Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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Immunoglobulin G4-Related Disease in the Thorax: Imaging Findings and Differential Diagnosis

흉부에서 발생한 IgG4 연관 질환: 영상 소견 및 감별진단

  • Yookyung Kim (Department of Radiology, Ewha Womans University, College of Medicine) ;
  • Hye Young Choi (Department of Radiology, Ewha Womans University Mokdong Hospital)
  • 김유경 (이화여자대학교 의과대학 영상의학교실) ;
  • 최혜영 (이화여자대학교 목동병원 영상의학과)
  • Received : 2021.05.04
  • Accepted : 2021.06.15
  • Published : 2021.07.01
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Abstract

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.

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.

Keywords

Acknowledgement

All authors appreciate Eun Ju Chun MD from Seoul National University Bundang Hospital and Yoon Kyung Kim MD from Samsung Medical Center who provided interesting cases for this review article.

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