Scleroderma

November 19, 2013



Case History: A 52-year-old woman with low back pain.

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Image 1: Inversion Recovery Coronal (A) and Axial (B, C). Please note B is slightly cranial to C in position.




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Image 2: CT through the lung bases using a lung window



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Image 3: CT through the upper mediastinum using a soft tissue window


Findings: MRI of the lower extremities demonstrates high signal on the IR 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 demonstrates interstitial lung disease with reticulonodular opacities at the periphery and mild honeycombing. CT of the upper mediastinum demonstrates a patulous esophagus with fluid and air seen within the distended lumen.

Second case, same diagnosis: A 62-year-old woman with palpable mass of left elbow.

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Image 4: Sagittal Proton Density MRI through the left elbow



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Image 5: AP and lateral radiographs of the left elbow


Findings: Sagittal MRI demonstrates low signal masses in the posterior soft tissue that on radiographs corresponds with large, confluent, tumorlike calcification in the posterior soft tissue

Diagnosis: Scleroderma

Comments: Connective tissue disorder more prevalent in women than men that produces symptomatology generally in the second to third decade and continues to progress. 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 tumorlike 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
http://www.medicinenet.com/scleroderma/article.htmhttp://www.emedicine.com/med/topic2076.htm
• Bassett LW, Blocka KL, Furst DE, Clements PJ, Gold RH. Skeletal findings in progressive systemic sclerosis (scleroderma). AJR Am J Roentgenol. 1981 Jun;136(6):1121-6

Case courtesy Hospital for Special Surgery. ©2013 Hospital for Special Surgery.
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