Type I Polyglandular Autoimmune Syndrome Medication

Updated: Nov 11, 2021
  • Author: Saleh A Aldasouqi, MD, FACE, ECNU; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Medication

Medication Summary

The drugs listed here are used primarily for the replacement of deficient hormones and electrolytes (except for ketoconazole). The medications detailed in this list are the major, well-established drugs available for each category. However, newer agents, especially in the antifungal category, have been introduced; these may be administered by qualified physicians, especially to critically ill patients in the ICU.

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Corticosteroids

Class Summary

These are used for adrenocortical insufficiency replacement. Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Hydrocortisone (Cortef, A-Hydrocort, Solu-Cortef)

DOC because of mineralocorticoid activity and glucocorticoid effects. Useful for treatment of many diseases, especially autoimmune and inflammatory diseases. Used in PGA-I for primary adrenal failure.

Fludrocortisone

Partial replacement therapy for primary and secondary adrenocortical insufficiency. Most commonly prescribed synthetic mineralocorticoid. Possesses glucocorticoid qualities. Encourages sodium reabsorption at distal renal tubules, GI mucosa, and the sweat and salivary glands.

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Antifungals

Class Summary

These drugs treat mucocutaneous candidiasis. Their mechanism of action may involve an alteration of ribonucleic acid (RNA) and deoxyribonucleic acid (DNA) metabolism or an intracellular accumulation of peroxide that is toxic to the fungal cell.

Ketoconazole

First azole used in clinical practice. Imidazole broad-spectrum antifungal agent that inhibits synthesis of ergosterol, causing cellular components to leak, resulting in fungal cell death. Also acts on several P450 enzymes including the first step in cortisol synthesis, cholesterol side-chain cleavage, and conversion of 11-deoxycortisol to cortisol. May inhibit ACTH secretion when used at therapeutic doses. Possess narrow therapeutic index.

Fluconazole (Diflucan)

Fungistatic activity. Synthetic oral antifungal (broad-spectrum bis-triazole) that selectively inhibits fungal cytochrome P-450 and sterol C-14 alpha-demethylation, which prevents conversion of lanosterol to ergosterol, thereby disrupting cellular membranes.

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Vitamins and mineral salts

Class Summary

These are used as nutritional supplements.

Calcitriol (Vectical, Rocaltrol)

Active metabolite of vitamin D that binds to and activates the vitamin D receptor in kidney, parathyroid gland, intestine, and bone. The active form of vitamin D stimulates intestinal calcium transport and absorption. This medication is essential in individuals with hypoparathyroidism.

Ergocalciferol (Calciferol, Drisdol, Calcidol)

Stimulates absorption of calcium and phosphate from small intestine and promotes release of calcium from bone into blood. Precursor of active form of vitamin D (calcitriol). Because it is a precursor, a significant delay between dose administration and effect exists. Liver must be intact for intermediate to be formed (calcidiol, 25-hydroxy vitamin D). Many drugs may affect this step. Has lipid storage, so overdoses may cause prolonged hypercalcemia.

Measure of efficacy is serum calcium concentration.

Calcium carbonate (Oystercal, Caltrate)

Calcium moderates nerve and muscle performance by regulating action potential excitation threshold. For hypoparathyroidism, use a supplementation of at least 2 g of elemental calcium/d.

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