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Erdheim-Chester disease with vascular involvement mimics large vessel vasculitis
  1. Christin Windisch1,
  2. Iver Petersen2,
  3. Birte Schulz2,
  4. Thomas Winkens3,
  5. Eric Lopatta4,
  6. Peter Oelzner1,
  7. Gunter Wolf1,
  8. Thomas Neumann1
  1. 1Department of Internal Medicine III, Jena University Hospital, Jena, Germany
  2. 2Institute of Pathology, Jena University Hospital, Jena, Germany
  3. 3Clinic of Nuclear Medicine, Jena University Hospital, Jena, Germany
  4. 4Institute of Diagnostic and Interventional Radiology II, Jena University Hospital, Jena, Germany
  1. Correspondence to Dr Christin Windisch, Department of Internal Medicine III, Jena University Hospital, Erlanger Allee 101, Jena D-07747, Germany; Christin.Windisch{at}

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Case report

A 70-year-old woman was admitted to the hospital with a 3-year history of progressively reduced general health, vertigo, joint pain and exertional dyspnoea. Physical examination revealed a reduced general health status, a 3/6 systolic murmur at the aortic valve area, a dry cough and pitting oedema of both ankles. Abnormal laboratory parameters included an elevated C-reactive protein (71.7 mg/L; normal <5 mg/L) and hypochromic normocytic anaemia (haemoglobin 7.1 mmol/L, normal 7.6–9.5 mmol/L; mean corpuscular haemoglobin 1.59 fmol, normal 1.74–2.05 fmol). Echocardiography displayed pericardial effusion that was punctured, detecting granulocytes, macrophages and mesothelial cells in cytological analysis. Radiographs of the large joints showed diffuse symmetric osteoplastic changes. 18F-fluorodeoxyglucose (FDG) positron emission tomography/CT (PET/CT) depicted infiltration of the thoraco-abdominal aorta, short occlusion of the left subclavian artery and stenosis of both renal arteries with slightly raised glucose metabolism in the corresponding vessels. Furthermore, symmetric raised bone metabolism of the long bones of the upper and lower extremities, numerous ribs, backbone, …

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  • Contributors The authors were involved in the clinical (PO, GW and TN) or pathological (IP and BS) and diagnostic management (EL and TW) of the patient as well as writing the manuscript (CW, PO, GW and TN), which was read and approved by all authors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.