Guidelines for the Treatment of Chronic Hypoparathyroidism in Adults


Year: 2016

Number: 3/4


Guidelines for the Treatment of Chronic Hypoparathyroidism in Adults

Hypoparathyroidism is an orphan disease of varying severity and uncertain natural history. There is a paucity of evidence from trials testing the efficacy of therapeutic strategies with defined clinical endpoints such as reduction in the progression of complications, enhanced quality of life, and improved survival. Management of the condition has relied on personal clinical experience and expert opinion. Successful management is viewed as short-term stability of biochemical parameters and symptom control. With the goal of improving clinical care, recent clinical guidelines were developed by two groups using different approaches. In 2014/15, the European Society of Endocrinology sponsored the development of a practical guideline for clinicians to provide recommendations for treatment and monitoring strategies. A systematic literature search was done by CBO (Dutch Institute for Health Care Improvement) along with a critical review of existing studies by an international working group of experts in endocrinology, nephrology, endocrine surgery, and epidemiology (Eur J Endo, vol 173, G1, 2015). Evidence was synthesized to address what constitutes the best treatment for adults with chronic hypoparathyroidism. Quality of evidence was evaluated according to GRADE (Grading of Recommendations Assessment, Development and Evaluation) principles and assigned as very low, low, moderate, and strong. All the standard treatment targets were graded as very low including levels of serum calcium, phosphate, magnesium, and calcium x phosphate product, urinary calcium, and vitamin D status. Standard treatment approaches (calcium, activated vitamin D analogues) were recommended with very low levels of evidence to support them. Routine replacement of PTH was not recommended. The frequency of clinical symptom monitoring and lab testing was advised to be regular intervals (every 3 to 6 months). Renal imaging was recommended for symptoms of stone disease or an increase in serum creatinine. Additional guidance was provided for care of patients with autosomal dominant hypocalcemia and women with hypoparathyroidism during pregnancy and breastfeeding. In 2015, an International Conference on the Management of Hypoparathyroidism met to develop evidence-based reports on presentation, epidemiology and management of hypoparathyroidism in adults followed by a summary statement and guidelines (J Clin Endo Metab, vol 101, 2273, 2016). GRADE was not used. Six goals for conventional management (calcium, vitamin D or analogues, magnesium, thiazide diuretics, phosphate binders, dietary interventions) were defined. Goals included absence of signs and symptoms of hypocalcemia, maintenance of serum calcium and calcium X phosphate product within target ranges, and avoidance of renal complications (nephrocalcinosis, nephrolithiasis). Several indications for considering the use of recombinant human PTH (1-84) were formulated including inadequate control of serum calcium, excessive doses of supplements, renal complications, elevated serum phosphate, malabsorption, and reduced quality of life. Future research should include studies designed to compare different treatment outcomes for the prevention of complications and enhancement of quality of life for patients with this rare and at times debilitating chronic disorder.