대한흉부영상의학회 Weekly Chest Cases

Case No : 744 Date 2012-01-31

Courtesy of Sang Young Oh, Mi Young Kim / Asan Medical Center
Age/Sex 68 / M
Chief ComplaintFitigue. History of central diabetes insipidus (1 year ago).

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Figure 1.

Diagnosis With Brief Discussion

Diagnosis: Erdheim-Chester disease
Radiologic Findings: Posteroanterior chest radiograph shows diffuse reticular interstitial thickening throughout both lungs and bilateral pleural effusion. Chest CT scans show presence of periaortic infiltration extending from aortic arch to abdominal aorta, bilateral perirenal infiltration, and pericardial effusion.
Brief Review: Erdheim-Chester disease is a rare form of non-Langerhans histiocytosis of unknown etiology. Erdheim-Chester disease is characterized by tissue infiltration by foamy histiocytes (xanthogranulomatosis) with immunohistochemical characteristics different from those of Langerhans cell histiocytosis.
Erdheim-Chester disease is characterized by heterogeneous systemic manifestations. Bone pain is the most frequent symptom. Bone involvement consists of symmetric osteosclerosis of the long tubular bones, predominating in the metaphysic and diaphysis of the lower limbs. About half of all patients have extraskeletal manifestations including exophthalmos, diabetes insipidus, xanthelasma, interstitial lung disease, bilateral adrenal enlargement, retroperitoneal fibrosis with perirenal and/or ureteral obstruction, renal impairment, testis infiltration, central nervous system, and/or cardiovascular involvement.
Symmetric and bilateral peri- and pararenal soft-tissue infiltration (hairy kidney) extending to the adrenal glands and renal pedicles or circumferential periaortic infiltration from the aortic arch to the primary iliac artery origin (coated aorta) or both are suggestive of Erdheim-Chester disease. The periaortic infiltration may extend to the ascending aorta, coronary arteries, and aortic branches, with risk of vascular stenosis. Infiltration of the pulmonary arteries, superior vena cava, pericardium, and myocardium may also occur. When pulmonary involvement is present, a constellation of CT findings is commonly suggestive of the diagnosis.
There is no proven therapy, although variable success with interferon-alpha and chemotherapeutic agents have been reported. Average survival periods reported 32 months.
References: 1. Devouassoux G, Lantuejoul S, Chatelain P, et al. Erdheim-Chester disease: a primary macrophage cell disorder. Am J Respir Crit Care Med 1998; 157:650-653
2. Shamburek RD, Brewer HB, Jr., Gochuico BR. Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease. Am J Med Sci 2001; 321:66-75
3. Murray D, Marshall M, England E, et al. Erdheim-chester disease. Clin Radiol 2001; 56:481-484
4. Braiteh F, Boxrud C, Esmaeli B, et al. Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. Blood 2005; 106:2992-2994
5. Haroche J, Amoura Z, Trad SG, et al. Variability in the efficacy of interferon-alpha in Erdheim-Chester disease by patient and site of involvement: results in eight patients. Arthritis Rheum 2006; 54:3330-3336
Keywords: multiple, lymphoproliferative disease,

No. of Applicants : 106

▶ Correct Answer : 41/106, 38.7%

- korea university , Korea (South) Hyelarn Lee
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- University of British Columbia , Canada Amr Ajlan
- Korea university , Korea (South) SUMIN HA
- CHU NANCY , France, Metropolitan LEGOU Francois
- CH de la C�te Basque , France Paul ARDILOUZE
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- CHU LYON sud , France matthieu ROUSSET
- Bupyong Serim Hospital , Korea (South) Hee Seok Choi
- Kyungpook National University Hospital , Korea (South) Jaekwang Lim
- chp st martin , France Mariotte benoit
- McGill University Health Center , Canada Alexandre Semionov
- Radnet Teleradiology Istanbul , Turkey Armagan Sarac
- Montreal , Canada D J
- Kasr alaini , Egypt Ziad Moqbel
- Kaser El-Aini medical hospital , Egypt hamdi turkey
- Kyung Hee Universitiy Hospital at Gangdong , Korea (South) Seong Jong Yun
- UWO , Canada S Lee
- Suncheon Plus Medicine , Korea (South) Park Hyun
- China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
- HUEC , Brazil Diogo Pinheiro
- IRSA La Rochelle France , France Denis Chabassiere
- royal melbourne hospital , Australia kimchi kings
- McGill University , Canada Ben Smith
- CHRU lille , France manuel toledano
- AMCH , India hage tadu
- Japanese Red Cross Society Himeji Hospital , Japan Yuichiro Kanie
- Seoul St Mary Hospital , Korea (South) Chae Lim
- Hanyang university medical center , Korea (South) YOONAH SONG
- Harihar Hospital & Research Centre, Kullu (H.P.) , India Maneesh Verma
- Yonsei uni Severance hospital , Korea (South) Seok Ko
- Ruby Hall Clinic , India sudarshan rawat
- Beaulieu clinic Geneva , Switzerland gilles GENIN
- IRSA , France, Metropolitan BIGOT
- aarthi scans , India abirami mahadevan
- IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
- All India Institute of medical sciences , India Justin Moses
- Severance hospital , Korea (South) Saerom Hong
- universidad de chile , Chile enrique aguilera
- KUMC ansan , Korea (South) kihwan kim
- NASA SCANS , India RAKESH BHATIA

▶ Correct Answer as Differential Diagnosis : 8/106, 7.5%

- Az Osp Santa Maria Terni , Italy angelo carloni
- James Paget Hospital , U.K , United Kingdom nabil mahmood
- All India Institute of Medical Sciences , India Jitesh Ahuja
- jaslok hospital & research centre mumbai , India JAINENDRA JAIN
- Chonnam National University Hospital , Korea (South) Jung hyun Kim
- Kizawa Memorial Hospital , Japan Yo Kaneko
- Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani
- onomitisiritushiminnbyouinn , Japan HIdeyuki Kobayashi

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 744 Date 2012-01-31

Discussion

No. of Applicants : 106