How should I approach the management and follow-up of a patient diagnosed with MEN type 1?

For a patient diagnosed with Multiple Endocrine Neoplasia Type 1 (MEN1), management and follow-up in primary care should be guided by specialist advice, as MEN1 is a complex condition affecting multiple endocrine glands ¹.

• Primary Hyperparathyroidism (PHPT) Management and Follow-up:
  • Arrange monitoring of serum calcium, vitamin D, estimated glomerular filtration rate (eGFR), and creatinine every 12 months ¹.
  • Monitor bone mineral density by dual-energy X-ray absorptiometry (DXA) every 2 years, depending on specialist advice ¹.
  • Arrange an X-ray or vertebral fracture assessment of the spine if clinically indicated, for example, if there is height loss and/or back pain ¹.
  • Arrange renal imaging, such as ultrasound, X-ray, or CT, if renal stones are suspected ¹.
  • Arrange a cardiovascular risk assessment ¹,².
  • Re-refer to an endocrinology specialist if symptoms of hypercalcaemia develop, the adjusted serum calcium concentration increases to 0.25 mmol/L or more above the normal range (or 2.85 mmol/L or above), the eGFR is less than 60 mL/min/1.73 m², there are renal stones or increased risk of renal stones, or if osteoporosis confirmed on DXA or a fragility fracture occurs ¹.
  • If the person has had successful parathyroid surgery, check calcium levels annually ¹,².
  • If surgery has been unsuccessful, a multidisciplinary team (MDT) review at a specialist centre is required, and monitoring should continue ².
  • Provide advice on sources of information and support, including information about the condition, treatments, and ongoing care and monitoring ¹,².
• Pancreatic Involvement (e.g., Pancreatic Neuroendocrine Tumours, Chronic Pancreatitis) Management and Follow-up:
  • Offer monitoring by clinical and biochemical assessment for pancreatic exocrine insufficiency and malnutrition at least every 12 months, to be agreed with the specialist centre ³. Adjust treatment of vitamin and mineral deficiencies accordingly ³.
  • Offer adults with chronic pancreatitis a bone density assessment every 2 years ³.
  • Be aware that people with chronic pancreatitis have an increased risk of developing pancreatic cancer ³.
  • For people with Type 3c diabetes, assess every 6 months for potential benefit of insulin therapy ³.
• Pituitary Involvement (e.g., Cushing's Syndrome) Management and Follow-up:
  • Arrange long-term follow-up in primary care to screen for and treat any complications arising from Cushing’s syndrome, such as diabetes mellitus, hypertension, osteoporosis, and increased cardiovascular risk ⁴.
  • Assessment, monitoring, and treatment should be individualised based on complications present and coordinated between secondary and primary care ⁴.
  • Consider discussing and offering age-appropriate vaccinations such as influenza, herpes zoster, and pneumococcus, as people with Cushing’s syndrome are at increased risk of infection ⁴.
  • Life-long monitoring is needed post-operatively for ACTH-secreting pituitary tumours as recurrence is common ⁴.
• Pregnancy:
  • Discuss the management of primary hyperparathyroidism for pregnant women with an MDT in a specialist centre, and refer for specialist care if needed ².