• Tuberculosis and Respiratory Diseases
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• v.77 no.2
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• pp.85-89
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• 2014

Intrathoracic involvement of immunoglobulin G4 (IgG4)-related disease has recently been reported. However, a subset of the disease presenting as interstitial lung disease is rare. Here, we report a case of a 35-year-old man with IgG4-related lung disease with manifestations similar to those of interstitial lung disease. Chest computed tomography showed diffuse ground glass opacities and rapidly progressive pleural and subpleural fibrosis in both upper lobes. Histological findings showed diffuse interstitial lymphoplasmacytic infiltration with an increased number of IgG4-positive plasma cells. Serum levels of IgG and IgG4 were also increased. The patient was diagnosed with IgG4-related lung disease, treated with anti-inflammatory agents, and showed improvement. Lung involvement of IgG4-related disease can present as interstitial lung disease and, therefore, should be differentiated when evaluating interstitial lung disease.

• 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 the Korean Association of Oral and Maxillofacial Surgeons
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• v.46 no.1
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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.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.1168-1174
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• 2022

Immunoglobulin G4 (IgG4)-related disease is a rare systemic fibroinflammatory condition characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in various organs. IgG4-related lung disease shows varied radiologic features on chest CT. Patients usually present with a solid nodule or mass mimicking lung cancer; therefore, distinguishing between IgG4-related disease and other conditions is often challenging. Additionally, co-existing radiologic findings of IgG4-related lung disease may mimic metastasis or lymphangitic carcinomatosis of the lung. We report two cases of histopathologically confirmed IgG4-related lung disease mimicking lung cancer. Chest CT revealed a solid nodule or mass with ancillary radiologic findings, which suggested lung cancer; therefore, IgG4-related lung disease was radiologically indistinguishable from lung cancer in both cases. Measurement of serum IgG4 levels and clinical evaluation to confirm involvement of various organs may be useful to establish the differential diagnosis. However, surgical biopsy evaluation is needed for confirmation.

• Journal of the Korean Society of Radiology
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• v.79 no.5
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• pp.276-281
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• 2018

Immunoglobulin G4 (IgG4)-related lung disease has been actively studied over the past few years. Radiologic findings of IgG4-related lung disease vary among patients, but there are no long-term follow up studies regarding variations in imaging features over the course of disease progression. In two cases with relatively long-term follow up, diverse early and late computed tomography (CT) findings of IgG4-related lung disease are reviewed in this report. In contrast to nodular or diffuse ground-glass opacity, which was predominantly noted in CT scans at earlier stages of disease, honeycombing and traction bronchiectasis were regarded as late radiologic manifestations. Solid nodules might be visible in both early and late stages; however, development of new solid nodules and enlargement of preexisting nodules could occur during disease progression. Interlobular septal thickening and mediastinal/hilar lymphadenopathy were persistent, even in later stages of the disease. These findings might be useful in making an accurate and timely diagnosis of IgG4-related lung 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 Korean Neurosurgical Society
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• v.55 no.5
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• pp.300-302
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• 2014

Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Recently, the possibility that IgG4-related sclerosing disease may underlie some cases of intracranial hypertrophic pachymeningitis has been suggested. We herein report the tenth case of IgG4-related intracranial hypertrophic pachymeningitis and review the previous literature. A 45-year-old male presented with left-sided focal seizures with generalization. Magnetic resonance imaging (MRI) revealed a diffuse thickening and enhancement of the right convexity dura matter and falx with focal nodularity. The surgically resected specimens exhibited the proliferation of fibroblast-like spindle cells and an infiltration of mononuclear cells, including predominantly plasma cells. The ratio of IgG4-positive plasma cells to the overall IgG-positive cells was 45% in the area containing the highest infiltration of plasma cells. On the basis of the above findings, IgG4-related sclerosing disease arising from the dura mater was suspected. IgG4-related sclerosing disease should be added to the pachymeningitis spectrum.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.826-837
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• 2021

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition involving multiple organs, including the salivary or lacrimal glands, orbit, pancreas, bile duct, liver, kidney, retroperitoneum, aorta, lung, and lymph nodes. It is histologically characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, storiform fibrosis, and obliterative phlebitis. In the thoracic involvement of IgG4-RD, mediastinal lymphadenopathy and perilymphangitic interstitial thickening of the lung are the most common findings. Peribronchovascular and septal thickening and paravertebral band-like soft tissue are characteristic findings of IgG4-RD. Other findings include pulmonary nodules or masses, ground-glass opacity, alveolar interstitial thickening, pleural effusion or thickening, mass in the chest wall or mediastinum, and arteritis involving the aorta and coronary artery. Radiologic differential diagnosis of various malignancies, infections, and inflammatory conditions is needed. In this review, we describe the imaging findings of IgG4-RD and the radiologic differential diagnoses in the thorax.

• Journal of the Korean Society of Radiology
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• v.84 no.3
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• pp.719-725
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• 2023

Immunoglobulin G4 (IgG4)-related disease is a rare systemic fibroinflammatory condition characterized by organomegaly or tumefactive lesions associated with lymphoplasmacytic infiltration rich in IgG4 plasma cells. We report a case of IgG4-related disease involving the subcutaneous layer of the left upper arm in a 48-year-old female presenting with an unusual soft tissue mass. US and MRI showed an irregular infiltrative soft tissue mass, indicating possible malignancy or inflammation. We discuss the diagnostic criteria, histopathologic features, radiological features, and treatment of IgG4-related disease.