• 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.

• Tuberculosis and Respiratory Diseases
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• 제79권3호
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• pp.184-187
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• 2016

Since IgG4-related pancreatitis was first reported in 2001, IgG4-related disease has been identified in other organs such as salivary gland, gallbladder, thyroid, retroperitoneum and kidney; but lung invasion is rare. A 63-year-old man presented with hemoptysis at the pulmonary clinic and chest computed tomography revealed about 4.1 cm irregular shaped mass with spiculated margin at the left upper lobe. Despite no elevation of serum IgG4 level, he was finally diagnosed as IgG4-related lung disease by transthoracic needle biopsy. After treatment with oral glucocorticoids, hemoptysis disappeared and the size of lung mass was decreased.

• 대한영상의학회지
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• 제82권4호
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• pp.1005-1010
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• 2021

본 증례는 좌안의 통증과 시력저하를 호소한 83세 남자 환자에게서 진단된 면역글로불린 G4 (immunoglobulin G4; 이하 IgG4) 관련 질환의 CT와 MRI 소견에 대한 보고이다. 이는 CT에서 주변의 골미란을 동반한 날개입천장 오목의 등밀도 연부조직 종괴로 관찰되었으며 이병변은 자기공명영상 T2 강조영상에서 등신호에서 저신호강도를 보이며 비균질한 조영증강을 보였다. 환자는 벌집굴 절제술과 조직검사를 시행하였고 조직학적 검사상 IgG4 관련 질환으로 진단되었다. 환자는 기저질환으로 당뇨병이 있었고, 이런 면역저하 환자군에서 흔하게 보이는 침습적 진균성 부비동염과의 영상학적 감별이 어려운 경우였다. 저자들은 침습적 진균성 부비동염과 유사한 영상 소견을 보였던 IgG4 관련 질환의 증례를 문헌 고찰과 함께 보고하고자 한다.

• 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.

• Journal of Yeungnam Medical Science
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• 제37권2호
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• pp.136-140
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• 2020

Immunoglobulin G4 (IgG4)-associated autoimmune hepatitis (AIH) is a very rare subtype of autoimmune hepatitis and characterized by marked elevated serum IgG and hepatic infiltration of IgG4-expressing plasma cells. Pathologic confirmation of hepatic IgG4-expressing plasma cells is usually required for the final diagnosis of IgG4-associated AIH. Herein, we report the case of a 47-year-old female diagnosed with autoantibody-negative IgG4-associated AIH mimicking lymphoproliferative disorders.

• Tuberculosis and Respiratory Diseases
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• 제76권4호
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• pp.179-183
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• 2014

Immunoglobulin (Ig) G4-related disease is a recently recognized systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with elevated circulating levels of IgG4. The disease can either be localized to one or two organs, or present as diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or metachronously. In the pulmonary manefestations, lesions associated with IgG4-related disease have been described in the lung parenchyma, airways and pleura, as well as the mediastinum. We report a case of IgG4-related disease presenting as massive pleural effusion and thrombophlebitis.

• Tuberculosis and Respiratory Diseases
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• 제76권1호
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• pp.38-41
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• 2014

Immunoglobulin G4 (IgG4)-related disease is a newly recognized condition characterized by fibroinflammatory lesions with dense lymphoplasmacytic infiltration, storiform-type fibrosis and obliterative phlebitis. The pathogenesis is not fully understood but multiple immune-mediated mechanisms are believed to contribute. This rare disease can involve various organs and pleural involvement is even rarer. We report a case of IgG4-related disease involving pleura. A 66-year-old man presented with cough and sputum production for a week. Chest radiography revealed consolidation and a pleural mass at right hemithorax. Treatment with antibiotics resolved the consolidation and respiratory symptoms disappeared, but the pleural mass was unchanged. Video-assisted thoracoscopic surgery was performed. Histopathology revealed dense lymphoplasmacytic infiltration and storiform fibrosis with numerous IgG4-bearing plasma cells. The serum IgG4 level was also elevated. Further examination ruled out the involvement of any other organ. The patient was discharged without further treatment and there is no evidence of recurrence to date.

• 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.

• 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.

• Journal of Chest Surgery
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• 제52권4호
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• pp.239-242
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• 2019

Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.