An Unusual Case of Hypercalcemia in a Patient with Early Onset Dermatomyositis with Dystrophic Calcifications

Presentation Number: MON 314
Date of Presentation: April 3rd, 2017

Elizabeth Chacko*1, Ranjodh Singh Gill2, Huzaefah Syed3 and Francesco S. Celi1
1Virginia Commonwealth University, Richmond, VA, 2VA Commenwealth University, Richmond, VA, 3Virginia Commonwealth University

Abstract

Background:

Dystrophic calcifications are common sequelae of Dermatomyositis occurring in approximately one third of patients affected by Juvenile Dermatomyositis (onset age < 16 year). Hypercalcemia is a rare complication, and most of the documented cases are in the pediatric population. Here, we describe a case of hypercalcemia in a 25 year old patient with recent diagnosis of onset of dermatomyositis and multiple foci of subcutaneous calcinosis.

Case:

A 25 year old Hispanic female initially presented in July of 2015 with three month history of truncal rash and diffuse muscle weakness refractory to treatment with steroids. EMG and muscle biopsy confirmed the diagnosis of dermatomyositis. After failure to improve with methotrexate and mycophenolate mofetil, a treatment with IV Ig and hydroxychloroquine was initiated. The patient had a prolonged course with multiple hospitalizations due to complications including calcinosis cutis with ulceration and cellulitis, dysphagia requiring Percutaneous Endoscopic Gastrostomy (PEG) placement, respiratory failure requiring tracheostomy placement and multiple episodes of bacteremia. She was treated with IV Ig throughout her hospitalization. In June 2016, the conditions improved and she was transferred to rehabilitation for one month. She responded well to treatment with significant improvement in muscle weakness, skin rash and mobility. Her tracheostomy was decannulated and PEG was removed. Her serum calcium and phosphorus remained within normal limits until September 2016 when she was noted to have an abrupt rise in her serum calcium level up to 14 mg/dL with phosphorus 4.6 mg/dL, albumin of 3.3 g/dL, intact PTH 6.3 pg/ml (8.7-77.1), PTHrp < 1.1 pmol/l (<2.0), 25 OH vitamin D 15.3 ng/ml(>30), calcitriol of 14.6 pg/ml (19.9-79.3), total urinary calcium of 494 mg/24 hr and N-terminal telopeptide (NTX)/Creat 109 nM/mM Cr (<64). The patient received aggressive hydration and parenteral bisphosphonate therapy will be initiated following 25 OH vitamin D repletion.

Conclusion:
The hypercalcemia in this case is a non PTH mediated process, and the active bone resorption as indicated by the bone specific alkaline phosphatase and NTX elevations cannot be attributed to immobilization. We speculate that the hypercalcemia is the result of an immunological process associated with IV Ig therapy.


 

Nothing to Disclose: EC, RSG, HS, FSC