• Journal of the Korean Society of Radiology
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• v.82 no.3
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• pp.575-588
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• 2021

Immunoglobulin G4 (IgG4)-related disease is a systemic fibro-inflammatory disease characterized by pathologic findings in various organs. Imaging is critical for the diagnosis and treatment assessment of patients with IgG4-related disease. In this pictorial essay, we review the key features of multiple imaging modalities, typical pathologic findings, and differential diagnosis of IgG4-related disease. This systematic pictorial review can further our understanding of the broad-spectrum manifestations of this disease.

• 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.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.

• Journal of the Korean Society of Radiology
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• v.82 no.2
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• pp.487-492
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• 2021

Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by dense lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and fibroblast proliferation. The retroperitoneal involvement of IgG4-related disease usually appears as a soft-tissue mass covering the abdominal aorta or entrapping the ureters, resulting in hydronephrosis. Here, we present a case of IgG4-related disease with retroperitoneal involvement in a 75-yearold woman with an unusual manifestation. A preoperative computed tomography (CT) scan revealed an irregular infiltrative retroperitoneal mass invading the normal anatomic barriers, raising the suspicion of malignancy or inflammation. Contrast-enhanced CT revealed a homogeneous progressive enhancement of the mass.

• Korean Journal of Head & Neck Oncology
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• v.36 no.2
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• pp.27-31
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• 2020

Neck mass has various etiologies, including inflammatory, congenital, neoplastic causes. The IgG4-related disease can cause symptoms in the head and neck areas with an inflammatory neck mass. It also shows clinical and pathological findings from inflammation caused by immune reactions, such as lymphocyte and plasma cell infiltration, storiform fibrosis, obliteration phlebitis, and invigorated serum IgG4 levels. The treatment guideline has not been established and still under debate, but systemic glucocorticoid seems to be effective in the most cases. In this brief report, a 48-year-old male patient presented with voice change for 3 weeks. Left side paramedian vocal fold palsy was observed in the flexible laryngoscopy. About 2.5×2.0×1.2cm size, heterogeneously enhanced neck mass with irregular margin encasing left carotid artery was noted on preoperative contrast enhanced neck CT scan, and it was suspicious of left carotid body tumor. The pathology shows IgG4-related disease rather than carotid body tumors. We report this case of IgG4-related disease, which can be misdiagnosed to carotid body tumors.

• 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.33 no.1
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• pp.40-43
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• 2016

Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.

• 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.

• Journal of Yeungnam Medical Science
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• v.39 no.2
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• pp.153-160
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• 2022

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

• 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.