Visceral calciphylaxis

Orpha code: 280068OMIM code:

Definition

A rare, life-threatening, non-inflammatory vasculopathy characterized by diffuse precipitation of calcium in viscera (mainly in the heart or lungs, but also in the stomach or kidneys) leading to fibrosis and thrombosis, which eventually causes tissue necrosis. Depending on the affected organ, patients may present with dyspnea, cough and respiratory failure or acute heart block and subsequent sudden cardiac death. The disease predominantly affects patients with end-stage kidney disease (ESKD) on dialysis.

Disease data
Classification

Clinical subtype

ORPHA code
280068
OMIM code
-
ICD10 code
E83.5
ICD11 code
EB90.42

No additional description.

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