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04-03-2012 | Immunology | Article

Whole-body MRI may reveal Erdheim–Chester progression


Free abstract

MedWire News: Whole-body magnetic resonance imaging (MRI) could aid in the monitoring of patients with Erdheim-Chester disease, French clinicians suggest.

The extremely rare condition, a type of non-Langerhans' cell histiocytosis, presents as an inflammatory cell infiltration of the bone marrow that results in long-bone sclerosis, but is a systemic disease that can affect many different organs, explain Julien Haroche (Groupe Hospitalier Pitié-Salpêtrière, Paris) and co-workers.

A raft of imaging techniques, including MRI, computed tomography, positron emission tomography, and technetium 99-m bone scintigraphy are currently used to monitor Erdheim-Chester disease extent; prognosis is poorer in patients with cardiovascular or central nervous system manifestations.

Following the successful use of whole-body MRI to replacement multimodal imaging for patients with solid tumors, the team hypothesized that whole-body MRI could also be used to determine prognosis in patients with Erdheim-Chester disease.

To investigate, five patients whose Erdheim-Chester disease had previously been well characterized using multimodal imaging underwent diffusion-weighted whole-body MRI in the coronal view with background body signal suppression (DWIBS), and short tau inversion recovery sequences (STIRS).

As reported in Rheumatology, whole-body MRI DWIBS and STIRS revealed typical histiocytic infiltration in the long-bone bone marrow in the inferior limbs. Coronal T1-weighted imaging revealed both perirenal fat infiltration, and specific osteosclerosis of the left maxillary sinus walls.

Furthermore, whole-body MRI coronal T1-weighted imaging produced accurate imaging of cardiac disease extent, including infiltration of the right atrial wall.

However, whole-body MRI did not reveal pituitary infiltration known to have occurred in one patient. "It might be necessary to perform a more focused MRI evaluation of the hypothalamo-hypophyseal region during whole-body MRI when limited hypophyseal and pituitary stalk involvement is suspected," Haroche et al write.

The researchers admit that in, the absence of follow-up imaging studies in these patients, the value of whole-body MRI is yet unknown. Nevertheless, they conclude: "As whole-body MRI can perform a non-irradiating whole-body examination in a single session, these results suggest that WB-MRI could be a promising alternative to established multimodal imaging in [Erdheim-Chester disease]."

By Lynda Williams

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