• Journal of Yeungnam Medical Science
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• 제39권2호
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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.

• 대한영상의학회지
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• 제82권4호
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• pp.826-837
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• 2021

면역글로불린G4 연관 질환(immunoglobulin G4-related disease; 이하 IgG4-RD)는 IgG4를 생산하는 면역세포에 의한 만성 염증성질환으로, 주로 타액선, 누액선, 안와, 췌장, 담도, 간, 신장, 후복막, 대동맥, 폐, 림프절 등 다양한 장기를 침범하고, 조직학적으로 IgG4 양성 형질세포와 림프구의 침윤 및 나선형의 섬유화(storiform fibrosis), 폐색정맥염(obliterative phlebitis)을 특징으로 한다. IgG4-RD의 흉부 침범에서 가장 흔한 소견은 종격동 림프절 비대와 폐의 림프관주위 간질 비후이다. 폐의 기관지혈관주위 간질 비후와 우측 척추곁 밴드형 연부조직은 IgG4-RD의 특징적 소견이고, 그 외에도 폐결절 혹은 종괴, 간유리음영, 폐포 간질비후, 흉막삼출 및 비후, 흉벽이나 종격동 종괴, 대동맥과 관상동맥의 혈관염이 발생할 수 있다. 영상의학적으로는 악성 종양이나 감염 및 다양한 염증성질환과의 감별진단이 필요하다. 본 연구에서는 흉부에서 발생하는 IgG4-RD의 영상 소견과 감별진단에 대해 기술하였다.

• Tuberculosis and Respiratory Diseases
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• 제76권1호
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• pp.42-45
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• 2014

An immunoglobulin G4 (IgG4)-related disease is a recently emerging entity, and a few cases of IgG4-related disease in lung and pleura have been reported. Herein, we report the case of a 74-year-old man with IgG4-related disease of lung and pleura, clinically suspicious of malignant mesothelioma. Chest computed tomography showed diffuse nodular pleural thickening, and microscopic finding disclosed diffuse thickening of visceral pleura with infiltrations of many lymphoplasma cells with increased number of IgG4-positive plasma cells and a few multinucleated giant cells. It is important for pathologists and clinicians to recognize this rare entity and its histologic finding, because it can be confused with malignant tumors on the radiologic examination although it can be treated with steroid therapy.

• 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.

• Tuberculosis and Respiratory Diseases
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• 제67권4호
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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.

• 대한영상의학회지
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• 제84권3호
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• pp.686-691
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• 2023

면역글로불린(immunoglobulin; 이하 Ig) G4 연관 질환은 어떤 장기도 침범할 수 있는 섬유염증성 질환이며, 심근염의 형태로 발현되는 경우는 매우 드물다. 호흡곤란과 흉통이 있는 52세 남성이 심장자기공명영상에서 좌심실벽에 부종 및 심장간막과 심내막에 반점형과 결절형 지연 조영증강을 보이는 심근염 소견을 보였다. 혈액검사상 혈청 IgG4 상승과 호산구 증가증이 동반되었으며 심장 생검으로 호산구성 심근염과 IgG4 양성 세포가 확인되었다. 이에 저자들은 IgG4 연관 질환이 호산구성 심근염으로 발현된 드문 증례를 보고하고자 한다.

• 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.

• 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.

• 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.

• Tuberculosis and Respiratory Diseases
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• 제77권2호
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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.