Definition: serum [Ca2+] >10.1mg/dL, ionized >1.36 mmol/L.
Etiology/Risk Factors
- Almost always due to a combination of increased bone resorption/increased GI absorption and decreased renal excretion.
- 90% due to hyperparathyroidism or malignancy. If mild, more likely primary hyperparathyroidism; if moderate to severe, more likely malignancy-related.
- 10% due mostly to granulomatous disease, as well as some rare causes.
- Differential diagnosis:
- Calcium excess.
- Hyperparathyroidism (primary and tertiary).
- Immobilization/Inherited (familial hypocalciuric hypercalcemia).
- Meds (thiazide, lithium, vitamin D, vitamin A).
- Paget’s disease.
- Adrenal insufficiency/Addison’s disease.
- Neoplasm (metastases, PTHrP producing, calcitriol producing, or osteolytic/blastic activity. Commonly, breast, lung, myeloma, lymphoma).
- Zollinger-Ellison syndrome.
- Endocrine (hyperthyroidism causing excess bone resorption).
- Sarcoid and other granulomatous diseases (TB, histoplasmosis, berylliosis, Hodgkin’s).
Evaluation
1. Check PTH level:
- If high, this is suggestive of primary hyperparathyroidism. If minimally elevated or normal (inappropriate for calcium level), check 24-hour urine calcium excretion.
- HIGH: >250 mg (men) or >200 mg (women), suggestive of primary hyperparathyroidism.
- LOW: milk alkali syndrome (rarely), familial hypocalciuric hypercalcemia.
- Low PTH (appropriately suppressed):
- Look for offending medications (vitamin D, calcium supplements).
- Consider granulomatous disease, endocrinopathies (thyroid or adrenal insufficiency), or malignancy; see step 2.
2. If PTH level is low, consider the following additional workup:
- Labs:
- Serum:
- Albumin, alkaline phosphatase.
- 25-OH and 1,25-OH vitamin D (1,25-OH is elevated in granulomatous disease).
- PTHrP.
- SPEP with immunofixation.
- Consider AM cortisol, TSH, insulin-like growth factor.
- Urine:
- Urine calcium and creatinine (ideally, 24 hour collection).
- UPEP with immunofixation.
- Serum:
- Imaging:
- CXR (for sarcoid, TB, fungal infections).
- Bone scan (for malignancy).
- Bone survey (for multiple myeloma).
- CT scan (for primary tumor).
- If workup is negative, consider immobility, though this is a diagnosis of exclusion.
Management
- Evaluate for symptoms: “stones, bones, abdominal groans, with psychiatric overtones.”
- Renal: polyuria, nephrolithiasis, chronic kidney disease (nephrocalcinosis), AKI (calcium-induced vasoconstriction and hypovolemia).
- GI: anorexia, nausea, vomiting, constipation; can rarely lead to pancreatitis.
- Neuro: weakness, fatigue, confusion, stupor, coma.
- Musculoskeletal: bone pain.
- Cardiac: shortened QT, hypertension.
- Treatment:
- Hypercalcemia causes loop diuretic-like effect and partial nephrogenic DI which can lead to volume depletion.
- Volume resuscitation with normal saline: at least 3-4 L in first 24 hours.
- In contrast to classic teaching, recent studies show no clear benefit of loop diuretics in addition to fluids. Can consider IV diuresis after volume repletion in patients where volume overload is a concern.
- Bisphosphonates block osteoclastic bone resorption and have been shown to decrease skeletal morbidity but not mortality in metastatic malignancy.
- Pamidronate: 90 mg IV over 4 hours (for Ca >13.5).
- Zoledronic acid: 4 mg IV over 15 min-30 min (adjusting for GFR), along with crystalloid. Better studied in malignancy.
- Both agents work over 72 hrs. Side effects include ↓ magnesium/phosphorus and low-grade fever. Be sure to check electrolytes frequently and replete Mg and Phos as needed after administration.
- Calcitonin (salmon): more rapid onset but weak/transient effect (1-3 mg/dL) that wanes after 2-3 days due to tachyphylaxis. Be wary of cramps and flushing.
- 4-8 SQ/IM q 12 hours x 1 to 3 days.
- Glucocorticoids decrease GI absorption and prevent 1,25-OH vitamin D formation in granulomatous disease; may be anti-neoplastic (myeloma, lymphoma).
- Hypercalcemia causes loop diuretic-like effect and partial nephrogenic DI which can lead to volume depletion.
Key Points
- 90% of hypercalcemia is due to hyperparathyroidism or malignancy.
- Evaluation is based on determining whether hypercalcemia is PTH-mediated.
- Primary initial treatment of hypercalcemia is aggressive fluid resuscitation.
- Presence of hypercalcemia is a very poor prognostic indicator in non-parathyroid malignancy (hypercalcemia of malignancy—30 day mortality >50% with median survival 4-6 weeks).
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LeGrand SB, Leskuski D, Zama I. Narrative Review: Furosemide for Hypercalcemia: An Unproven yet Common Practice.Susan B. LeGrand, Ann Intern Med August 19, 2008 149:259-263
Major P, Lortholary A, Hon J, et al. Zoledronic acid is superior to pamidronate in the treatment of hypercalcemia of malignancy: a pooled analysis of two randomized, controlled clinical trials. J Clin Oncol 2001;19:558-567