대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesCases by Disease Category

Case No : 1488 Date 2026-04-27

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  • Courtesy of Minsun Koo, Jin Young Yoo, Bae Young Lee / Eunpyeong St. Mary
  • Age/Sex 62 / F
  • Chief ComplaintCough, sputum for 1 month
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7

Diagnosis With Brief Discussion

Diagnosis
IgG4-related disease
Radiologic Findings
Fig 1. Chest PA shows no remarkable abnormal finding in both lungs and mediastinal contour.
Fig 2-7. CT scans reveals diffuse smooth circumferential wall thickening of trachea and both bronchi in both central lung areas, involving tracheal posterior membrane.
Brief Review
IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterized by focal or diffuse organ infiltration of IgG4-bearing plasma cells. Thoracic involvement has been reported in up to 30% of patients and may affect all thoracic compartments, including the mediastinum, airways, lung parenchyma, and pleurae, with the mediastinum and lung parenchyma being the most frequently involved sites. Based on the literature, four radiologic patterns of lung involvement in IgG4-RD have been described: a solid nodular type, a round-shaped ground-glass opacity type, an alveolar interstitial type, and a bronchovascular type. The bronchovascular type is characterized by thickening of the bronchovascular bundles and interlobular septa on imaging, corresponding histologically to lymphoplasmacytic infiltration with stromal fibrosis along the peribronchial glands and interstitium, while sparing the bronchial epithelium.
In contrast, central airway involvement of IgG4-RD is relatively rare. It may present as diffuse wall thickening of the central airways, with or without associated airway stenosis, and requires differentiation from other conditions such as amyloidosis, granulomatosis with polyangiitis, and sarcoidosis.
Thoracic manifestations of IgG4-RD may occur either in isolation or in association with involvement of other organs. Patients may be asymptomatic or may present with respiratory symptoms including cough, dyspnea, hemoptysis, chest pain, or respiratory failure. Systemic corticosteroid therapy has been shown to improve both clinical symptoms and radiologic abnormalities.
References
1. Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Kobayashi T, Yoshikawa J, Miyayama S, Yasui M, Nakanuma Y, Matsui O. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology. 2009 Apr;251(1):260-70. doi: 10.1148/radiol.2511080965. Epub 2009 Feb 12. PMID: 19221056.
2. Muller R, Ebbo M, Habert P, Daniel L, Briantais A, Chanez P, Gaubert JY, Schleinitz N. Thoracic manifestations of IgG4-related disease. Respirology. 2023 Feb;28(2):120-131. doi: 10.1111/resp.14422. Epub 2022 Nov 27. PMID: 36437514; PMCID: PMC10100266.
3. Wheeler S, Andeen N, Reddy R. Isolated IgG4 related disease of the trachea. Respir Med Case Rep. 2024 Apr 28;49:102031. doi: 10.1016/j.rmcr.2024.102031. PMID: 38712313; PMCID: PMC11070757.
4. Mart
Keywords

No. of Applicants : 77

▶ Correct Answer : 3/77,  3.9%
  • - The Catholic University of Korea Seoul St. Mary , Korea (South) JIHO PARK
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) OHMIN KWON
  • - , Korea (South) JIYUN KIM
▶ Correct Answer as Differential Diagnosis : 20/77,  26.0%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROKI SAKURADA
  • - Kyoto University , Japan AKIHIKO SAKATA
  • - Oita university , Japan AYUMI KAMEI
  • - Other , Korea (South) SEONGSU KANG
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - University of Yamanashi , Japan HIROYUKI MORISAKA
  • - Chiba University Hospital , Japan HIROKI MIYAZAKI
  • - Teikyo University Hospital, Mizonokuchi , Japan TOSHIHIRO FURUTA
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - , Japan SHUNJIRO NOGUCHI
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - , Japan TOMOHIRO KAWAJI
  • - Kobe Children , Japan SHUHEI NORIMOTO
  • - Kantou Rousai Hospital , Japan KAORU SUMIDA
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Ehime University , Japan KOTARO MATSUMOTO
  • - University of Yamanashi , Japan KOJIRO ONOHARA
  • - , Japan YUJI OHIZUMI
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