Hypercalcemia with Elevated 1-25 (OH) 2 Vitamin D: Extra Pulmonary Sarcoidosis or Not?

Presentation Number: MON 322
Date of Presentation: April 3rd, 2017

Radhika Annam*1 and Smita N Kargutkar2
1Monmouth Medical center, Long Branch, NJ, 2Barnabas Health Medical Group, Long Branch, NJ

Abstract

Introduction:

Hypercalcemia is a relatively common clinical problem which is mostly seen secondary to primary hyperparathyroidism or malignancy accounting for greater than 90% of cases. But rarely is also associated with granulomatous disorders most commonly sarcoidosis and tuberculosis. Hypercalcemia in sarcoidosis and other granulomatous disorders is due to parathyroid hormone independent extra renal excess conversion of 25 (OH) vitamin D to 1-25(OH) 2 vitamin D by macrophage 1-α-hydroxylase within granulomas. We present a patient with hypercalcemia, hypercalciuria with elevated 1-25 (OH) 2 vitamin D levels and low PTH without any clinical evidence of sarcoidosis.

Clinical case:

A 37- year old Caucasian female with the past medical history of gonadotropin secreting pituitary micro adenoma treated with leuprolide in the past, presented with recurrent kidney stones treated with lithotripsy. Work-up revealed hypercalcemia 11 mg/dl (8.6-10.4 mg/dl), hypercalciuria (774.8 mg/24hr), low PTH <6.3 pg/ml (13.8-85 pg/ml), elevated 1-25 (OH) 2 vitamin D 117.0 pg/ml (15-60 pg/ml) low 25 (OH) vitamin D 20.7 ng/ml (32.0-100.0 ng/ml) normal PTH-r peptide (n < 0.74 pmol/L) and elevated serum ACE level 119 U/l (9-67 U/l).

Serum globulin levels were elevated 4.2 g/dl (1.7-3.7 g/dl), serum electrophoresis was slightly abnormal with elevated IgG 2912 mg/dl (700-1600 mg/dl), elevated IgM 330 mg/dl (40-230 mg/dl) and normal IgA 136 mg/dl (91-414 mg/dl). She was evaluated by Hem-oncologist for possible monoclonal gammopathy of undetermined significance but as per their opinion it was unlikely the cause of hypercalcemia. Her biochemical testing was consistent with sarcoidosis although clinically she did not have lymphadenopathy or any other stigmata to suggest possible sarcoidosis. CT scan of neck chest and abdomen did not show any evidence of lymphadenopathy to show pulmonary sarcoidosis or any other granulomatous disorders. Abdominal and pelvis CT scan was significant only for bilateral non obstructing calculi. She underwent Kveim-test which was normal. Recently on follow up she was advised to get PET- CT scan to look for any evidence of possible extra pulmonary sarcoidosis. Her other medications included oral contraceptive pill Desogestrel.

Conclusion:

It is extremely rare having hypercalcemia with elevated 1-25 (OH) 2 vitamin D levels and low PTH without any evidence of sarcoidosis and this case could possibly be extrapulmonary sarcoidosis. It is imperative that physicians have a high index of suspicion to identify the underlying etiology of hypercalcemia in order to treat the patient appropriately.

 

Nothing to Disclose: RA, SNK