Initially, given the elevated inflammatory markers, new skin lesions and low complements (i.e., C4), the patient was treated with prednisone 60 mg orally once daily for a possible lupus flare. Despite high steroid doses, the patient’s skin lesions continued to spread and worsen. A biopsy result of her skin lesions was obtained, showing diffuse calcium deposits throughout the dermis and subcutaneous layers (including the blood vessels), which was consistent with calciphylaxis.
An extensive workup was performed to determine the etiology of the patient’s calciphylaxis. It involved the evaluation for anti-phospholipid syndrome (APLS), cryoglobulinemia, anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis, endocarditis, septic emboli, malignancy and hyperparathyroidism. Unfortunately, this workup proved inconclusive. Parathyroid hormone (PTH), parathyroid hormone-related protein (PTHrP), cryoglobulin levels, myeloperoxidase antibodies (MPO), proteinase 3 antibodies (PR3), beta-2-glycoprotein, cardiolipin antibodies, lupus anticoagulant, creatinine kinase and serum protein electrophoresis (SPEP) were all negative or within normal limits.
A computed tomography (CT) scan of the chest, abdomen and pelvis revealed diffuse subcutaneous calcifications and diffuse bilateral groundglass opacities in the lungs (see Figure 2). There were no masses or nodules suspicious for malignancy. A nuclear bone scan demonstrated abnormal radiotracer uptake in bilateral lungs, soft tissues in the upper and lower extremities, and her stomach, which was consistent with calciphylaxis (see Figure 3).
Initially, the patient was started on sodium thiosulfate 25 g IV three times per week. Prednisone was tapered down quickly to 20 mg once a day to aid with wound healing and minimize infection risk. Both mycophenolate mofetil and belimumab were withheld during patient’s initial hospitalization to minimize further infection risk. Despite these efforts, the wounds rapidly worsened over the following week and a half.
Given the concern the patient’s underlying connective tissue disease (CTD) was driving the calciphylaxis, intravenous immunoglobulin therapy (IVIG) was given at a dose of 2 mg/kg split into three consecutive days. However, the patient’s skin lesions continued to spread. Sodium thiosulfate was discontinued after approximately two weeks of therapy, and the patient was given a trial of pamidronate 30 mg IV. Unfortunately, the patient became hypocalcemic and could not receive further pamidronate doses. The patient also received plasmapheresis every other day for a total of five sessions over a two-week period, during which the lesions appeared to stabilize, but then started getting worse again.
The patient was transferred to a nearby hospital for a trial of hyperbaric oxygen therapy, but was deemed not a candidate due to severe mitral regurgitation in the setting of a ruptured chordae tendonae as a result of calciphylaxis affecting the heart structures.
