A case of Erdheim-Chester disease initially mistaken for Ormond's disease.
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.


METHODS
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.


METHODS
Erdheim-Chester disease with retroperitoneal fibrosis and bone sclerosis.


RESULTS
Treatment with glucocorticoids failed. The patient's symptoms improved significantly after initiation of interferon-alpha therapy.
Loddenkemper
Konstanze
K
Department of Rheumatology and Clinical Immunology, Charité University Medicine, Berlin, Germany. konstanze.loddenkemper@charite.de
Hoyer
Bimba
B
Loddenkemper
Christoph
C
Hermann
Kay-Geert
KG
Rogalla
Patrick
P
Förster
Gregor
G
Buttgereit
Frank
F
Hiepe
Falk
F
Burmester
Gerd-Rüdiger
GR
D000740:Anemia / Q000209:etiology; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D006869:Hydronephrosis / Q000209:etiology; D007370:Interferon Type I / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis*
D000970:Antineoplastic Agents; D007370:Interferon Type I; D011994:Recombinant Proteins