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.

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.

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.

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.

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.

Immunoglobulin G4-Related Kidney Disease: A Comprehensive Pictorial Review of the Imaging Spectrum, Mimickers, and Clinicopathological Characteristics

서니은,김진희,변재호,이승수,김형중,이문규 대한영상의학회 2015 Korean Journal of Radiology Vol.16 No.5

Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review. Awareness of the broad imaging spectrum of IgG4-KD and differential diagnosis from its mimickers will thus facilitate its early diagnosis and treatment.

이혈구 감소증이 동반된 Immunoglobulin G4 연관 간질성 신염

이순규 ( Soonkyu Lee ),박윤정 ( Yune Jung Park ),조의주 ( Uiju Cho ),최영진 ( Young Jin Choi ),김완욱 ( Wan Uk Kim ) 대한류마티스학회 2015 대한류마티스학회지 Vol.22 No.6

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease found in many organs including biliary tract, salivary gland, kidney, and lung. Tubulointerstitial nephritis is the most common renal manifestation, but hematologic involvement of IgG4-RD is rare. Here, we report on a case of a 57-year-old male with IgG4-related interstitial nephritis with bicytopenia, which was initially thought to be systemic lupus erythematosus. He presented with proteinuria, anemia, thrombocytopenia, and low complement levels. Histological findings showed an increased number of IgG4-positive plasma cells (>200/high power field), and an elevated IgG4/IgG ratio (>90%). Serum levels of IgG and IgG4 were also increased. This case emphasized the importance of differential diagnosis of IgG4-RD and immune complex glomerulonephritis. (J Rheum Dis 2015;22:401-404)

헤노흐-쇤라인 자색반과 동반된 IgG4 연관 요세관간질 신염

양현 ( Hyun Yang ),최수경 ( Soo Kyoung Choi ),김보경 ( Bo Kyoung Kim ),유지연 ( Ji Yeon Yoo ),고은실 ( Eun Sil Koh ),장윤식 ( Yoon Sik Chang ),정성진 ( Sung Jin Chung ) 대한내과학회 2014 대한내과학회지 Vol.87 No.1

면역글로불린 G4 (IgG4) 연관 질환은 표적 장기의 림프구 및 IgG4 양성 형질세포의 침윤과 섬유화를 특징으로 하는 질환이다. 췌장, 폐, 침샘, 눈물샘, 담도, 후복막강, 신장 등거의 모든 장기를 침범할 수 있으며 이 중 신장을 침범하는 경우는 흔하지 않지만 신장을 침범하게 되면 대부분 요세관간질 신염의 양상을 나타낸다. 일반적으로 IgG4 연관 요세관간질 신염의 경우 단독으로 신장만을 침범하는 경우는 드물고 타 장기 침범과 함께 보고되는 경우가 많으나 헤노흐- 쇤라인 자색반과 동반된 경우는 매우 드물다. 저자들은 본 보고에서 반복적인 자색반이 있는 환자에서 신기능 저하 소견이 있어 신생검을 포함한 검사를 진행하였고 신장 외 장기침범이 없으면서 헤노흐-쇤라인 자색반이 동반된 IgG4 연관 요세관간질 신염을 진단하고 치료한 증례를 기술하였다. Tubulointerstitial nephritis is one of the common manifestations of immunoglobulin G (IgG)4-related disease; however, among all cases of tubulointerstitial nephritis undergoing renal biopsies, IgG4-related tubulointerstitial nephritis seems to be relatively rare because of its trivial urinary findings. A previously healthy 54-year-old man was referred to our clinic with a 4-week history of lower leg purpura and renal dysfunction. A kidney biopsy was planned because of bilateral renomegaly, by imaging studies, and elevated serum creatinine levels. Pathological findings in the kidney showed prominent infiltration of IgG4-postive plasma cells in the tubulointerstitium, but not the glumeruli. A skin biopsy revealed leukocytoclastic vasculitis, accompanied by deposition of IgA and C3 in the vascular wall, indicating Henoch-Schonlein purpura (HSP). Although cases of combined IgG4-related disease and microvasculitis, including HSP, are extremely rare, the possibility of an association between two diseases deserves attention. (Korean J Med 2014;87:96-100)

폐암으로 오인된 면역글로불린 G4 연관 경화성 질환 1예

권수희,이영경,심미숙,이향임 대한영상의학회 2013 대한영상의학회지 Vol.69 No.1

Immunoglobulin (Ig) G4-related sclerosing disease is a recently described systemic fibro-inflammatory disease associated with an elevated circulating level of IgG4 and extensive IgG4-positive lymphoplasmacytic infiltration, resulting in sclerosing inflammation involving various body organs. We experienced one case where surgery confirmed IgG4-related sclerosing disease as a solitary lung mass mimicking lung cancer. We report radiologic findings including chest computed tomography and positron emission tomography computed tomography, with clinical manifestations of IgG4-related sclerosing disease. 면역글로불린 G4 연관 경화성 질환은 혈청 면역글로불린 G4 수치의 상승과 췌장, 간, 쓸개관, 후복막 등의 여러 신체 기관에 면역글로불린 G4 양성 림프형질세포의 광범위한 침윤으로 인하여 경화성 염증을 일으키는 전신 섬유 염증성 병변으로 알려져 있다. 저자들은 폐암과 유사한 단일 폐 종괴의 형태로 나타나 수술 시행 후 면역글로불린 G4 연관 경화성 질환으로 확진된 증례를 경험하였다. 이에 임상적인 소견과 함께 흉부전산화단층촬영(CT)과 양전자단층촬영(positron emis-sion tomography CT) 소견을 보고하고자 한다.

Immunoglobulin G4-Related Thyroid Disease: A Single-Center Experience and Literature Review

김미화,Bictdeun Kim,장아름,전민지,최영준,이유미,송동은,김원구 대한내분비학회 2022 Endocrinology and metabolism Vol.37 No.2

Background: Immunoglobulin G4 (IgG4)-related disease is an entity that can involve the thyroid gland. The spectrum of IgG4-related thyroid disease (IgG4-RTD) includes Hashimoto thyroiditis (HT) and its fibrotic variant, Riedel thyroiditis, as well as Graves’disease. The early diagnosis of IgG4-RTD is important because it is a medically treatable disease, and a delay in the diagnosis mightresult in unnecessary surgery. We present a case series of IgG4-RTD with a review of the literature. Methods: We retrospectively reviewed the clinical presentation and the radiological and pathological findings of patients diagnosedwith IgG4-RTD between 2017 and 2021 at a tertiary medical center in Korea. We also conducted a literature review of IgG4-RTD. Results: Five patients were diagnosed with IgG4-RTD during the study period. The patients’ age ranged from 31 to 76 years, and threepatients were men. Most patients visited the clinic for a neck mass, and hypoechogenic nodular lesions were observed on neck ultrasonography. Three patients had IgG4 HT, and two patients had IgG4 Riedel thyroiditis. All patients developed hypothyroidism that necessitated L-thyroxine replacement. The diagnosis of IgG4-RTD was confirmed after a pathological examination of the surgical specimen in the first two cases. However, the early diagnosis was possible after a core needle biopsy in three clinically suspected patients. Conclusion: The diagnosis of IgG4-RTD requires clinical suspicion combined with serology and histological analyses using IgG4immunostaining. The early diagnosis of IgG4-RTD is difficult; thus, biopsy with IgG4 immunostaining and serum IgG4 measurements will help diagnose patients suspected of having IgG4-RTD.