Familial Renal Amyloidosis Medication

Updated: Nov 21, 2018
  • Author: Helen J Lachmann, MD, MRCP; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Medication

Medication Summary

The aims of current medical therapy are to support compromised organ function and to ameliorate symptoms.

Patients are at increased risk of hemorrhage because of increased vascular fragility and/or substantial GI amyloid deposits. Unless overwhelming indications for anticoagulation therapy are present, it is best avoided.

No existing treatment specifically results in mobilization and regression of amyloid deposits, but novel drug compounds that inhibit the formation, persistence, and/or effects of amyloid deposits are presently in development.

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Antihypertensive agents

Class Summary

Hypertension is common and can accelerate the decline in renal function. Maintain blood pressure within the lower end of normal range.

Ramipril (Altace)

Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion.

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Diuretics

Class Summary

Often help treat symptomatic peripheral edema resulting from nephrotic syndrome.

Furosemide (Lasix)

Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. Dose must be individualized to patient. Depending on response, administer at increments of 20-40 mg, no sooner than 6-8 h after previous dose, until desired diuresis occurs.

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Proton pump inhibitors

Class Summary

Acute GI bleeding or perforation is the cause of death in a large proportion of patients with lysozyme amyloidosis, and long-term prophylactic treatment with a proton pump inhibitor is advisable.

Omeprazole (Prilosec)

Decreases gastric acid secretion by inhibiting the parietal cell H+/K+ -ATP pump.

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Histamine2-receptor antagonists

Class Summary

Reversible competitive blockers of histamine at the H2 receptors, particularly those in the gastric parietal cells, where they inhibit acid secretion. The H2 antagonists are highly selective, do not affect the H1 receptors, and are not anticholinergic agents.

Ranitidine (Zantac)

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

Cimetidine (Tagamet)

Inhibits histamine at H2 receptors of gastric parietal cells, which results in reduced gastric acid secretion, gastric volume, and hydrogen concentrations.

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Prokinetic agents

Class Summary

Gastric emptying may be delayed, and some patients respond quite well to prokinetic agents or antiemetics.

Metoclopramide (Reglan)

A dopamine antagonist that stimulates gastric emptying and small intestinal transit.

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