• Journal of The Korean Ophthalmological Society
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• v.59 no.11
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• pp.1071-1076
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• 2018

Purpose: To report a case of immunoglobulin G4 (IgG4)-related ophthalmic disease associated with adult xanthogranulomatous disease. Case summary: A 38-year-old male with a history of cholecystectomy visited our clinic for bilateral periorbital swelling. Histopathology of the orbital biopsy showed diffuse infiltration of foamy histiocytes with Touton giant cells and lymphoid follicles, with a diagnosis of adult-onset xanthogranuloma. After excisional biopsy, he was treated with azathioprine and prednisolone. Four years after treatment, he again visited the clinic due to bilateral, yellowish eyelid masses. Serological examinations were all nonspecific findings, except for elevation of IgG and IgG4 levels. Magnetic resonance imaging showed bilateral symmetric soft tissue enlargement with slightly heterogeneous T1/T2 isosignal intensity, with contrast enhancement at the superolateral aspect of extraconal spaces. Excisional biopsy and blepharoplasty were performed. Immunohistochemical sections showed that the IgG4+/IgG plasma cell ratio was 10-20% and the IgG4 plasma cell count was 22/high power field (HPF). His past sections of 2013 from the pathology department were again stained and showed that the IgG4+/IgG plasma cell ratio was 40-50% and the IgG4 plasma cell count was 59/HPF. Thus, he was definitely diagnosed with IgG4-related ophthalmic disease. Conclusions: If there is recurrent eyelid swelling, IgG4-related ophthalmic disease should be considered as a differential diagnosis. And the patient with adult xanthogranulomatous disease can be diagnosed with IgG4-related ophthalmic disease.

• Journal of Clinical Otolaryngology Head and Neck Surgery
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• v.29 no.2
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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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• v.30 no.2
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• pp.128-131
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• 2013

Immunoglobulin G4 (IgG4)-related disease is an inflammatory condition characterized by IgG4 positive plasma cell infiltration. It can affect any organ in the body and mainly involves the pancreas, liver, biliary tracts, orbits, salivary glands and lymph nodes. It can manifest as an inflammatory pseudotumor. Pseudolymphoma as an inflammatory pseudotumor is a group of benign tumors that exhibit histological and clinical features suggestive of malignant lymphoma. Studies on IgG4-related disease are rarely reported, and no case of the disease that involved the maxillary bone and adjacent soft tissue, except for the skin, has been reported. Therefore, we report herein a case of pseudolymphomatous IgG4-related disease that involved the maxilla, with a literature review.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.33 no.2
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• pp.110-114
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• 2022

IgG4-related disease is a fibroinflammatory condition by infiltration of IgG4-positive plasma cells that often presents as a tumorous lesion. This disease can affect nearly every organ system. After the pancreas, the head and neck region is second most common site for presentation of IgG4-related disease such as Mikulicz's disease, Küttner tumor. The involvement of IgG4-related disease in laryngeal lesions is extremely rare. We have experienced a case of IgG4-related disease with pseudotumor formation in the larynx that is suggestive of malignancy in radiologic findings. But the pathology findings was finally confirmed as IgG4-related disease. Oral treatment with prednisolone was initiated, and the edematous mass reduced in size without permanent functional impairment of vocal fold mobility. We report our experience with a literature review.

• Journal of Gastric Cancer
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• v.18 no.1
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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.

• Korean Circulation Journal
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• v.53 no.3
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• pp.189-190
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• 2023

• Korean Journal of Radiology
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• v.24 no.6
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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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• v.52 no.4
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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.

• Tuberculosis and Respiratory Diseases
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• v.76 no.4
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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.

• Journal of the Korean Society of Radiology
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• v.83 no.2
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• pp.450-450
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• 2022