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Scleroderma and Related Disorders

Posted Dec 21 2009 10:08pm
By Leyla H. Alparslan MD

Musculoskeletal System

• Radiographs can demonstrate the characteristic features of systemic sclerosis including calcinosis, loss of normal distal soft tissue and skin creases, and osteolysis that particularly involves the distal tufts.
• Computed tomography (CT) is useful in demonstrating paraspinal calcifications.
• Magnetic resonance imaging (MRI) is valuable for assessing paraspinal calcifications and their effect on neurologic structures, as well as for identifying areas of inflammatory myopathy.

Pulmonary Involvement
• Radiographs may be normal in early interstitial lung disease.
• High-resolution computed tomography (HRCT) is more sensitive than radiography in the assessment of early interstitial lung disease and is able to differentiate active inflammation from fibrosis.
• Bronchoalveolar lavage (BAL) abnormalities may precede HRCT findings.

Peripheral Vascular System
• Arteriography: Vasospasm and pruning of the small digital vessels are characteristic findings.
Pulmonary Hypertension and Cardiac Involvement
• On posteroanterior erect chest radiographs, the transverse diameter of the right interlobar artery should ≤16 mm. Enlargement occurs in pulmonary artery hypertension.
• Right heart catheterization optimally evaluates pulmonary arterial hypertension.
• Myocardial disease can be evaluated using radionuclide imaging, coronary angiography, or cardiac MRI.

Renal Involvement
• Ultrasound and biopsy are used for exclusion of other causes of renal disease.
• Nuclear scans can demonstrate decreased renal blood flow and glomerular filtration rate.

Gastrointestinal System
• Radiographs and CT may show obstruction, pseudoobstruction, volvulus, intestinal perforation, and pneumatosis cystoides intestinalis.
• Barium swallow: Esophageal dilatation, diminished or absent peristalsis in the distal two thirds of the esophagus, and a patulous gastroesophageal junction are typical features. Stricture occurs secondary to chronic gastroesophageal reflux.
• Barium follow through: Delay in transit time with decreased motility, pseudodiverticula, and hidebound appearance.
• Barium enemas are usually avoided because they may result in impaction.
• Scintigraphy: Delayed esophageal transit time and gastric emptying and gastroesophageal reflux are typical features. Delayed scans are useful for detection of pulmonary aspiration.

Available online 9 November 2009.
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