V KHELGI1, 2, S GOVINDARAJULU1, K VENUTHURAPALLI1, 2, A GUPTA1, 2
1Renal service, Darling Downs Hospital and Health Service, Toowoomba, Queensland; 2Rural School of Medicine, University of Queensland Toowoomba, Queensland
Background: Calcyphylaxis or Calcific uremic arteriolopathy (CUA) is a rare condition characterised by cutaneous vascular calcification leading to painful non-healing ulceration and necrosis. CUA is common in patients with End Stage Renal Disease (ESRD) and associated with high mortality and morbidity. There is no standard treatment available at present.
Methods: We retrospectively analysed data of eight Calcyphylaxis patients treated in DDHHS area between June 2002 and June 2016. All the relevant data was collected including demographics, dialysis mode, pathology results at the time of diagnosis (Calcium, phosphate, ALP and PTH levels), and the different modalities of treatment used in management.
Results: 8 patients were diagnosed with CUA, all of whom with ESRD. 7 received maintenance Haemodialysis (HD) and 1 peritoneal dialysis (PD). Five patients (62.5%) developed CUA since November 2015 coinciding with delisting of cinacalcet from Pharmaceutical Benefit Scheme (PBS). 5 patients were on calcitriol and only 1 was on warfarin. Patient on PD received Intraperitoneal STS while on HD received intravenous at the end of dialysis session. There was equal 1:1 sex distribution. Abdomen and thigh are the commonest site of involvement (5 cases). 6 cases were confirmed with biopsy and other 2 based on clinical signs. All the patients received STS, while only 1 was treated with hyperbaric oxygen. 3 achieved complete response, 1 partial response (ongoing treatment) and 4 deaths due to no response.
Conclusions: Treatment of Calcyphylaxis continues to be a challenge and requires multidisciplinary team approach. STS therapy seems to be the mainstay of the pharmacological management. Withdrawal of cinacalcet and increased use of calcitriol appear to be associated with increased prevalence of CUA.