From the Guidelines
Hypoparathyroidism with an immune etiology requires calcium and vitamin D supplementation as the cornerstone of treatment, with a goal to maintain serum calcium levels in the low-normal range (8.0-8.5 mg/dL) while avoiding hypercalciuria, as recommended in the practical guidelines for managing adults with 22q11.2 deletion syndrome 1. The treatment of hypoparathyroidism with an immune etiology involves calcium and vitamin D supplementation. Patients should take elemental calcium (1-2 grams daily divided into multiple doses) along with active vitamin D (calcitriol 0.25-2 mcg daily).
- The goal of treatment is to maintain serum calcium levels in the low-normal range (8.0-8.5 mg/dL) while avoiding hypercalciuria.
- Regular monitoring of calcium levels, both in serum and urine, is essential to prevent complications such as kidney stones and renal failure.
- Autoimmune hypoparathyroidism occurs when the immune system attacks and destroys the parathyroid glands, leading to insufficient parathyroid hormone (PTH) production.
- This condition may occur in isolation or as part of autoimmune polyglandular syndrome type 1.
- Unlike surgical hypoparathyroidism, the immune-mediated form is permanent and requires lifelong treatment.
- Patients should be educated about symptoms of hypocalcemia (tingling, muscle cramps, seizures) and hypercalcemia (nausea, confusion, kidney stones).
- In severe cases of hypocalcemia, intravenous calcium gluconate may be necessary.
- Some patients may benefit from recombinant PTH therapy (teriparatide or PTH 1-84) if conventional therapy is inadequate, although the use of vitamin D analogs such as calcitriol has shown promise in treating related conditions like alopecia areata 1.
From the Research
Hypoparathyroidism Immune
- Hypoparathyroidism is a rare endocrine disease characterized by a deficiency of parathyroid hormone, and its standard therapy is based on oral calcium and active vitamin D supplementation 2.
- The disease can be caused by various factors, including autoimmune diseases, genetic abnormalities, and destruction or infiltrative disorders of the parathyroids 3.
- Treatment of symptomatic or profound asymptomatic hypocalcemia is aimed at rapid intravenous administration of calcium and oral supplementation of vitamin D metabolites, while long-term management involves oral calcium and vitamin D analogs 3.
- Parathyroid hormone replacement, such as teriparatide, has been shown to be effective in improving serum calcium and lowering serum phosphate, as well as reducing the doses of calcium and calcitriol supplementation required 2, 4, 5.
- Continuous subcutaneous infusion of teriparatide has also been reported to be effective in restoring long-term calcium homeostasis in patients with hypoparathyroidism 5.
Treatment Options
- Conventional therapy of hypoparathyroidism consists of oral calcium and either activated vitamin D or vitamin D supplements at varying doses 6.
- Teriparatide, a pharmaceutical form of parathyroid hormone, can be administered through subcutaneous injections or continuous delivery systems 2, 4, 5.
- Thiazide diuretics can also be used to enhance renal calcium reabsorption and increase serum calcium, particularly in patients with activating mutations of the calcium-sensing receptor 3.
Autoimmune Hypoparathyroidism
- Autoimmune hypoparathyroidism is a rare cause of hypoparathyroidism, and its treatment is similar to that of other forms of the disease 2.
- One case study reported the use of teriparatide in a patient with autoimmune hypoparathyroidism, which resulted in a diminishment of symptoms and the ability to maintain normocalcemia without parenteral calcium 2.