-related sclerosing.

자연경과에서 비대상성 간경변으로 진행한 IgG4 연관 경화성 담관염

한지수 ( Jisoo Han ),전제혁 ( Jae Hyuck Jun ),정경화 ( Kyung Hwa Jung ),정대현 ( Dae Hyun Jeong ),박재철 ( Jae Cheol Park ),노수민 ( Soomin Noh ),형례창 ( Li Chang Hsing ),김명환 ( Myung-hwan Kim ) 대한췌담도학회 2017 대한췌담도학회지 Vol.22 No.4

IgG4 연관 질환은 다양한 조직의 섬유 경화성 반응과 연관되어 IgG4 양성의 형질세포와 임파구가 침착되는 염증 반응을 특징으로 하는 전신 침범 자가면역 질환이다. 이 중 IgG4 연관 경화성 담관염은 IgG4 연관 질환의 담관 침범을 말한다. IgG4 연관 경화성 담관염에 대한 대부분의 증례 보고는 단기간의 임상 경과만을 보고하였고 이 단기간의 경과의 대부분은 스테로이드 치료 후 담도 협착과 황달의 호전을 보였다. IgG4 연관 경화성 담관염의 장기적 예후와 자연 경과는 아직 잘 보고되지 않았으며 국내에서는 아직까지 IgG4-SC 환자가 간경변으로 진행되었다는 보고는 없다. 저자들은 IgG4-SC 환자가 제대로 치료를 받지 않고 오랜 시간 지났을 때 자연 경과로서 비대상성 간경변으로 진행하고 더 이상 스테로이드에 반응하지 않은 1예를 경험하여 문헌고찰과 함께 보고하는 바이다. Immunoglobulin G4-related disease (IgG4-RD) is characterized by extensive IgG4-positive plasma cells infiltration resulting in inflammation and fibrosis of the affected organs. Patient with IgG4-RD shows an excellent response to glucocorticoid therapy especially when given at early onset stages. IgG4-related sclerosing cholangitis (IgG4-SC) is considered biliary manifestation of IgG4-RD. The natural history and longterm prognosis of IgG4-SC is not well defined and most of the previous case series have reported short duration of follow-up. We present the case of development of decompensated liver cirrhosis from IgG4-related sclerosing cholangitis with a 6-year natural clinical course. Korean J Pancreas Biliary Tract 2017;22(4):179-183

복강 내 발생한 Immunoglobulin G4 연관 염증성 가성종양

모인호 ( In Ho Moh ),김진배 ( Jin Bae Kim ),신수린 ( Su Rin Shin ),정성원 ( Sung Won Jung ),박상훈 ( Sang Hoon Park ),김정원 ( Jeong Won Kim ),신미경 ( Mi Kyung Shin ),이명석 ( Myung Seok Lee ) 대한소화기학회 2012 대한소화기학회지 Vol.60 No.4

The term inflammatory pseudotumor (IPT) has been used to describe inflammatory and fibrosing tumoral processes of an undetermined cause that may involve a variety of organ system. IgG4-related disease is a newly recognized fibroinflammatory condition characterized by IgG4-producing plasma cell expansion in affected organs and, often but not always, elevated serum IgG4 concentrations. IgG4-related IPTs, a subtype of IPT, are characterized by dense infiltration of IgG4-positive plasma cells and stromal fibrosis. The association between inflammatory pseudotumor and IgG4 was first reported with a regard to sclerosing pancreatitis. Despite there are many reports on intraperitoneal IPTs including both cellular and lymphoplasmacytic type, only a few cases have been confirmed to be IgG4-related. We experienced a case of intraperitoneal IgG4-related inflammatory pseudotumor in an 83-year-old woman presenting with epigastric pain and malaise. Surgical specimens revealed an IgG4-related inflammatory pseudotumor.

Immunoglobulin G4-related sclerosing cholangitis

George Goodchild,Stephen P. Pereira,George Webster 대한내과학회 2018 The Korean Journal of Internal Medicine Vol.33 No.5

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition of which IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation. In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC, namely, primary sclerosing cholangitis and cholangiocarcinoma, outline diagnostic criteria for IgG4-SC, provide insight into possible pathophysiological mechanisms underlying the disease and discuss short and long-term management options of this recently described disease.

간문부 담관암과 유사한 소견을 보인 면역글로불린 G4 연관 경화성 담관염

장지웅,김명환,김태균,박도현,이상수,서동완,이성구 대한췌담도학회 2012 대한췌담도학회지 Vol.17 No.1

Immunoglobulin G4 (IgG4)-associated sclerosing cholangitis is a steroid responsive biliary disease, frequently involving the bile duct, that is often associated with autoimmune pancreatitis. It is characterized by elevation of immunoglobulin G4 in serum and infiltration of immunoglobulin G4 positive plasma cells in bile ducts. The clinically important thing is to differentiate this rare disease from primary sclerosing cholangitis and cholangiocarcinoma. However, the preoperative diagnosis is likely to be missed due to the lack of specific symptoms and specific imaging features. Therefore, making an early diagnosis requires a high index of suspicion. This article reports on a 71-year-old man with IgG4-associated sclerosing cholangitis mimicking hilar cholangiocarcinoma.

면역글로불린 G4 연관 경수막염 1예

김지인,송진택,권형주,이지용 대한신경집중치료학회 2016 대한신경집중치료학회지 Vol.9 No.2

Background: Immunoglobulin G4 (IgG4) related disease is an immune-mediated fibroinflammatory condition that can affect multiple organs. There are only few reports of pachymeningeal involvement by IgG4 related disease in Korea. Case Report: A 50-year-old woman was admitted due to generalized seizure and headache. Brain magnetic resonance imaging (MRI) showed nodular dural thickening and enhancement in the right frontal areas. A meningeal biopsy was performed. On pathology, chronic inflammation, fibrosis and plasma cell infiltration were revealed. Immunoglobulin G4 immunohistochemistry showed prominent IgG4+ plasma cell infiltrate. Serum IgG level was normal but IgG4 subclass level was significantly elevated. She was treated with intravenous dexamethasone followed by oral prednisolone. After 1 year of treatment, brain MRI showed marked decrease of dural thickening and enhancement. Conclusions: We report a case of IgG4 related pachymeningitis improved with corticosteroid. IgG4 related disease should be included in the differential diagnosis in patients with hypertrophic pachymeningitis.