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%

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

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

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

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1385 Date 2024-05-07

Discussion

No. of Applicants : 82