• 대한영상의학회지
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• 제82권4호
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• pp.826-837
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• 2021

면역글로불린G4 연관 질환(immunoglobulin G4-related disease; 이하 IgG4-RD)는 IgG4를 생산하는 면역세포에 의한 만성 염증성질환으로, 주로 타액선, 누액선, 안와, 췌장, 담도, 간, 신장, 후복막, 대동맥, 폐, 림프절 등 다양한 장기를 침범하고, 조직학적으로 IgG4 양성 형질세포와 림프구의 침윤 및 나선형의 섬유화(storiform fibrosis), 폐색정맥염(obliterative phlebitis)을 특징으로 한다. IgG4-RD의 흉부 침범에서 가장 흔한 소견은 종격동 림프절 비대와 폐의 림프관주위 간질 비후이다. 폐의 기관지혈관주위 간질 비후와 우측 척추곁 밴드형 연부조직은 IgG4-RD의 특징적 소견이고, 그 외에도 폐결절 혹은 종괴, 간유리음영, 폐포 간질비후, 흉막삼출 및 비후, 흉벽이나 종격동 종괴, 대동맥과 관상동맥의 혈관염이 발생할 수 있다. 영상의학적으로는 악성 종양이나 감염 및 다양한 염증성질환과의 감별진단이 필요하다. 본 연구에서는 흉부에서 발생하는 IgG4-RD의 영상 소견과 감별진단에 대해 기술하였다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제46권1호
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• pp.3-11
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• 2020

Immunoglobulin G4 (IgG4)-related dacryoadenitis and sialoadenitis (IgG4-DS) are part of a multiorgan fibroinflammatory condition of unknown etiology termed IgG4-related disease (IgG4-RD), which has been recognized as a single diagnostic entity for less than 15 years. Histopathologic examination is critical for diagnosis of IgG4-RD. CD4+ T and B cells, including IgG4-expressing plasma cells, constitute the major inflammatory cell populations in IgG4-RD and are thought to cause organ damage and tissue fibrosis. Patients with IgG4-RD who have active, untreated disease exhibit significant increase of IgG4-secreting plasmablasts in the blood. Considerable insight into the immunologic mechanisms of IgG4-RD has been achieved in the last decade using novel molecular biology approaches, including next-generation and single-cell RNA sequencing. Exploring the interactions between CD4+ T cells and B lineage cells is critical for understanding the pathophysiology of IgG4-RD. Establishment of pathogenic T cell clones and identification of antigens specific to these clones constitutes the first steps in determining the pathogenesis of the disease. Herein, the clinical features and mechanistic insights regarding pathogenesis of IgG4-RD were reviewed.

• 임상이비인후과
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• 제29권2호
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• pp.301-306
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• 2018

Immunoglobulin G4-related disease (IgG4RD) is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, as well as varying degrees of fibrosis. We report a case of a 70-year-old man with a rapid-growing cervical mass for several months. Computed tomography and positron emission tomography showed a huge, ill-defined mass involving left thyroid lobe and encasing the common carotid artery, which was clinically and radiologically suspicious for anaplastic thyroid carcinoma. Ultrasonography-guided core needle biopsy was performed and histopathology examination revealed to be consistent with IgG4RD, and the IgG4/IgG ratio was 0.6. After oral corticosteroid was administered, the mass was dramatically resolved. Because IgG4RD often presents as a single localized and infiltrated mass lesion, it can be confused and misdiagnosed as a malignancy. Thus, clinicians should consider IgG4RD as a differential diagnosis in a rapid-growing neck mass to prevent unnecessary and excessive treatments.

• Journal of Gastric Cancer
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• 제18권1호
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• pp.99-107
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• 2018

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disorder characterized by specific pathological findings and elevated serum IgG4 level. IgG4-RD in the stomach is rare, and occasionally diagnosed as gastric subepithelial tumor (SET) by endoscopy or computed tomography scan. Two female patients in the age group of 40-50 years were diagnosed with 4 cm sized gastric SET. One underwent laparoscopic gastric wedge resection. Another one had a history of subtotal gastrectomy for early gastric cancer and idiopathic thrombocytopenic purpura with oral steroids administration. She underwent a completion total gastrectomy with splenectomy for the gastric SET and ITP. The pathology showed storiform fibrosis, and IgG4 was positive in immunohistochemistry (IHC) stain. IgG4-RD is known as a medical disease that could be treated with oral steroids. The difficulty in preoperative diagnosis of the disease occasionally causes unnecessary gastric resection. Thus, preoperative diagnostic methods for IgG4-RD such as deep biopsy with IHC stain or magnetic resonance imaging are needed.

• 대한영상의학회지
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• 제83권4호
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• pp.910-917
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• 2022

면역글로불린 G4 (immunoglobulin G4; 이하 IgG4) 관련 질병은 한때는 장기별 별개의 질환으로 진단되었던 질환들을 근래에 공통적인 병리학적, 혈액학적 및 임상적 특징을 가지는 면역 매개 질환을 일컫는다. 이 분류의 질환은 다양한 장기를 침범할 수 있으며, 두경부 역시 침범할 수 있다. 두경부를 침범하였을 경우, 주로 눈물샘, 안와, 갑상선, 뇌하수체 및 뇌수막 등을 주로 침범한다. 이에, 초기 급성중이염과 유양돌기염으로 증상으로 내원한 65세 여자환자에서 안면신경, 뇌경막, 비인강과 경부임파절에 동시에 발생하여 자기공명영상과 조직검사로 진단된 IgG4 연관 질환에 대하여 증례 보고를 하고자 한다.

• 대한영상의학회지
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• 제84권3호
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• pp.686-691
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• 2023

면역글로불린(immunoglobulin; 이하 Ig) G4 연관 질환은 어떤 장기도 침범할 수 있는 섬유염증성 질환이며, 심근염의 형태로 발현되는 경우는 매우 드물다. 호흡곤란과 흉통이 있는 52세 남성이 심장자기공명영상에서 좌심실벽에 부종 및 심장간막과 심내막에 반점형과 결절형 지연 조영증강을 보이는 심근염 소견을 보였다. 혈액검사상 혈청 IgG4 상승과 호산구 증가증이 동반되었으며 심장 생검으로 호산구성 심근염과 IgG4 양성 세포가 확인되었다. 이에 저자들은 IgG4 연관 질환이 호산구성 심근염으로 발현된 드문 증례를 보고하고자 한다.

• 대한영상의학회지
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• 제81권4호
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• pp.996-1002
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• 2020

면역글로불린 G4 관련 질환은 신체 전 장기를 침범할 수 있는 전신적인 섬유-염증성 질환이다. 면역글로불린 G4 관련 질환이 난소를 침범한 경우는 드물어서 현재까지 2건의 증례만이 보고되었다. 컴퓨터단층촬영 및 자기공명영상에서 양측 난소의 악성 종양으로 오인되었으나 병리학적으로 면역글로불린 G4 관련 질환으로 진단한 증례를 보고하고자 한다.

• 생약학회지
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• 제51권4호
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• pp.340-348
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• 2020

This study was conducted to evaluate the immunomodulatory effects of red doraji (Platycodon grandiflorum, RD) prepared by repeated steaming and drying process in the immune-suppressed mice induced by pre (RD-A) or post-treatment (RD-B) with cyclophosphamide. The immune-stimulating effects of ethanol RD extract in in vivo at 150 (RD-1) and 300 mg/kg body weight (RD-2) for RD-A and RD-B groups were measured and compared to the NC group supplied with distilled water only or positive control group. After 14 days of oral supplement, serum IgA, IgG, and cytokine levels, splenocytes proliferation rate, NK cell activity, and gene expression of cytokines were measured as immune related biomarkers. Serum IgA, IgG, IL-1β, and IL-12 levels increased in both RD-A and RD-B groups while serum TNF-α level decreased in RD-A group compared to the NC group. Splenocytes proliferation rate, NK cell activity, and cytokine (IL-1β, IL-6, IFN-γ) expression levels were also improved by RD supplement in the both groups. The RD showed more significant immunomodulatory effects at higher dose (RD-2) rather than the lower dose (RD-1). Thus, RD has an immune efficacy in a dose dependent manner and can be used as an immune stimulating source to improve immunity.

• Korean Journal of Radiology
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• 제24권6호
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• pp.590-598
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• 2023

Objective: To investigate whether the levels of inflammation detected by 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) can predict disease relapse in immunoglobulin G4-related disease (IgG4-RD) patients receiving standard induction steroid therapy. Materials and Methods: This prospective study analyzed pretherapy FDG PET/CT images from 48 patients (mean age, 63 ± 12.9 years; 45 males and 3 females) diagnosed with IgG4-RD between September 2008 and February 2018, who subsequently received standard induction steroid therapy as the first-line treatment. Multivariable Cox proportional hazards models were used to identify the potential prognostic factors associated with relapse-free survival (RFS). Results: The median follow-up time for the entire cohort was 1913 days (interquartile range [IQR], 803-2929 days). Relapse occurred in 81.3% (39/48) patients during the follow-up period. The median time to relapse was 210 days (IQR, 140-308 days) after completion of standardized induction steroid therapy. Among the 17 parameters analyzed, Cox proportional hazard analysis identified whole-body total lesion glycolysis (WTLG) > 600 on FDG-PET as an independent risk factor for disease relapse (median RFS, 175 vs. 308 days; adjusted hazard ratio, 2.196 [95% confidence interval: 1.080-4.374]; P = 0.030). Conclusion: WTLG on pretherapy FDG PET/CT was the only significant factor associated with RFS in IgG-RD patients receiving standard steroid induction therapy.

• 임상이비인후과
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• 제29권2호
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• pp.240-244
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• 2018

Granulomatosis with polyangiitis (GPA) and Immunoglobulin (Ig) $G_4$-related disease ($IgG_4$-RD) are rare diseases and early diagnosis and proper management are imperative to prevent multi-organ damage. The authors present a case of a 60 years old woman who had facial paralysis and hearing loss. Lt intact canal wall tympanomastoidectomy, Lt facial nerve decompression and ossiculoplasty with partial ossicular replacement prosthesis (PORP) was done. During operation, middle ear tissue was biopsied and GPA with $IgG_4$-RD was diagnosed. After methyl prednisolone (MPD) pulse therapy and azathioprine therapy, the severity of paralysis was improved. We present this case because common otologic symptoms like facial palsy and hearing loss could be initial symptoms of rare systemic disease.