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A case of Erdheim-Chester disease initially mistaken for Ormond's disease
Konstanze Loddenkemper1, Bimba Hoyer, Christoph Loddenkemper
1Department of Rheumatology and Clinical Immunology, Charité University Medicine, Berlin, Germany. konstanze.loddenkemper@charite.de
Background:
A 54-year-old man presented with fever, abdominal pain, anemia, elevated C-reactive protein level and decreased renal function. Idiopathic retroperitoneal fibrosis (Ormond's disease) had been diagnosed in the past, leading to surgical ureterolysis. Re-evaluation of the CT scans and histological biopsy samples revealed unusual sclerosis of the long bones and diffuse infiltrates of foamy histiocytes in the bone marrow and the retroperitoneum.
Investigations:
Physical examination, laboratory tests including hemoglobin concentration, erythrocyte sedimentation rate, C-reactive protein level, beta2-microglobulin level, creatinine level, CT of the chest and abdomen, bone scintigraphy, bone marrow and soft tissue biopsies and immunohistochemistry.
Diagnosis:
Erdheim-Chester disease with retroperitoneal fibrosis and bone sclerosis.
Management:
Treatment with glucocorticoids failed. The patient's symptoms improved significantly after initiation of interferon-alpha therapy.
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