Calcific uremic arteriolopathy (Calcyphilaxis): a rare disease? Report of
three cases
INTRODUCTION: Calcific uremic arteriolopathy (CUA; CALCYPHILAXIS) is a syndrome that occurs prevalently
in patients with chronic kidney disease on dialysis. It is characterized by the medial calcification
of skin small arteries leading to necrotic lesions. Several risk factors have been identified: obesity, female
gender, diabetes mellitus, hyperphosphatemia, inflammation, treatment with vitamin D, calcium-based
phosphate binders and warfarin.
MATERIALS AND METHODS: We report three cases of CUA observed from October 2011 to September 2014.
RESULTS: The mean age at diagnosis was 56 years (range 33-68). Biochemistry showed: mean levels of
PTH=1277 pg/ml (range 1000-1696), serum calcium =10.2 mg/dl (range 9.4-11.1), phosphorus=4.5 mg/dl
(range 3.4-5.5). All patients were taking vitamin D, two patients were on warfarin therapy.
Following actions were undertaken: interruption of calcium-based phosphate binders, vitamin D and
warfarin therapy, initiation of cinacalcet and sodium thiosulfate therapy, use of dialysate with lowest
available calcium concentration (1.25 mmol/l), Hyperbaric Oxygen Therapy, surgical dressings of skin lesions
three times a week.
Significant improvement was observed in mean levels of PTH (331 pg/ml, range 200-465), serum calcium
(8.3 mg/dl, range 7.4-9.6) and phosphorus (3.4 mg/dl, range 2.6-3.8). In two out of three patients complete
healing of ulcerative lesions was obtained.
CONCLUSIONS: These cases underline the importance of early diagnosis of CUA especially in patients with
concomitant risk factors and careful clinical monitoring, being CUA characterized by a rapid evolution
and high mortalit