Myositis and a Mass in Scleroderma

August 13, 2013

Radiographs, CT and MRI images from two patients with scleroderma demonstrate muscle and lung involvement and other signs of connective tissue disorder.

A 22-year-old woman presented with lower extremity pain and weakness. The radiological images show clear evidence to support a diagnosis of scleroderma.


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Coronal view;  


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   Axial view

 

MRI images of the lower extremities (above) demonstrate high signal on the inversion recovery sequence, indicating an edema pattern within the gluteus medius (left more than right) and then in both sets of adductor musculature. 

CT of the lung bases (below, left) demonstrates interstitial lung disease with reticulonodular opacities at the periphery and mild honeycombing.  CT of the upper mediastinum (below, right) demonstrates a patulous esophagus with fluid and air seen within the distended lumen.


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Images from a different case with the same diagnosis, a 62-year-old woman with a palpable mass of the left elbow, are shown below.


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Sagittal proton density MRI through the left elbow (right) demonstrates low signal masses in the posterior soft tissue that, on radiographs below, correspond with large, confluent, tumorlike calcification in the posterior soft tissue.


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COMMENTS:

Connective tissue disorder, more prevalent in women than in men, produces symptomatology generally in the second to third decade and continues to progress. The underlying pathology is a fibrosis that affects multiple organ systems. 

Classic findings include myositis of the proximal musculature that later precipitates fatty atrophy, and large conglomerate tumor-like or sheet-like calcification of the soft tissue.

It is frequently associated with other syndromes and produces an interstitial lung disease, preferentially at the lung bases, and esophageal dysmotility or a patulous esophagus

RESOURCES:

Resnick, D. Kranskdorf, M. Bone and Joint Imaging. 3rd Edition. 2005

Scleroderma, Medicine Net.  Scleroderma, Medscape Reference

Bassett LW, Blocka KL, Furst DE, et al. Skeletal findings in progressive systemic sclerosis (scleroderma).  AJR Am J Roentgenol. (1981) 136(6):1121-1126