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Case 245: Erdheim-Chester Disease.

Abstract History A 53-year-old man experienced headache and double vision that progressed over 1 year. After a traumatic fall, he was hospitalized, and proptosis was identified at physical examination. Laboratory tests were remarkable for leukocytosis. Hematocrit level, thyroid stimulating hormone level, autoimmune antibody level, erythrocyte sedimentation rate, and C-reactive protein level were normal. Computed tomography (CT) of the head revealed bilateral intraconal masses, for which magnetic resonance (MR) imaging of the orbits was subsequently performed ( Fig 1 ). CT imaging of the chest and abdomen ( Fig 2 ) revealed periaortic and retroperitoneal stranding. Perinephric biopsy was performed, and a diagnosis of immunoglobulin G4 (IgG4)-related disease was made based on identification of a few plasma cells per high-power field that were positive for IgG4. Orbital biopsy was then performed, but the results were inconclusive for IgG4-related disease. The patient was discharged and given steroid therapy for presumed IgG4-related disease. [Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text] Several months later, the patient returned to our institution with progressive symptoms despite ongoing steroid treatment. His case was reviewed by several specialists to develop alternative treatments for IgG4-related disease. After review of the available images, a neuroradiology fellow (M.D.M.) performed history taking and a physical examination and subsequently recommended radiography of the lower extremities ( Fig 3 ). [Figure: see text][Figure: see text].
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title radiology
Publication Year Start




PMID- 28825890
OWN - NLM
STAT- In-Process
DA  - 20170821
LR  - 20170821
IS  - 1527-1315 (Electronic)
IS  - 0033-8419 (Linking)
VI  - 284
IP  - 3
DP  - 2017 Sep
TI  - Case 245: Erdheim-Chester Disease.
PG  - 910-917
LID - 10.1148/radiol.2017141151 [doi]
AB  - History A 53-year-old man experienced headache and double vision that progressed 
      over 1 year. After a traumatic fall, he was hospitalized, and proptosis was
      identified at physical examination. Laboratory tests were remarkable for
      leukocytosis. Hematocrit level, thyroid stimulating hormone level, autoimmune
      antibody level, erythrocyte sedimentation rate, and C-reactive protein level were
      normal. Computed tomography (CT) of the head revealed bilateral intraconal
      masses, for which magnetic resonance (MR) imaging of the orbits was subsequently 
      performed ( Fig 1 ). CT imaging of the chest and abdomen ( Fig 2 ) revealed
      periaortic and retroperitoneal stranding. Perinephric biopsy was performed, and a
      diagnosis of immunoglobulin G4 (IgG4)-related disease was made based on
      identification of a few plasma cells per high-power field that were positive for 
      IgG4. Orbital biopsy was then performed, but the results were inconclusive for
      IgG4-related disease. The patient was discharged and given steroid therapy for
      presumed IgG4-related disease. [Figure: see text][Figure: see text][Figure: see
      text][Figure: see text][Figure: see text][Figure: see text] Several months later,
      the patient returned to our institution with progressive symptoms despite ongoing
      steroid treatment. His case was reviewed by several specialists to develop
      alternative treatments for IgG4-related disease. After review of the available
      images, a neuroradiology fellow (M.D.M.) performed history taking and a physical 
      examination and subsequently recommended radiography of the lower extremities (
      Fig 3 ). [Figure: see text][Figure: see text].
FAU - Mamlouk, Mark D
AU  - Mamlouk MD
AD  - From the Department of Radiology and Biomedical Imaging, University of
      California, San Francisco, San Francisco, Calif.
FAU - Aboian, Mariam S
AU  - Aboian MS
AD  - From the Department of Radiology and Biomedical Imaging, University of
      California, San Francisco, San Francisco, Calif.
FAU - Glastonbury, Christine M
AU  - Glastonbury CM
AD  - From the Department of Radiology and Biomedical Imaging, University of
      California, San Francisco, San Francisco, Calif.
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Radiology
JT  - Radiology
JID - 0401260
EDAT- 2017/08/22 06:00
MHDA- 2017/08/22 06:00
CRDT- 2017/08/22 06:00
AID - 10.1148/radiol.2017141151 [doi]
PST - ppublish
SO  - Radiology. 2017 Sep;284(3):910-917. doi: 10.1148/radiol.2017141151.