Calciphylaxis (Calcific Uremic Arteriolopathy/cua)

What is Calciphlaxis ?

The term “ Calciphlaxis” refers to a an inflammatory disease of small- and medium-sized arteries characterized by 

  • Calcification
  • Thromboembolism
  • painful cutaneous ulcerations, that may cause serious infections that can lead to death

Why does Calciphlaxis occur ?

Although there are many reports of calciphylaxis associated with nonuremic causes, the majority of cases occur in patients with end-stage renal disease on dialysis. 

Possible risk factors, that can trigger calciphlaxis include:

  • Being female
  • Obesity
  • Diabetes
  • Abnormalities in blood-clotting factors
  • An imbalance of calcium, phosphorus and aluminum in the body
  • Some medications, such as warfarin (Coumadin, Jantoven), calcium-binding agents or corticosteroids.

 

In most cases, calciphylaxis happens because calcium deposits form in and block the blood vessels that supply the tissue. Although this can happen in all tissues (eyes, lungs, brain, muscles, and intestines in rare cases) the main involvement is seen at the skin at the lower limbs or areas with higher fat content, such as the breasts, buttocks, and abdomen. 

  • early stages: lesions of calciphylaxis are focal and appear as erythema or livedo reticularis with or without subcutaneous plaques or ulcers. 
  • Advanced stages: As the disease progresses, the ischemic changes coalesce to form denser violaceous, painful, plaque-like subcutaneous nodules with eschar. In the advanced stages, the eschar or ulceration involves an extensive area. 

What Forms of Calciphylaxia are there?

Calciphylaxis can be classified as 

  • uremic (in patients with End-Stage Renal disease/ESRD: 1 – 4 % of all patients) 
  • nonuremic (in patients with normal renal function or earlier stages of chronic kidney disease)

What are the consequences of Calciphylaxis

Proximal areas with increased adipose tissue such as the abdomen, thighs, and buttocks are most commonly involved, although distal sites such as the digits can also be affected. 

  • Extreme pain is a hallmark of calciphylaxis 
  • Skin ulcer leading to considerable morbidity

How can you treat Calciphlaxis ?

Multidisciplinary management mainly involves:

  • Pain control
  • avoiding local trauma (Prevention)
  • stopping causative agents such as warfarin and corticosteroids
  • treating and preventing infection
  • intensive hemodialysis with an increase in both frequency and duration
  • intravenous sodium thiosulphate; non-calcium-phosphorus binders and cinacalcet in patients with elevated parathyroid hormone
  • hyperbaric oxygen
  • debridement and wound closure (very conservative)