대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesCases by Disease Category

Case No : 1184 Date 2020-07-05

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  • Courtesy of Suji Lee, Hye-Jeong Lee / Severance Hospital, Yonsei University College of Medicine
  • Age/Sex 69 / M
  • Chief ComplaintDyspnea on exertion / PHx: old CVA
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
IgG4-related aortitis/periaortitis
Radiologic Findings
Fig 1. Chest PA shows fibrostreaky opacity in left upper lobe and right cardiophrenic angle blunting.
Fig 2-4. CT scans shows diffuse infiltrative soft tissue encasing aortic arch and its branches extended to descending aorta. Soft tissue attenuation infiltrates right pleural is also noted.
Fig 5. PET-CT scans show intense FDG uptake of the infiltrative soft tissues.
Brief Review
IgG4-related disease(IgG4-RD) is a systemic inflammatory disease characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells on histopathologic examination. Clinical manifestations of this disease have been demonstrated in nearly every organ system. Hepato-pancreato-biliary infiltration, retroperitoneal fibrosis and/or aortitis, lymphadenopathy, and Mikulicz disease (IgG4related dacryoadenitis and sialadenitis) are common systemic involvements of this disease, with IgG4-related aortitis/periaortitis and periarteritis being its vascular manifestations.

Computed tomography (CT) findings of IgG4-RD include arterial wall thickening and homogeneous wall enhancement. The dissection of the great vessels and myocardial ischemia due to coronary artery involvement are fatal manifestations of IgG4-RD. Fluorodeoxyglucose(FDG) positron-emission tomography (PET)-CT shows increased FDG uptake in multiple involved organs. The differential diagnosis includes inflammatory aortic aneurysm, lymphoma, large-vessel vasculitis such as Takayasu arteritis. Systemic involvement is also important for diagnosis of this disease. Recommended treatment of IgG4-RD is corticosteroid with conservative treatment.
References
1. Horger M, Lamprecht H-G, Bares R, Spira D, Schmalzing M, Claussen CD, et al. Systemic IgG4-related sclerosing disease: spectrum of imaging findings and differential diagnosis. American Journal of Roentgenology. 2012;199(3):W276-W82.

Keywords
thoracic aorta, IgG4 related disease, aortitis,

No. of Applicants : 71

▶ Correct Answer : 14/71,  19.7%
  • - The University of Kyoto Hospital , Japan TOMOAKI OTANI
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - TWMU Medical Center East , Japan MASAFUMI KAIUME
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - Chungbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Other , Korea (South) DONGYOUNG JEONG
  • - Chonnam National University Hospital , Korea (South) SEUNGYOON CHAE
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - , India SUSHANT JOSHI
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 24/71,  33.8%
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Asan Medical Center , Korea (South) EUNJI CHOI
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Scans world , India PHILSON JOSEPH MUKKADA
  • - DELTA CARE HOSPITAL, THANJAVUR, INDIA , India SIVARAJA SUBRAMANIAM
  • - St. Lukes International Hospital , Japan DAISUKE YAMADA
  • - The Jikei university , Japan TAKU GOMI
  • - Hamamatsu University Hospital , Japan HAYATO NOZAWA
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Gifu University Hospital , Japan Yo Kaneko
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Korea University Ansan Hospital , Korea (South) MOIN HA
  • - Osaka City General Hospital , Japan SHU MATSUSHITA
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Asan Medical Center , Korea (South) JIHOON KIM
  • - Kitano Hospital , Japan SATOSHI IKEDA
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - , Japan KAZUMA TERAUCHI
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - Chonnam National University Hwasun Hospital , Korea (South) WONGI JEONG
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
▶ Semi-Correct Answer : 3/71,  4.2%
  • - Hereford County Hospital , United Kingdom JOY A THOMAS
  • - Mayo Clinic , Japan HIROAKI TAKAHASHI
  • - , Japan YUMI MAEHARA
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