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 NICE CKS.

• Primary Hyperparathyroidism (PHPT) Management and Follow-up:
  • Arrange monitoring of serum calcium, vitamin D, estimated glomerular filtration rate (eGFR), and creatinine every 12 months NICE CKS.
  • Monitor bone mineral density by dual-energy X-ray absorptiometry (DXA) every 2 years, depending on specialist advice NICE CKS.
  • 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 NICE CKS.
  • Arrange renal imaging, such as ultrasound, X-ray, or CT, if renal stones are suspected NICE CKS.
  • Arrange a cardiovascular risk assessment NICE CKS,NICE NG132.
  • 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 NICE CKS.
  • If the person has had successful parathyroid surgery, check calcium levels annually NICE CKS,NICE NG132.
  • If surgery has been unsuccessful, a multidisciplinary team (MDT) review at a specialist centre is required, and monitoring should continue NICE NG132.
  • Provide advice on sources of information and support, including information about the condition, treatments, and ongoing care and monitoring NICE CKS,NICE NG132.
• 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 NICE NG104. Adjust treatment of vitamin and mineral deficiencies accordingly NICE NG104.
  • Offer adults with chronic pancreatitis a bone density assessment every 2 years NICE NG104.
  • Be aware that people with chronic pancreatitis have an increased risk of developing pancreatic cancer NICE NG104.
  • For people with Type 3c diabetes, assess every 6 months for potential benefit of insulin therapy NICE NG104.
• 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 NICE CKS.
  • Assessment, monitoring, and treatment should be individualised based on complications present and coordinated between secondary and primary care NICE CKS.
  • 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 NICE CKS.
  • Life-long monitoring is needed post-operatively for ACTH-secreting pituitary tumours as recurrence is common NICE CKS.
• Pregnancy:
  • Discuss the management of primary hyperparathyroidism for pregnant women with an MDT in a specialist centre, and refer for specialist care if needed NICE NG132.