Primary hyperparathyroidism: a summary of recommendations from NICE

Guideline name: Hyperparathyroidism (primary): diagnosis, assessment and initial management [NG132]

Update type: New guideline

Published: May 2019

Takeaway

• NICE has published a new guideline which focuses on the diagnosis, assessment and management of primary hyperparathyroidism, with the aim of reducing long-term complications and improving quality of life.
• Measurement of albumin-adjusted serum calcium and parathyroid hormone (PTH) are crucial in the diagnosis of the condition in primary care.

Diagnosis in primary care

Albumin-adjusted serum calcium

• Measure albumin-adjusted serum calcium if any of the following criteria are present
  • Hypercalcaemia symptoms (thirst, frequent or excessive urination, or constipation).
  • Osteoporosis or a previous fragility fracture.
  • Renal calculi.
  • Incidental finding of elevated albumin-adjusted serum calcium (≥2.6 mmol/L or above).
• Measure albumin-adjusted serum calcium for individuals with chronic non-differentiated symptoms.
• Measurement of ionised calcium is not recommended.
• If the initial albumin-adjusted serum calcium level is ≥2.6 mmol/L or ≥2.5 mmol/L along with features of primary hyperparathyroidism, at least 1 more repeat measurement is necessary. Consider further measurements on the basis of symptoms and albumin-adjusted serum calcium levels.

PTH

• Measure PTH using a random sample and concurrently measure albumin-adjusted serum calcium.
• Repeated PTH measurement is not routinely recommended in primary care.
• Seek specialist advice if PTH measurement is either:
  • Above the midpoint of reference range with suspicion of primary hyperparathyroidism or
  • Below the midpoint of reference range with albumin-adjusted serum calcium level ≥2.6 mmol/L.
• Further investigations are not recommended if:
  • PTH is within reference range but below the midpoint and
  • Albumin-adjusted serum calcium level is <2.6 mmol/L.
• If PTH is below the lower limit of the reference range, seek alternative diagnoses including malignancy.

Assessment in secondary care

• If there is a probable diagnosis of primary hyperparathyroidism, measure vitamin D and offer vitamin D supplements if required.
• Urine calcium excretion should be measured to rule out familial hypocalciuric hypercalcaemia
• If a diagnosis of primary hyperparathyroidism is confirmed, perform the following:
  • Assessment of symptoms and comorbidities.
  • Measurement of estimated GFR or serum creatinine.
  • Dual-energy X-ray absorptiometry scan of the lumbar spine, distal radius and hip.
  • Ultrasound scan of the renal tract.

Referral for surgery

• Individuals with a confirmed diagnosis of primary hyperparathyroidism can be referred to a surgeon specialising in parathyroid surgery if the following criteria are present:
  • Symptoms of hypercalcaemia (thirst, frequent or excessive urination, or constipation) or
  • End-organ disease (renal stones, fragility fractures or osteoporosis) or
  • Albumin-adjusted serum calcium level ≥2.85 mmol/L.
• Surgical options include 4-gland exploration and focused parathyroidectomy.

Post-surgical follow-up

• Measure albumin-adjusted serum calcium and PTH prior to discharge post-surgery and albumin-adjusted serum calcium 3 to 6 months after surgery to assess the success of the surgery.

Non-surgical management

• If surgery is unsuccessful, unsuitable or has been declined by patient, consider treatment with cinacalcet, provided albumin-adjusted serum calcium level is
  • ≥2.85 mmol/L with symptoms of hypercalcaemia or
  • ≥3.0 mmol/L without symptoms of hypercalcaemia.
• The decision to continue cinacalcet should be based on the extent of reduction of symptoms and albumin-adjusted serum calcium level.
• Bisphosphonates can lower the risk of fractures in at-risk patients with primary hyperparathyroidism.
• Bisphosphonates are not recommended for chronic hypercalcaemia of primary hyperparathyroidism.

Monitoring

• If parathyroid surgery was successful:
  • Measure albumin-adjusted serum calcium once a year.
  • Seek specialist opinion if osteoporosis is present.
  • Seek specialist opinion if renal stones are present.
• If parathyroid surgery was not performed or it was unsuccessful:
  • Measure albumin-adjusted serum calcium and eGFR or serum creatinine once a year. If treated with cinacalcet, follow monitoring guidelines prescribed in the summary of product characteristics.
  • Conduct a DXA scan at diagnosis and every 2 to 3 years.
  • Offer ultrasound of the renal tract at diagnosis, if renal stone is suspected.

Pregnancy

• Parathyroid surgery is suitable for women with primary hyperparathyroidism who are considering pregnancy.
• Consult a multidisciplinary team (MDT) in a specialist centre for management of primary hyperparathyroidism during pregnancy. The MDT should comprise an obstetrician, a physician specialising in primary hyperparathyroidism, a surgeon, a midwife, and an anaesthetist.
• Cinacalcet and bisphosphonates are not recommended for pregnant women with primary hyperparathyroidism.
• Women with primary hyperparathyroidism have an increased risk for hypertensive disease in pregnancy. Follow NICE guideline on hypertension in pregnancy.

Information and support

• Provide patients with primary hyperparathyroidism all the necessary information regarding the condition and treatments options
• Provide advice to patients with primary hyperparathyroidism regarding symptom reduction, and preparation for surgery or other treatments.
• Discuss ongoing care and monitoring with patients.