Calciphylaxis and its diagnosis: A review

Abstract

Calciphylaxis also known as Calcific uremic arteriolopathy (CUA), is a rare fatal complication usually associated with end-stage renal disease (ESRD). It is characterized by skin ulceration and necrosis leading to significant pain. The disease calciphylaxis is pathological state resulting in accumulation of calcium content in medial wall of small blood vessels along with the fibrotic changes in intima. The aetiopathogenesis of this disease, small vessel vasculopathy, remains complicated, and unclear. It is believed that development of calciphylaxis depends on medial calcification, intimal fibrosis of arterioles and thrombotic occlusion. The disease is rare, life-threatening medical condition that occurs mostly in population with kidney disease or in patients on dialysis. Skin biopsy and radiographic features are helpful in the diagnosis of calciphylaxis, but negative results do not necessarily exclude the diagnosis. This article highlights steps undertaking in the diagnosis of calciphylaxis.

Vascular Calcification—New Insights into Its Mechanism

“…Risk factors for calciphylaxis are high calcium-phosphate product [42], elevated level of parathyroid hormone [43,44], hypoalbuminemia [45,46], diabetes [46][47][48], female sex [45,49], obesity [50], and warfarin overdose [51,52]. This disease is rare, but fatal and even if it is diagnosed at an early stage, the mortality rate is exceptionally high and the success rate of healing is low [53]. The exact cause of calciphylaxis is still unknown, but its pathology includes tunica medial calcification, necrosis of tissue.…”

Skin lesions in a man with end-stage renal disease

“…Calciphylaxis, better characterized as calcific uremic arteriolopathy, is a condition seen most often in patients with ESRD. Calciphylaxis has a significant morbidity and mortality, with 1-year mortality of 45% to 80%; rates are higher in patients with ulcerated lesions 1,2. The pathogenesis of this complex disorder is unclear, but involves subcutaneous vascular calcification, intimal fibrosis of the cutaneous arterioles, and thrombotic occlusion that leads to ischemic skin necrosis 2,3.…”

“…Patients with chronic kidney disease or ESRD are at high risk for calciphylaxis because of chronic electrolyte imbalances; patients on peritoneal dialysis are at the highest risk. Through complex interactions, renal failure leads to secondary hyperparathyroidism and an imbalance of calcium and phosphorous levels 2,3. This imbalance can lead to multiple comorbidities, including bone mineral density disorder, vascular calcification, and calciphylaxis.…”

“…Clinicians should take care in selecting patients for biopsy—prescribe empiric treatment for patients with classic lesions with pain and ulceration, and recommend biopsy for patients with more ambiguous lesions 4. Bone scans with single-photon emission CT can localize calcified areas in the dermis and subcutaneous fat, but are not widely used clinically 2. Laboratory evaluation is largely unhelpful in the diagnosis of calciphylaxis.…”

Nonuremic Calciphylaxis Associated With Hypercalcemia and Rheumatologic Diseases

“…Skin biopsy of the lesions may assist in the diagnosis, but it lacks sensitivity, as the tissue may not necessarily demonstrate calcification. 16 When a biopsy is available, it helps rule out mimics but by itself does not confirm the diagnosis. If diagnosis, and thereby treatment, is delayed, both morbidity and mortality increase significantly.…”