Calciphylaxis and Hypotension: Report of a Case and Review of the Literature

The Role of Iron in Calciphylaxis—A Current Review

“…Although there is no cure for CUA, treatments include intensive dialysis, hyperbaric oxygen, parathyroidectomy, aggressive wound care and cessation of possible drug causes [ 7 , 11 ]. With iron supplementation being a crucial component of anaemia management, cessation of iron treatment has important implications for patients due to the adverse effects associated with anaemia.…”

“…It is a very serious and frequently fatal condition of cutaneous microvascular calcification and thought to be an independent risk factor linked to all-cause mortality [ 2 , 3 ]. Although rare, it is most commonly documented in patients with chronic kidney disease (CKD), in particular those with end-stage renal disease (ESRD), affecting up to 1–4% of ESRD patients, associated with high morbidity and mortality [ 4 , 5 , 6 ] Once diagnosed, prognosis is often poor, even with treatment, with an associated mortality of around 45–80% at one year [ 2 , 7 ]. CKD patients are at increased risk for developing vascular calcification due to their associated uraemia which predisposes to inflammation and endothelial dysfunction, although it has rarely been reported in patients without evidence of renal dysfunction, termed non-uraemic CUA [ 2 , 8 , 9 ].…”

“…CUA can have a wide spectrum of different clinical presentations including painful cutaneous plaques or purpura, erythematous lesions, livedo racemosa, subcutaneous nodules, excruciatingly painful ischaemic, necrotic skin ulceration and subsequent gangrene [ 2 , 7 , 8 , 10 , 11 ]. A frequent complication, and major cause of mortality, in patients with CUA is that of secondary skin infection, which can result in septicaemia and death [ 11 ].…”

“…The precise pathophysiology of microvascular calcification is unclear; it is suggested that there is likely an underlying imbalance between promoters and inhibitors of vascular calcification in those who develop this condition [ 8 ]. Numerous risk factors are suggested in its development; namely ESRD, female gender, Caucasian ethnicity, diabetes mellitus, obesity or significant rapid weight loss, local trauma, abnormalities in coagulation such as protein C or S deficiency, autoimmune disorders, recurrent hypotension, hyperparathyroidism, hypercalcaemia, hyperphosphataemia, elevated alkaline phosphatase, hypoalbuminaemia and exposure to ultraviolet light [ 2 , 5 , 7 , 8 , 9 , 12 , 13 ]. Additionally, there are several medications which have been associated with increased risk, such as, vitamin K antagonists, calcium, calcium-containing phosphate binders, phosphate, erythropoiesis-stimulating agents (ESAs), iron, vitamin D supplements and immunosuppressants, such as methotrexate and corticosteroids [ 5 , 6 , 7 , 12 ].…”

“…Numerous risk factors are suggested in its development; namely ESRD, female gender, Caucasian ethnicity, diabetes mellitus, obesity or significant rapid weight loss, local trauma, abnormalities in coagulation such as protein C or S deficiency, autoimmune disorders, recurrent hypotension, hyperparathyroidism, hypercalcaemia, hyperphosphataemia, elevated alkaline phosphatase, hypoalbuminaemia and exposure to ultraviolet light [ 2 , 5 , 7 , 8 , 9 , 12 , 13 ]. Additionally, there are several medications which have been associated with increased risk, such as, vitamin K antagonists, calcium, calcium-containing phosphate binders, phosphate, erythropoiesis-stimulating agents (ESAs), iron, vitamin D supplements and immunosuppressants, such as methotrexate and corticosteroids [ 5 , 6 , 7 , 12 ]. In a previous study it was found that vitamin K antagonist use, high C-reactive protein, low albumin and low haemoglobin in association with CUA were markers of poor prognosis similar to observations from a French cohort [ 3 , 14 ].…”