CREST Syndrome Questions & Answers

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

Overview

What is CREST syndrome?

What is the historical background of CREST syndrome?

What is the American College of Rheumatology Classification Criteria for Rheumatic Diseases and what are its limitation in categorizing CREST syndrome?

What are common features of diffuse CREST syndrome?

What are the classification criteria for CREST syndrome proposed by Nadashkevich et al?

What is the Maricq and Valter classification for CREST syndrome?

Which pathologic features are found in CREST syndrome?

What is the pathologic hallmark of CREST syndrome?

What is the pathogenesis of skin fibroblasts in CREST syndrome?

What is the role of mononuclear infiltration in the pathogenesis of CREST syndrome?

What is the role of granulocyte macrophage colony-stimulating factor (GM-CSF) in the pathogenesis of CREST syndrome?

What is the role of interleukin 4 in the pathogenesis of CREST syndrome?

What is the role of connective tissue growth factor (CTGF) in the pathogenesis of CREST syndrome?

What is the role of serum tissue inhibitor of metalloproteinase-1 (TIMP-1) in the pathophysiology of CREST syndrome?

What is the role of vascular abnormalities in the pathophysiology of CREST syndrome?

What is the role of genetics in the pathogenesis of CREST syndrome?

What is the role of antiendothelial cell antibodies in the pathogenesis of CREST syndrome?

What is the primary pathogenic trigger of CREST syndrome?

What is the role of microchimerism in the pathogenesis of CREST syndrome?

What causes CREST syndrome?

What causes calcinosis in CREST syndrome?

What causes Raynaud phenomenon in CREST syndrome?

What causes esophageal dysmotility in CREST syndrome?

What causes sclerodactyly in CREST syndrome?

What is the incidence of CREST syndrome in the US?

What is the global incidence of CREST syndrome?

What are the racial predilections of CREST syndrome?

How does the incidence of CREST syndrome vary by sex?

How does the prevalence of CREST syndrome vary by age?

What is the prognosis of CREST syndrome?

Presentation

What are the signs and symptoms of Reynaud phenomenon in CREST syndrome?

What are the signs and symptoms of calcinosis in CREST syndrome?

Which clinical history is characteristic of Raynaud phenomenon in CREST syndrome?

What is the esophageal involvement in CREST syndrome?

What are the esophageal symptoms of CREST syndrome?

Which complications of esophageal dysmotility may be present in CREST syndrome?

What are the signs and symptoms of sclerodactyly in CREST syndrome?

What are the signs and symptoms of telangiectases in CREST syndrome?

Other than the cardinal signs, what are the symptoms of CREST syndrome?

Which calcinosis findings suggest CREST syndrome?

What are the physical findings of Raynaud phenomenon in CREST syndrome?

What are the physical findings of esophageal dysmotility in CREST syndrome?

What are the physical findings of sclerodactyly in CREST syndrome?

What are the physical findings of telangiectasia in CREST syndrome?

What are complications of CREST syndrome?

DDX

Which disorders should be included in the differential diagnoses of CREST syndrome?

Workup

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

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

Which tests may be performed to assess esophageal dysmotility in CREST syndrome?

Treatment

What are the treatment options for CREST syndrome?

How is calcinosis managed in CREST syndrome?

What is the role of oral corticosteroids in the treatment of CREST syndrome?

Which medications are used in the treatment of calcinosis in CREST syndrome?

How is an elevated core body temperature maintained in patients with Raynaud phenomenon who have CREST syndrome?

What is the efficacy of behavior therapy for the treatment of CREST syndrome?

What is the role of calcium channel blockers in the treatment of CREST syndrome?

What is the role of topical agents in the treatment of CREST syndrome?

What is the role of prostaglandins in the treatment of CREST syndrome?

What is the role of antiplatelet therapy in the treatment of CREST syndrome?

What is the role of low molecular heparin in the treatment of CREST syndrome?

What is the role of plasma exchange in the treatment of CREST syndrome?

What is the role of losartan in the treatment of CREST syndrome?

Which medications have been investigated for the treatment of CREST syndrome?

What is the role of alpha-adrenergic antagonists in the treatment of CREST syndrome?

What is the role of fluoxetine in the treatment of CREST syndrome?

What is included in the treatment of Raynaud phenomenon in CREST syndrome?

How is esophageal dysmotility managed in CREST syndrome?

How is sclerodactyly managed in CREST syndrome?

How is telangiectasia managed in CREST syndrome?

What is the role of surgical excision in the treatment of CREST syndrome?

What is the role of cervical sympathectomy in the treatment of CREST syndrome?

What are the surgical treatments for esophageal dysmotility in CREST syndrome?

What is the surgical treatment for sclerodactyly in CREST syndrome?

What is the role of bowel resection in the treatment of CREST syndrome?

Which specialist consultations may be beneficial in the treatment of CREST syndrome?

Which dietary restrictions are beneficial in the treatment of CREST syndrome?

Which activity modifications are beneficial in the treatment of CREST syndrome?

How is Raynaud phenomenon prevented in CREST syndrome?

How is esophageal dysmotility prevented in CREST syndrome?

Medications

What are the goals of drug treatment for CREST syndrome?

Which medications in the drug class Antihypertensive agents are used in the treatment of CREST Syndrome?

Which medications in the drug class Proton-pump inhibitors are used in the treatment of CREST Syndrome?

Which medications in the drug class Histamine H2 antagonists are used in the treatment of CREST Syndrome?

Which medications in the drug class Phosphodiesterase enzyme inhibitors are used in the treatment of CREST Syndrome?

Which medications in the drug class Antidepressants are used in the treatment of CREST Syndrome?

Which medications in the drug class Prostaglandins are used in the treatment of CREST Syndrome?

Which medications in the drug class Calcium channel blockers are used in the treatment of CREST Syndrome?