Abstract
BackgroundCalciphylaxis is a life threatening complication in renal patients. Of great importance is the identification of concomitant factors for calciphylaxis. Due to the variability of clinical presentation the evaluation of such factors may be obscured when calciphylaxis diagnosis is based just on clinical features. We aimed to characterize associated factors only in patients with calciphylaxis proven by histomorphological parameters in addition to clinical presentation.MethodsIn a single center retrospective study we analyzed 15 patients in an 8 year period from 2008 to 2016. Only patients with clinical features and histomorphological proof of calciphylaxis were included. Criteria for histological diagnosis of calciphylaxis were intimal hyperplasia, micro thrombi or von Kossa stain positive media calcification.ResultsThe mean age of patients was 64.8 years. Nine patients (60%) were female; 12 (80%) were obese with a Body-Mass-Index (BMI) > 30 kg/m2; 3 (20%) had no renal disease; 12 (80%) had CKD 4 or 5 and 10 (66.7%) had end-stage renal disease (ESRD). One-year mortality in the entire cohort was 73.3%.With respect to medication history, the majority of patients (n = 13 (86.7%)) received vitamin K antagonists (VKA); 10 (66.7%) were treated with vitamin D; 6 (40%) had oral calcium supplementation; 5 (33.3%) had been treated with corticosteroids; 12 (80%) were on proton pump inhibitors (PPI); 13 (86.7%) patients had a clinical proven hyperparathyroidism. Ten (66.7%) patients presented with hypoalbuminemia at diagnosis.ConclusionsThe evaluation of biopsy proven calciphylaxis demonstrates that especially treatment with vitamin K antagonists and liver dysfunction are most important concomitant factors in development of calciphylaxis. As progression and development of calciphylaxis are chronic rather than acute processes, early use of DOACs instead of VKA might be beneficial and reduce the incidence of calciphylaxis.
Highlights
Calciphylaxis is a life threatening complication in renal patients
Calciphylaxis, known as calcific uremic arteriolopathy (CUA), is a rare disease associated with chronic kidney disease (CKD)
Crystals precipitate in the media of small cutaneous blood vessels presumably because of an imbalance of serum calcium- and phosphate-levels [1] as well as a disorder of metabolic modulators such as parathyroid hormone [2]
Summary
Calciphylaxis is a life threatening complication in renal patients. Of great importance is the identification of concomitant factors for calciphylaxis. Due to the variability of clinical presentation the evaluation of such factors may be obscured when calciphylaxis diagnosis is based just on clinical features. We aimed to characterize associated factors only in patients with calciphylaxis proven by histomorphological parameters in addition to clinical presentation. Pathological laboratory findings of parathyroid hormone, serum calcium- and phosphate-levels are typical but not disease defining. Gold standard for diagnosis of calciphylaxis is a positive skin biopsy with well defined diagnostic criteria like the von Kossa stain positive media calcification, intimal hyperplasia, micro thrombi, epidermal ulceration or extravascular soft tissue calcification [6]. In the majority of studies the rate of biopsy proven calciphylaxis is low and diagnosis is often facilitated by clinical and laboratory features [7]. We performed a study of associated factors for calciphylaxis including only patient with biopsy proven calciphylaxis
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