Abstract: FR-PO640
Non-Uremic Calciphylaxis: Early Diagnosis of a Rare Entity and Successful Treatment with Sodium Thiosulfate
Session Information
- Trainee Case Reports - IV
October 26, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 402 Bone and Mineral Metabolism: Clinical
Authors
- Eleid, Rocio, University of North Dakota, Fargo, North Dakota, United States
- Phadke, Gautam M., University of North Dakota School of Medicine, Fargo, North Dakota, United States
Introduction
Calciphylaxis is a rare cutaneous disease, with varied lesions: from bullae to necrotic ulcers. Pain out of proportion to clinical examination is a distinct finding. Key finding is Vitamin K deficiency, contributing to progressive microvascular calicifcation and microthrombi. Diabetes, liver cirrhosis and patients on warfarin are Vitamin K deficient.
Case Description
A 64 yo male with DM2, HTN and cirrhosis was referred to the Nephrology Clinic for evaluation of AKI, hematuria, nephrotic range proteinuria. Kidney biopsy showed Nodular Diabetic Nephropathy, secondary IgA nephropathy (liver cirrhosis) and ATN. His Ca was 9.6 mg/dl, Phos 4.1 mg/dl, vitamin D 15ng/dl, and PTH 129 pg/ml. Three months after initial evaluation, measured GFR (Iohexol) was 19 ml/min/1.73m2 and he presented with left lower extremity, painful, necrotic ulcers. Despite surgical debridement, lesions increased in size and he developed new lesions on the right leg. Skin biopsy via telescoping technique showed intraluminal occlusion of small and medium sized vessels with calcified appearing material consistent with calciphylaxis. Sodium thiosulfate was started at 12.5 g twice/week, it was increased to 25 g twice/week and that dose was kept for 7months. Patient lesions improved and thiosulfate was discontinued.
Discussion
This illustrates the importance of an early diagnosis and treatment of non uremic calciphylaxis with attention to "at risk" patients: liver cirrhosis, in our case. The patient is currently been referred for simultaneous liver-kidney transplantation.
Top Left: Lesion at presentation
Top Right: Lesion resolved
Bottom: H&E, shows calcification and fibrointimal hyperplasia