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[Calcific uremic arteriolopathy (Calcyphilaxis): a rare disease? Report of three cases]
Insights
Calcific uremic arteriolopathy (CUA) is a serious skin condition in dialysis patients. Early diagnosis and a multi-faceted treatment approach, including medication changes and hyperbaric oxygen therapy, can lead to lesion healing.
Area of Science:
- Nephrology
- Dermatology
- Vascular Medicine
Background:
- Calcific uremic arteriolopathy (CUA), also known as CALCYPHILAXIS, is a severe condition affecting patients with chronic kidney disease on dialysis.
- Characterized by medial calcification of small arteries in the skin, CUA leads to painful necrotic lesions.
- Identified risk factors include obesity, diabetes, hyperphosphatemia, and certain medications like vitamin D, calcium-based phosphate binders, and warfarin.
Observation:
- This report details three cases of CUA diagnosed between October 2011 and September 2014.
- Patients presented with elevated parathyroid hormone (PTH), serum calcium, and phosphorus levels.
- All patients were on vitamin D, and two were on warfarin therapy.
Findings:
- A comprehensive treatment strategy was implemented, involving the cessation of calcium-based phosphate binders, vitamin D, and warfarin.
- Therapies included cinacalcet, sodium thiosulfate, low-calcium dialysate, and hyperbaric oxygen therapy.
- Significant improvements were noted in PTH, calcium, and phosphorus levels, with two of three patients achieving complete healing of skin lesions.
Implications:
- These cases highlight the critical need for prompt diagnosis of CUA in at-risk dialysis patients.
- Close clinical monitoring is essential due to CUA's rapid progression and high mortality rate.
- Aggressive management involving medication adjustment and advanced therapies can improve outcomes in CUA patients.
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 mortality.
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