대한흉부영상의학회 Weekly Chest Cases

Case No : 1385 Date 2024-05-07

Courtesy of Pa Hong / Samsung Seoul Hospital
Age/Sex 58 / M
Chief ComplaintDyspnea

Figure 1
Figure 2
Figure 3
Figure 4
Figure 5

Diagnosis With Brief Discussion

Diagnosis: Erdheim-Chester disease (cardiopulmonary manifestations of Erdheim-Chester disease)
Radiologic Findings: Fig 1. Chest PA shows enlarged cardiac configuration and septal thickenings in bilateral lower lung zones.
Fig 2-4 Axial enhanced CT scans reveals circumferential periaortic infiltration, pericardial thickening with effusion, and perirenal infiltration.
Fig 5. Axial non-enhanced CT scan with lung window setting shows fissural and interlobular septal thickenings in bilateral lower lung zones.
Brief Review: Erdheim-Chester Disease (ECD) is a rare, systemic non-Langerhans cell histiocytosis. It is characterized by xanthomatous infiltrates of foamy histiocytes and predominantly affects middle-aged and older adults. ECD commonly presents with symmetrical bone pain, especially in the long bones. Extraskeletal involvement occurs in about 50% of patients, affecting organs such as the retroperitoneum, kidneys, lungs, central nervous system, orbit, skin, heart, breast, sinonasal mucosa, and skeletal muscles. Lung involvement is noted in 20%–35% of cases, leading to symptoms like cough and progressive dyspnea.
In the lungs, imaging typically shows smooth interlobular septal thickening and subpleural opacities, and additionally centrilobular nodules and areas of ground-glass opacity are also be seen.
Cardiovascular involvement is characterized by circumferential periaortic soft-tissue thickening, often referred to as a "coated aorta," which can extend into branch vessels and coronary arteries. Smooth pleural thickening with effusion and pericardial thickening and effusion are also common findings. In cases of other extraskeletal involvement, the affected organs may show specific changes, such as retroperitoneal, renal, or central nervous system abnormalities.
Progrosis of ECD is highly variable, ranging from stable to rapidly progressive and fatal extraosseous disease. Prognosis is generally poorer in cases with neurologic or cardiac involvement.
References: 1. Jitesh Ahuja, Jeffrey P. Kanne, Cristopher A. Meyer, Sudhakar N. J. Pipavath, Rodney A. Schmidt, Jonathan O. Swanson, and J. David Godwin. Histiocytic Disorders of the Chest: Imaging Findings. RadioGraphics 2015 35:2, 357-370
Keywords

No. of Applicants : 82

▶ Correct Answer : 60/82, 73.2%

- University of Yamanashi , Japan KOJIRO ONOHARA
- Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
- Jiangsu province hospital , China WANGJIAN ZHA
- Other , Korea (South) SEONGSU KANG
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- Kyoto University , Japan AKIHIKO SAKATA
- Osaka metropolitan university Hospital , Japan TATSUSHI OURA
- Fukuoka university , Japan KEISUKE SATO
- The University of Tokyo Hospital , Japan JUN KANZAWA
- Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
- Uniyersity of Yamanashi , Japan TAKAAKI HASHIMOTO
- Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
- Kyoto university , Japan HIROKAZU SAWAMURA
- McGill University Health Center , Canada ALEXANDRE SEMIONOV
- , Japan KENTARO KOTANI
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
- Oita University, Faculty of Medicine , Japan FUMITO OKADA
- University of Yamanashi , Japan HIROYUKI MORISAKA
- Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
- Other , Korea (South) JY LEE
- , Japan SUZUNE TSUKAMOTO
- GHICL , France MANUEL TOLEDANO
- Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
- Shiga General Hospital , Japan YUSAKU MORIBATA
- medical scanning , Japan HIROAKI ARAKAWA
- Paras hospital, Panchkula , India SHALEEN RANA
- Mie University , Japan HIKARI FUKUI
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Matsunami General Hospital , Japan TARO TAKEDA
- Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
- , Korea (South) JIN WOO YOON
- , Korea (South) KANGHWI LEE
- , Korea (South) JIN YOUNG LEE
- , Viet Nam PHI THAI PHAM
- Gifu University Hospital , Japan Yo Kaneko
- Shiga University of Medical Science , Japan AKITOSHI INOUE
- Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
- , Korea (South) HEONSEOK LEE
- , France LAURA RAMIREZ
- , Japan SHUNJIRO NOGUCHI
- the first affiliatited hospital of nanjing medical univercity , China HAI XU
- Osaka University , Japan AKINORI HATA
- , Korea (South) DONG-HO BANG
- , Japan YOSHIKI ISHII
- Kantou Rousai Hospital , Japan KAORU SUMIDA
- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- Kyoto University Hospital , Japan YASUHISA KURATA
- , Senegal AXEL MBUYU KAYEMBE
- Oita university , Japan AYUMI KAMEI
- Toyota Kosei Hospital , Japan YUKI HAYASHI
- Secomedic Hospital , Japan FUMINORI MIYOSHI
- University of Yamanashi , Japan HIROAKI WATANABE
- Kyoto University , Japan SHO KOYASU
- ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
- Mie university , Japan SHIKO OKABE
- Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
- Other , Korea (South) CHAEHUN LIM
- Nerimahikarigaoka hospital , Japan TAKANA HAYASHI

▶ Correct Answer as Differential Diagnosis : 6/82, 7.3%

- The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
- Other , Korea (South) HYEJOO PARK
- Juntendo University , Japan YUTAKA IKENOUCHI
- Dokkyo Medical University , Japan HIROAKI ARAKAWA
- Ome Medical Center , Japan MIKIYA FUJII
- Mie university , Japan MIYUKO FUJITA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1385 Date 2024-05-07

Discussion

No. of Applicants : 82