What is the role of imaging studies in the workup of CREST syndrome?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Imaging studies are as follows:

  • Calcinosis: The diagnosis of calcinosis (suspected based on physical examination finding of palpable, hard, subcutaneous nodules) is confirmed with plain radiographs demonstrating dermal or subcutaneous radiodense deposits. Computed tomography and bone scanning (skeletal scintigraphy with diphosphate compounds) are more sensitive for identifying calcinosis when plain radiography findings are normal. [59]  Videodermatoscopy may also be a useful tool for diagnosing dystrophic calcifications such as those resulting from CREST syndrome; however, it is not yet widely used. [60]

  • Raynaud phenomenon: Digital ischemia and/or infarction secondary to Raynaud disease may prompt Doppler ultrasonography or angiography to identify anatomic occlusive disease, which may be amenable to angioplasty or bypass surgery.

  • Esophageal dysmotility: Multiple modalities are available to evaluate esophageal dysmotility. The least invasive evaluation involves radiologic barium studies with attention to the esophagus (cine-esophagram). Findings include dilatation with decreased or absent peristalsis in the distal esophagus, indicating advanced disease. Esophageal stricture and ulcerations indicate late findings of erosive esophagitis. The best use of a barium swallow is to exclude a stricture when the patient reports dysphagia. Esophageal transit time can be demonstrated by fluoroscopy, but this is not clinically useful. Radionuclide scanning is specific for gastroesophageal reflux and pulmonary aspiration, but it is expensive and is not sensitive.

  • Sclerodactyly: No imaging studies are necessary.

  • Telangiectasia: No imaging studies are necessary for cutaneous telangiectasia.

  • Cough or fatigue: If patients have cough or fatigue, perform transthoracic echocardiography to look for pulmonary hypertension. More advanced pulmonary hypertension may demonstrate signs of right-sided heart enlargement on chest radiographs.


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