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CREST Syndrome

Sections CREST Syndrome
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Medication

Medication Summary

The goal of pharmacotherapy is to reduce morbidity and to prevent complications.

Class Summary

These agents are used as part of therapy for Raynaud phenomenon.

Nicardipine (Cardene)

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Nicardipine is used for its vasodilatation and possible antiplatelet effects. Start with the lowest dose available. Extended-dose preparations and agents with fewer negative inotropic effects are preferred.

Nifedipine (Procardia)

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Nifedipine relaxes coronary smooth muscle and produces coronary vasodilation, which, in turn, improves myocardial oxygen delivery; adverse reactions occur predominantly with short-acting formulations and include peripheral edema, headache, dizziness, and tachycardia; calcium channel blockers may worsen gastroesophageal reflux; SR formulations are associated with fewer adverse effects.

Class Summary

These are included as part of therapy for Raynaud phenomenon.

Alprostadil (Prostaglandin E1)

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Alprostadil is a strong vasodilator of all vascular beds.

Class Summary

This class is for the treatment of Raynaud phenomenon.

Fluoxetine (Prozac)

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Fluoxetine selectively inhibits presynaptic serotonin reuptake with minimal or no effect in the reuptake of norepinephrine or dopamine. It may cause more adverse GI effects than other SSRIs now currently available, which is the reason it is not recommended as a first choice.

Fluoxetine may be given as a liquid and a capsule. It may be given as 1 dose or in divided doses. The presence of food does not appreciably alter levels. Because of its long half-life (72 h), it may take up to 4-6 weeks to achieve steady state levels.

The long half-life is an advantage and a drawback. If it works well, an occasional missed dose is not a problem; if problems occur, eliminating all active metabolites takes a long time. The choice depends on adverse effects and drug interactions. Adverse effects of SSRIs seem to be quite idiosyncratic; thus, relatively few reasons exist to prefer one to another at this point if dosing is started at a conservative level and advanced as tolerated.

Class Summary

This class is for the treatment of Raynaud phenomenon.

Cilostazol (Pletal)

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Cilostazol affects vascular beds and cardiovascular function. It may improve blood flow by altering the rheology of red blood cells. It produces nonhomogenous dilation of vascular beds, with more dilation in femoral beds than in vertebral, carotid, or superior mesenteric arteries.

Cilostazol and its metabolites are inhibitors of phosphodiesterase III and, as a result, cyclic AMP is increased, which leads to inhibition of platelet aggregation and vasodilation.

Class Summary

This class is for the symptomatic relief of reflux resulting from esophageal dysmotility.

Famotidine (Pepcid)

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Famotidine competitively inhibits histamine at the H2 receptor of gastric parietal cells, resulting in reduced gastric acid secretion, gastric volume, and reduced hydrogen concentrations.

Nizatidine (Axid)

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Nizatidine competitively inhibits histamine at the H2 receptor of the gastric parietal cells, resulting in reduced gastric acid secretion, gastric volume, and reduced hydrogen concentrations.

Ranitidine (Zantac)

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Ranitidine inhibits histamine stimulation of the H2 receptor in gastric parietal cells, which, in turn, reduces gastric acid secretion, gastric volume, and hydrogen concentrations.

Class Summary

This class is for the treatment of reflux symptoms resulting from esophageal dysmotility.

Omeprazole (Prilosec)

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Omeprazole decreases gastric acid secretion by inhibiting the parietal cell H⁺/K⁺ ATP pump.

Lansoprazole (Prevacid)

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Lansoprazole inhibits gastric acid secretion.

Class Summary

This class is for Raynaud phenomenon.

Losartan (Cozaar)

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Losartan is a nonpeptide angiotensin II receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. It may induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors. It does not affect the response to bradykinin and is less likely to be associated with cough and angioedema. It is used for patients unable to tolerate ACE inhibitors. Losartan is less effective in patients with scleroderma than in those with primary Raynaud phenomenon. It may modify some serum markers of vascular damage and possibly modulate some of the underlying tissue damage in scleroderma.

Nitroglycerin topical (Nitro-Bid, Nitro-Dur, Nitrogard)

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Nitroglycerin relaxes smooth muscle all over the body, including those of the LES and esophageal body.

Questions

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Media Gallery

Calcinosis on dorsal forearm.
Close-up view of calcinosis.
Raynaud phenomenon showing pallor of most of the finger tips with a violaceous discoloration (hyperemia) of the thumb tip.
Sclerodactyly (also with Raynaud phenomenon).
Sclerodactyly.
Telangiectasia of the face.
Telangiectasia of the lip.
Telangiectasia of the finger.
Close-up view of telangiectasia.

of 9

Author

Jeanie C Yoon, MD Clinical Instructor, Department of General Internal Medicine, University of Washington School of Medicine

Jeanie C Yoon, MD is a member of the following medical societies: American College of Physicians, Society of Hospital Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference wish to thank Dr. Bruce Gilliland for assistance in reviewing the manuscript and Dr. Jan V. Hirschmann and Dr. Netayna Sandler for their images.

The authors and editors of Medscape Reference also gratefully acknowledge the contributions of previous authors, Mary A. Wemple, MD, and Kyle L. Horner, MD, MS, to the development and writing of this article.

Sections CREST Syndrome
Overview
Presentation
DDx
Workup
Treatment
- Medical Care
- Surgical Care
- Consultations
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Medication
Questions & Answers
Media Gallery
References

CREST Syndrome Medication

Medication Summary

Calcium channel blockers

Class Summary

Nicardipine (Cardene)

Nicardipine (Cardene)

Nifedipine (Procardia)

Nifedipine (Procardia)

Prostaglandins

Class Summary

Alprostadil (Prostaglandin E1)

Alprostadil (Prostaglandin E1)

Antidepressants

Class Summary

Fluoxetine (Prozac)

Fluoxetine (Prozac)

Phosphodiesterase enzyme inhibitors

Class Summary

Cilostazol (Pletal)

Cilostazol (Pletal)

Histamine H2 antagonists

Class Summary

Famotidine (Pepcid)

Famotidine (Pepcid)

Nizatidine (Axid)

Nizatidine (Axid)

Ranitidine (Zantac)

Ranitidine (Zantac)

Proton-pump inhibitors

Class Summary

Omeprazole (Prilosec)

Omeprazole (Prilosec)

Lansoprazole (Prevacid)

Lansoprazole (Prevacid)

Antihypertensive agents

Class Summary

Losartan (Cozaar)

Losartan (Cozaar)

Nitroglycerin topical (Nitro-Bid, Nitro-Dur, Nitrogard)

Nitroglycerin topical (Nitro-Bid, Nitro-Dur, Nitrogard)

CREST Syndrome Medication

Medication Summary

Calcium channel blockers

Class Summary

Nicardipine (Cardene)

Nifedipine (Procardia)

Prostaglandins

Class Summary

Alprostadil (Prostaglandin E1)

Antidepressants

Class Summary

Fluoxetine (Prozac)

Phosphodiesterase enzyme inhibitors

Class Summary

Cilostazol (Pletal)

Histamine H2 antagonists

Class Summary

Famotidine (Pepcid)

Nizatidine (Axid)

Ranitidine (Zantac)

Proton-pump inhibitors

Class Summary

Omeprazole (Prilosec)

Lansoprazole (Prevacid)

Antihypertensive agents

Class Summary

Losartan (Cozaar)

Nitroglycerin topical (Nitro-Bid, Nitro-Dur, Nitrogard)

References

Tables

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