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An Unusual Cause of Hypercalcemia
An Unusual Cause of Hypercalcemia
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A 40-year-old man with severe chronic tophaceous gout was found on routine labs to have significant hypercalcemia (13.7 mg/dL) and acute kidney injury (creatinine 4.2 mg/dL). On exam, he had extensive tophi and numerous subcutaneous nodules across the extremities, trunk, buttocks, and earlobes.<br /><br />Workup showed PTH-independent hypercalcemia: PTH was suppressed, and evaluation for malignancy and other common causes was negative. CT imaging ruled out solid tumors and granulomatous disease, and tests including SPEP, UPEP, PTHrP, and light chains were normal. Notable abnormalities included elevated ionized calcium and elevated 1,25-dihydroxy-vitamin D. X-rays of the hands and feet demonstrated extensive, partially calcified tophaceous gout with marked erosions and bone loss. Skin biopsy revealed calcified amorphous material surrounded by mononuclear histiocytes, giant cells, and fibrosis, consistent with a granulomatous reaction.<br /><br />The authors propose that large urate crystal deposits in tophi triggered macrophage-mediated granulomatous inflammation, leading to excess 1α-hydroxylase activity, increased 1,25-dihydroxy-vitamin D, and resulting hypercalcemia. This mechanism is similar to that seen in other granulomatous diseases such as sarcoidosis and tuberculosis, but is rare in gout.<br /><br />The patient improved with IV fluids and calcitonin, and prednisone was started to reduce peri-tophi inflammation. Outpatient pegloticase was planned. The case highlights that severe tophaceous gout, though uncommon, should be considered as a rare cause of PTH-independent hypercalcemia.
Asset Subtitle
Natalie A. Yass
Meta Tag
Author List
Garrett Patrick, Morgan Hanrahan, Natalie A. Yass
Category
Clinical Vignettes
Concept
PTH-independent hypercalcemia
Concept
Severe tophaceous gout
Concept
Hypercalcemia
Concept
Granulomatous Disease
Concept
1α-Hydroxylase
Concept
1a-Hydroxylase
Distinguished
Non-Finalist
Presenter Organization
Medical College of Wisconsin
Presenting Author
Natalie A. Yass
Track
Adult
Keywords
tophaceous gout
hypercalcemia
acute kidney injury
PTH-independent hypercalcemia
1,25-dihydroxy-vitamin D
granulomatous inflammation
urate crystal deposits
calcified tophi
calcitonin
prednisone
PTH-independent hypercalcemia
Severe tophaceous gout
Hypercalcemia
Granulomatous Disease
1α-Hydroxylase
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