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Weekly Chest CasesArchive of Old Cases

Case No : 1341 Date 2023-07-03

  • Courtesy of Woo Hyeon Lim, Hyungin Park, Jong Hyuk Lee, Eui Jin Hwang, Soon Ho Yoon / Seoul National University Hospital
  • Age/Sex 61 / M
  • Chief ComplaintProptosis, visual acuity decrease
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Erdheim-Chester disease
Radiologic Findings
Fig 1. Chest PA shows interlobular septal thickening in both lower lung fields with right pleural effusion. Fig 2-4. CT scans reveal infiltrating soft tissue along aortic arch vessels, aorta, and retroperitoneum. Pericardial and bilateral pleural effusion was also noted.
Fig 5. On orbital MRI, homogeneously enhancing bilateral intraorbital lesions were found.
Fig 6. Right pleural effusion and interlobular septal thickening in both lungs were aggravated on follow-up CT scan.
Brief Review
Erdheim-Chester disease is a rare non-inherited, non-Langerhans cell histiocytosis and characterized by infiltration of lipid-laden histiocytes (foamy macrophages) showing CD68(+)/CD1a(-). Clinical manifestation could vary depending on the involved organ: various symptoms including bone pain, neurological signs, exophthalmos, retroperitoneal fibrosis, or dyspnea could be clinical manifestations of this disease depending on affected organs.
Musculoskeletal involvement is most common, and multifocal extraskeletal involvement is seen in 30-50%. Pulmonary manifestation is not common, and the lungs are affected in about 25% of cases. On chest CT, symmetric reticular abnormality, interlobular septal thickening, and multifocal ground-glass attenuation, centrilobular nodules could be pulmonary manifestations of Erdheim-Chester disease. In addition, pleural and pericardial effusion, cardiomegaly, peri-aortic infiltration, infiltration along other vessels (i.e. pulmonary artery and superior vena cava) were reported as thoracic manifestations of Erdheim-Chester disease. These radiologic abnormalities similar to interstitial edema pattern did not usually show response to diuretics. In upper abdomen, kidney, and retroperitoneal infiltration were often reported. In contrast to retroperitoneal fibrosis, inferior vena cava, and pelvic ureters are typically spared in this disease. Optic nerve edema, proptosis, or impaired eyeball movement could be caused by retro-orbital infiltration.
There had been try to treat Erdheim-Chester disease with steroids, radiotherapy, and chemotherapy, but treatment seemed to show little effect on this disease. Pulmonary fibrosis and cardiac failure are the most common cause of death.
References
1. Wittenberg KH, Swensen SJ, Myers JL. Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings. AJR Am J Roentgenol. 2000;174 (5): 1327-31.
2. Ahuja J, Kanne JP, Meyer CA et-al. Histiocytic disorders of the chest: imaging findings. Radiographics. 2015;35 (2): 357-70.
3. Chung JH, Park MS, Shin DH et-al. Pulmonary involvement in Erdheim-Chester disease. Respirology. 2005;10 (3): 389-92.
4. Shamburek RD, Brewer HB, Gochuico BR. Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease. Am. J. Med. Sci. 2001;321 (1): 66-75.
Keywords

No. of Applicants : 106

▶ Correct Answer : 56/106,  52.8%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan SUZUNE TSUKAMOTO
  • - , Japan HIROAKI ARAKAWA
  • - The University of Tokyo Hospital , Japan JUN KANZAWA
  • - , Japan KYOKO NAGAI
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - , United Kingdom SAMSON KADE
  • - Chonnam National University Hwasun Hospital , Korea (South) SEONGWOO CHO
  • - Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
  • - Seoul National University Hospital , Korea (South) HYUNGIN PARK
  • - Chonnam National University Hospital , Korea (South) HYUNJIN KIM
  • - Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
  • - Chonnam National University Hospital , Korea (South) JUNYEOL YANG
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - , Korea (South) JIN YOUNG LEE
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Inje University Pusan Paik Hospital , Korea (South) GANGWON JEONG
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Osaka University , Japan AKINORI HATA
  • - Ajou University Hospital , Korea (South) HYUNSEUNG LEE
  • - OITA UNIVERSITY , Japan FUMIKA ISHITOBI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
  • - Ajou University Hospital , Korea (South) JAYOUNG MOON
  • - Chonnam National University Hospital , Korea (South) JIWOO JEONG
  • - Hallym University Medical Center , Korea (South) JUNGMIN LIM
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Hukuda memorial hospital , Japan SHINTARO KANO
  • - Myongji Hospital , Korea (South) HYOUNG YEOB KIM
  • - , Japan KENTARO KOTANI
  • - Ajou University Hospital , Korea (South) YOU NA KIM
  • - Other , Korea (South) CHAEHUN LIM
  • - , India MANISH KUMAR JHA
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - , Japan SHUNJIRO NOGUCHI
  • - Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Eskisehir Sehir Hastanesi , Turkey MURAT SAHIN
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Seoul National University Hospital , Korea (South) SUNGHO HONG
  • - Chonnam National University Hospital , Korea (South) KIM SANG GYUN
  • - Kyoto University , Japan SHO KOYASU
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Chonbuk National University Hospital , Korea (South) SUBIN LEE
  • - Kantou Rousai Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Inje University Pusan Paik Hospital , Korea (South) GEOJEONG SEO
  • - Chonnam National University Hospital , Korea (South) SEUNG WAN KANG
  • - Toyota Kosei Hospital , Japan YUKI HAYASHI
  • - Kyoto university , Japan AKIHIKO SAKATA
  • - Seoul National University Hospital , Korea (South) YELIM CHOI
  • - Chonnam National University Hospital , Korea (South) EUN JU KIM
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 11/106,  10.4%
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - The University of Tokyo , Japan MOTO NAKAYA
  • - , Japan CHIAKI SATO
  • - Other , Korea (South) SEONGSU KANG
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - , Korea (South) HEONSEOK LEE
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - , Japan YUMI MAEHARA
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Juntendo University , Japan YUTAKA IKENOUCHI
▶ Semi-Correct Answer : 1/106,  0.9%
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
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