CREST Syndrome Medication

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

Medication Summary

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

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Calcium channel blockers

Class Summary

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

Nicardipine (Cardene)

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)

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.

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Prostaglandins

Class Summary

These are included as part of therapy for Raynaud phenomenon.

Alprostadil (Prostaglandin E1)

Alprostadil is a strong vasodilator of all vascular beds.

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Antidepressants

Class Summary

This class is for the treatment of Raynaud phenomenon.

Fluoxetine (Prozac)

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.

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Phosphodiesterase enzyme inhibitors

Class Summary

This class is for the treatment of Raynaud phenomenon.

Cilostazol (Pletal)

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.

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Histamine H2 antagonists

Class Summary

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

Famotidine (Pepcid)

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)

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)

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

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Proton-pump inhibitors

Class Summary

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

Omeprazole (Prilosec)

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

Lansoprazole (Prevacid)

Lansoprazole inhibits gastric acid secretion.

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Antihypertensive agents

Class Summary

This class is for Raynaud phenomenon.

Losartan (Cozaar)

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)

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

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