Endocrinology · PANCE / PANRE

Primary Hyperparathyroidism

Autonomous PTH oversecretion (most often a single adenoma) producing hypercalcemia.

Also known as: primary hyperparathyroidism, parathyroid adenoma, PHPT, hyperparathyroidism

Overview

Inappropriately elevated or non-suppressed PTH in the setting of hypercalcemia. Most commonly due to a solitary parathyroid adenoma (~85%); less often hyperplasia of all four glands (~10-15%), double adenoma (~4%), or parathyroid carcinoma (<1%).

Epidemiology

Most common cause of outpatient hypercalcemia. Prevalence ~1 in 1000; up to 1 in 500 women >50. Female-to-male ratio ~3:1. Peak incidence 50-60 years. Most cases now detected on routine chemistry panels (mild, asymptomatic).

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Question 1EndocrineMedium
A 35-year-old woman is evaluated after passing a kidney stone and is found on bone densitometry to have osteoporosis. Laboratory studies show serum calcium 11.8 mg/dL, phosphorus 2.1 mg/dL, intact parathyroid hormone 145 pg/mL (elevated), and a 24-hour urine calcium of 320 mg. Which of the following is the most likely diagnosis?
  • AFamilial hypocalciuric hypercalcemia
  • BMalignancy-associated hypercalcemia
  • CSecondary hyperparathyroidism
  • DPrimary hyperparathyroidism
Reveal answer & full explanation
Correct answer: D — Primary hyperparathyroidism
  • AFamilial hypocalciuric hypercalcemia
  • BMalignancy-associated hypercalcemia
  • CSecondary hyperparathyroidism
  • DPrimary hyperparathyroidism

Why Primary hyperparathyroidism is correct

  • Hypercalcemia (11.8 mg/dL) with an inappropriately elevated PTH (145 pg/mL) defines PTH-dependent hypercalcemia, and a parathyroid adenoma is the cause in roughly 85% of cases.
  • Excess PTH drives renal phosphate wasting (hypophosphatemia 2.1 mg/dL), increased bone resorption (osteoporosis), and hypercalciuria (urine calcium 320 mg/24h), producing this patient's stone and bone disease.

Why the others are wrong

  • Familial hypocalciuric hypercalcemia — also shows hypercalcemia with non-suppressed PTH but is defined by LOW urine calcium (calcium-to-creatinine clearance ratio typically <0.01); the elevated 24-hour urine calcium refutes it, a confused-with trap on 'high calcium, normalish PTH.'
  • Malignancy-associated hypercalcemia — PTHrP-mediated hypercalcemia suppresses PTH, so an elevated PTH excludes it; it is the premature-closure pick for any hypercalcemia.
  • Secondary hyperparathyroidism — a compensatory PTH rise to chronic hypocalcemia (renal failure, vitamin D deficiency) that presents with low or normal calcium, not hypercalcemia; buzzword-matching on 'high PTH.'
Question 2EndocrineMedium
A 66-year-old woman presents with fatigue, constipation, and depression for 6 months. BP 142/86. Labs: Na 138, K 3.2, Ca 11.8 mg/dL, albumin 4.0, parathyroid hormone (PTH) 98 pg/mL (normal 10–65), creatinine 1.0. 24-hour urine calcium is elevated. DEXA shows T-score -2.3 at the lumbar spine. She is asymptomatic regarding renal symptoms. Which of the following is the most appropriate management?
  • ABisphosphonate therapy
  • BCinacalcet therapy
  • CParathyroidectomy
  • DAnnual monitoring with labs
Reveal answer & full explanation
Correct answer: C — Parathyroidectomy
  • ABisphosphonate therapy
  • BCinacalcet therapy
  • CParathyroidectomy
  • DAnnual monitoring with labs

Why Parathyroidectomy is correct

  • This is primary hyperparathyroidism: elevated calcium (11.8 mg/dL) with an inappropriately elevated parathyroid hormone (PTH) of 98 pg/mL (normal 10–65)
  • Surgical indications include: age <50, serum calcium >1 mg/dL above upper limit of normal, T-score ≤-2.5 (osteoporosis), 24-hour urine calcium >400 mg, glomerular filtration rate (GFR) <60, or nephrolithiasis
  • This patient has calcium 11.8 mg/dL (>1 above upper limit of normal of ~10.5) and elevated 24-hour urine calcium; the T-score of -2.3 does not quite meet the ≤-2.5 threshold, but the calcium criterion alone is sufficient — multiple criteria are met
  • Parathyroidectomy is indicated and is curative

Why the others are wrong

  • Annual monitoring with labs — observation is appropriate only when no surgical criteria are met; this patient meets multiple criteria
  • Bisphosphonate therapy — does not correct the underlying disease
  • Cinacalcet therapy — reserved for patients who are not surgical candidates
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Risk factors

  • Female sex, postmenopausal age
  • Prior neck radiation
  • Lithium therapy (alters set point)
  • Familial syndromes: MEN1 (parathyroid + pituitary + pancreas), MEN2A (parathyroid + medullary thyroid + pheo), familial isolated hyperparathyroidism, HPT-jaw tumor syndrome (CDC73 mutation)
  • Chronic vitamin D deficiency (can mask presentation)

Pathophysiology

Loss of calcium-sensing regulation in one or more parathyroid glands → autonomous PTH secretion despite normal or elevated serum calcium. PTH increases bone resorption (osteoclast activation), enhances renal calcium reabsorption and phosphate excretion, and stimulates 1-alpha-hydroxylation of vitamin D (boosting intestinal calcium absorption). Net effect: hypercalcemia, hypophosphatemia, and skeletal/renal pathology.

Clinical presentation

Symptoms

  • 'Stones, bones, abdominal groans, psychic moans, fatigue overtones'
  • Most are asymptomatic — discovered on routine calcium
  • Renal: nephrolithiasis (calcium oxalate or phosphate), polyuria, polydipsia, nephrocalcinosis
  • Skeletal: bone pain, pathologic fracture, osteoporosis
  • GI: anorexia, nausea, constipation, peptic ulcer, pancreatitis
  • Neuropsychiatric: depression, fatigue, memory loss, confusion
  • Cardiovascular: hypertension, short QT, LVH

Signs / physical exam

  • Often a normal exam
  • Band keratopathy (rare), brown tumors (rare advanced disease)
  • Hypertension, sometimes bradyarrhythmia
  • DEXA: cortical bone loss (distal radius > spine), fractures

Classic findings

Postmenopausal woman with kidney stones, mild fatigue, osteoporosis, and incidentally elevated calcium on routine labs.

Differential diagnosis

  • Secondary hyperparathyroidism — Compensatory PTH elevation from hypocalcemia or vitamin D deficiency; calcium normal/low, phosphate often elevated (CKD)
  • Tertiary hyperparathyroidism — Autonomous PTH after prolonged secondary HPT (long-standing CKD); calcium elevated; usually requires parathyroidectomy
  • Familial hypocalciuric hypercalcemia (FHH) — AD inactivating CaSR mutation; mild lifelong hypercalcemia with LOW urinary calcium (Ca/Cr clearance ratio <0.01); DO NOT operate
  • Malignancy-associated hypercalcemia — PTH suppressed; PTHrP elevated (squamous cell, renal, breast) or osteolytic mets, or 1,25-OH vitamin D from lymphoma
  • Vitamin D toxicity — Elevated 25-OH vitamin D, suppressed PTH
  • Granulomatous disease (sarcoidosis, TB) — Elevated 1,25-OH vitamin D from macrophage activity, suppressed PTH
  • Milk-alkali syndrome — Large calcium carbonate ingestion; suppressed PTH, alkalosis, AKI
  • Hyperthyroidism, thiazides, lithium — Drug or hormonal causes; review medication list

Diagnostic workup

Diagnostic criteria

Hypercalcemia + inappropriately elevated or non-suppressed PTH + low urinary calcium excretion rules OUT FHH (urinary calcium high or normal in PHPT, low in FHH).

Labs

  • Serum calcium (corrected for albumin) and ionized calcium — confirm hypercalcemia
  • PTH (intact) — elevated or inappropriately normal
  • Phosphate (low), magnesium, alkaline phosphatase (elevated in bone disease)
  • 25-OH vitamin D (replace if deficient before final assessment), 1,25-OH vitamin D
  • 24-hour urine calcium and creatinine — calculate calcium/creatinine clearance ratio to exclude FHH (<0.01 = FHH)
  • BUN, creatinine, eGFR
  • DEXA scan (lumbar spine, hip, AND distal third of radius)
  • Consider genetic testing for MEN1/MEN2 if young, family history, or multigland disease

Imaging

  • Renal ultrasound or CT (assess for stones, nephrocalcinosis)
  • Pre-operative localization: Sestamibi (Tc-99m) scan ± SPECT/CT, neck ultrasound, 4D CT — identifies adenoma for minimally invasive parathyroidectomy
  • Not all guidelines require localization — surgeon-dependent

Diagnostic algorithm

DiagnosisCaPTHPhosphateUrine CaKey feature
Primary HPTHighHigh / inappropriateLowHigh / normalMost often single adenoma
FHHMildly highHigh / inappropriateNormalLOW (Ca/Cr <0.01)AD, lifelong; do not operate
Secondary HPT (CKD)Low / normalHighHighVariableCompensatory; treat underlying
Tertiary HPTHighHighVariableVariableAfter long-standing 2°; parathyroidectomy
Malignancy (PTHrP)HighLOW (suppressed)LowHighSquamous, renal, breast cancer
Vitamin D toxicityHighLowHighHighElevated 25-OH vitamin D
Differentiating causes of hypercalcemia by calcium, PTH, phosphate, and urinary calcium.

Complications

  • Osteoporosis, fragility fracture, osteitis fibrosa cystica (advanced)
  • Nephrolithiasis, nephrocalcinosis, CKD
  • Hypercalcemic crisis, pancreatitis, peptic ulcer
  • Surgical: recurrent laryngeal nerve injury, hypoparathyroidism (especially after subtotal/total parathyroidectomy)
  • Hungry bone syndrome post-op: profound hypocalcemia and hypophosphatemia
  • Persistent or recurrent hyperparathyroidism (missed gland, ectopic location)

PANCE pearls

  • Always check a 24-h urinary calcium to exclude FHH before parathyroidectomy — operating on FHH does NOT cure hypercalcemia.
  • Lithium and thiazide diuretics elevate calcium and PTH — stop if possible and re-test.
  • Always measure DEXA at distal third of radius — primary HPT preferentially affects cortical bone.
  • Intra-operative PTH should drop >50% within 10 min and to normal range — confirms removal of the offending gland.
  • Post-parathyroidectomy 'hungry bone syndrome': skeleton avidly takes up Ca and PO4, producing severe hypocalcemia — pre-treat with vitamin D and calcium.

References

  • Fourth International Workshop 2014 — Guidelines for the Management of Asymptomatic Primary Hyperparathyroidism (Bilezikian et al., J Clin Endocrinol Metab 2014)
  • AAES 2016 — American Association of Endocrine Surgeons Guidelines for Definitive Management of Primary Hyperparathyroidism (Wilhelm et al., JAMA Surg 2016)
  • Endocrine Society 2011 — Evaluation and Treatment of Hypercalcemia of Malignancy (Stewart, NEJM 2005) and ES guideline on vitamin D

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