• Journal of the Korean Society of Radiology
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• v.83 no.4
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• pp.910-917
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• 2022

Immunoglobulin G4 (IgG4) related disease (IgG4-RD) is currently considered an immune-mediated condition and is recognized as a disorder group with common pathological, hematological, and clinical characteristics. This disease may involve diverse organs of the head and neck, and include mainly the lacrimal gland, orbit, thyroid gland, pituitary gland, and the meninges. Here, we report a case of IgG4-RD in a 65-year-old female showing head- and neck-limited but synchronously and mainly manifesting as otalgia and facial neuritis.

• Journal of the Korean Society of Radiology
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• v.84 no.3
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• pp.757-762
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• 2023

First described in 1930 as a lipoid granulomatosis, Erdheim-Chester disease (ECD) is a rare histiocytosis encompassing a group of disorders caused by overproduction of histiocytes, a subtype of white blood cells. This disease most commonly involves the bones and can affect organs in the abdomen; however, biliary involvement is rarely reported. We report a case of ECD with biliary involvement, which rendered it difficult to radiologically distinguish ECD from immunoglobulin G4-related disease.

• Journal of the Korean Society of Radiology
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• v.84 no.3
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• pp.686-691
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• 2023

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fibroinflammatory disorder that can involve any organ system; however, myocarditis is extremely rare. A 52-year-old male with dyspnea and chest discomfort underwent cardiac MRI that revealed edema and nodular, patchy, mesocardial and subendoardial delayed enhancement of left ventricle, suggesting myocarditis. Laboratory findings revealed elevated serum IgG4 and eosinophilia. Cardiac biopsy confirmed eosinophilic myocarditis with IgG4-positive cells. Here, we present an unusual case of IgG4-RD manifesting as eosinophilic myocarditis.

• 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 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 the Korean Society of Radiology
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• v.85 no.5
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• pp.954-959
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• 2024

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition characterized by mass-forming inflammation with a sclerosing pattern that can affect nearly any organ. However, involvement of the diaphragm in IgG4-RD is exceptionally rare. We present the case of a 62-year-old male patient with chest radiographic abnormalities. Further investigation with CT revealed an infiltrative mass in the right hemidiaphragm. This mass, composed of engorged feeding vessels, an atypical manifestation of IgG4-RD, was also associated with lymphadenopathy. Surgical excision confirmed the presence of IgG4-positive cell infiltration, solidifying the diagnosis of IgG4-RD. Notably, the patient remained asymptomatic and did not require any treatment postoperatively. This case highlights the uncommon presentation of IgG4-RD as an infiltrative diaphragmatic mass.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.48-52
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• 2006

Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.67 no.4
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• pp.359-363
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• 2009

Immunoglobulin G4 (IgG4) related autoimmune diseases are characterized by high serum IgG4 concentrations, sclerosing inflammation of numerous IgG4-positive lymphoplasma cells of varying origin, and a positive response to steroid treatment. Autoimmune pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis are representative presentations of IgG4 related autoimmune disease. Herein, we describe 2 patients (40-years-old woman and 47-years-old man) diagnosed with pulmonary involvement of IgG4-related autoimmune disease. The patients were admitted for an evaluation of the lung mass or multiple lung nodules found on chest radiography. Surgical lung biopsies were performed and pathologic finding revealed lymphoplasmacytic sclerosing inflammation with numerous IgG4 positive cells. The patients had elevated serum total IgG and IgG4 levels. Treatment consisted of high dose methylpredinisolone (1 mg/kg/day) and demonstrated good responsiveness. However, one patient experienced 2 relapses while being tapered off of steroid treatment.