British Journal of Radiology (2007) 80, 227-229
© 2007 British Institute of Radiology
doi: 10.1259/bjr/79596309
An unusual cause of knee pain
M Charest, MD
E A Haider, MB BCh
and
C Rush, MD, CM
Department of Nuclear Medicine, Division of Radiology, Jewish General Hospital, McGill University, Room G-19, 3755 Cote St. Catherine Road, Montreal, Quebec, H3T 1E2, Canada
Correspondence: Dr Mathieu Charest, Department of Nuclear Medicine, Jewish General Hospital, 7361 Saint-Denis, Montreal, Quebec H2R 2E5, Canada. E-mail: charestm{at}myway.com.
A 78-year-old female patient, with a past medical history notable only for stable hypertension, presented with chronic left knee pain. There were no other local or systemic symptoms. The pain was not relieved by analgesics or intra-articular cortisone injections. Laboratory tests revealed a normal complete blood count (CBC), normal electrolytes, serum alkaline phosphatase, calcium, phosphate and parathyroid hormone values. Plain knee radiographs performed at a clinic were interpreted as "findings consistent with Paget's disease". Since the patient's pain persisted, she was referred to our institution 6 months later for further evaluation. The radiographs were repeated (
Figures 1 and 2). Do you agree with the initial diagnosis of Paget's disease?
The conventional radiographs of the left knee (
Figures 1 and 2) demonstrate diffuse metadiaphyseal medullary sclerosis with sparing of the epiphyses. This pattern of uptake was not considered characteristic of Paget's disease and a bone scan was ordered. Three phase bone scintigraphy was performed (Figure 3) utilizing 99Tcm-MDP. The flow study of the knees revealed marked hyperaemia involving the metadiaphyseal regions of the distal femora and proximal tibiae bilaterally. Delayed phase whole body scintigraphic images demonstrate intense abnormal increased uptake in the metaphyses of the distal femora and proximal tibiae, extending into the adjacent diaphyses with sparing of the epiphyses. Less intense abnormal uptake is seen in the distal tibiae with sparing of the mid tibial diaphyses. Selected views of the hands and wrists reveal increased uptake in the distal ulnae and radii.
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Figure 3. (a) Anterior projection flow and blood pool study of the knees demonstrating abnormal hyperaemia. (b) Whole body bone scintigraphy with additional images obtained of the hands and lateral views of the knees. There is intense increased uptake in the femora, tibiae and distal forearm bones in a pattern characteristic of Erdheim-Chester disease. Mandibular, vertebral, sacroiliac and first carpometacarpal joint abnormalities were attributed to other pathology.
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These scintigraphic findings are characteristic for Erdheim-Chester disease (ECD). First described in 1930 by the pathologist William Chester as lipoid granulomatosis [1], ECD is now classified as a non-Langerhan's cell histiocytosis. As opposed to Langerhan's histiocytes, ECD cells stain negative for S-100 protein and CD1a. In addition, the absence of Birbeck granules on electron microscopy reinforces this distinction [2]. ECD is characterized by a disseminated xantho-granulomatous infiltration of unknown origin. Although the skeleton is most frequently affected, involvement of the lungs, central nervous system and retroperitoneum has been widely reported [2]. In a review of 59 cases [3], bone pain was the most common symptom (28 patients) and was sometimes isolated (7 patients), as was the case in this patient. The average age at presentation for ECD is 54 years [4]. Age at diagnosis ranges from 21 years to 76 years. The prognosis is thought to be related to the extent of extraosseous involvement [2] with the survival being reported to be typically between 5 months and 10 years after onset of symptoms [4].
On nuclear bone scintigraphy, ECD has pathognomonic features. There is intense symmetrical osteoblastic activity predominantly involving the appendicular skeleton and particularly the long tubular bones [5]. The epiphyses are typically spared in ECD, as opposed to Paget's disease in which the abnormal uptake usually extends up to the joint surface. On conventional radiographs, there is symmetric metadiaphyseal diffuse or patchy medullary sclerosis with minimal cortical involvement in the affected bones [6].
The differential diagnosis includes Paget's disease, lymphoma, osteomyelitis and sclerotic sarcoidosis, but the symmetrical involvement and the sparing of the epiphyses make these diagnoses less likely. Lipid storage disease, such as Gaucher's disease may demonstrate peri-articular and metaphyseal symmetric accumulation of radiotracer, but osteolytic lesions rather than sclerosis is predominant in Gaucher's disease [4].
In summary, ECD should be suspected when this characteristic pattern of skeletal uptake is observed on bone scintigraphy.
Received for publication November 8, 2005.
Accepted for publication November 28, 2005.
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References
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- Chester W. Uber lipoidgranulomatose. Virchows Arch Path Anat 1930;279:561–602.[CrossRef]
- Egan AJM, Boardman L, Tazelaar H, Swensen S, Jett J, Yousem S, et al. Erdheim-Chester disease: clinical, radiologic and histopathologic findings in five patients with interstitial lung disease. Am J Surg Pathol 1999;23:17–26.[CrossRef][Medline]
- Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M, et al. Erdheim-Chester disease: clinical and radiological characteristics of 59 cases. Medicine (Baltimore) 1996;75:157–69.[Medline]
- Kenn W, Stäbler A, Zachoval R, Zietz Ch, Raum W, Wittenberg G. Erdheim-Chester disease: a case report and litterature overview. Eur Radiol 1999;9:153–8.[CrossRef][Medline]
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- Resnick D, Greenway G, Genant H, Brower A, Haghighi P, Emmett M. Erdheim-Chester disease. Radiology 1982;142:289–95.[Free Full Text]
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