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. 2023 May 31;120(21-22):365–372. doi: 10.3238/arztebl.m2023.0066

eTable 3. Occurrence of medial sclerosis in association with various diseases (examples) with references.

Disease Key feature Additional soft-tissue calcifications References
Diabetes mellitus Carbohydrate metabolism disorders No (e7)
Chronic kidney disease Impaired excretory and metabolic renal function No (e11)
Aging Unknown; disorders of the extracellular matrix?
Destruction of elastic fibers?
No (e22)
Primary medial sclerosis Unknown, hereditary genetic disorder? No (e23)
Disorders of Vitamin K metabolism Inhibition of anti-inflammatory factors, particularly mediated by the NF-kB pathway; inhibition of matrix Gla protein (MGP) carboxylation No (e24)
Disorders of Vitamin D metabolism Stimulation of calcium absorption, synergistic effect with parathyroid hormone on bone resorption? No (e25)
Atherosclerosis Intimal calcification Yes (e26)
Pseudoxanthoma elasticum Defect of the ABCC6 gene ATP Binding Cassette Subfamily C Member 6 Yes (e27)
Rheumatoid arthritis? Disorders of human leucocyte antigen (HLA), major histocompatibility complex (MHC), cytokine promotors, T-cell signaling pathway, and others Yes (e28)
β-thalassemia Mutation of the β-globulin gene, resulting in absent or low production of β-globulin Yes (e29)
Calciphylaxis Unknown Yes (e30)
Kawasaki disease Unknown; generalized inflammatory reaction secondary to infection in predisposed children? Yes (e31)
Singleton-Merten syndrome and other forms of type I interferonopathies Genetic mutation associated with activation of type I interferon activity Yes (e32)
Parathyroid gland dysfunction Disorders of parathyroid hormone metabolism Yes (e33)
Generalized Arterial Calcification of Infancy (GACI) Gene mutations affecting ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1), which cleaves ATP to inorganic pyrophosphate (PPi) and adenosine monophosphate (AMP) (extracellular) Yes (e34)
Arterial Calcification due to CD73 Deficiency (ACDC) Ecto-5‘-nucleotidase (NT5E) gene mutation which codes CD73—with its mutation, the conversion of adenosine monophosphate (AMP) into adenosine is defective Yes (e35)
Idiopathic Basal Ganglia Calcification (IBGC) Impaired extracellular transport of inorganic phosphate by mutations of the SLC20A2, PDGFRB, PDGFB, XPR1, and MYORG genes. Yes (e36)
Scleroderma Autoimmune disease frequently associated with changes in the human leukocyte antigen (HLA) complex Yes (e37)
Hutchinson-Gilford progeria syndrome Single nucleotide substitution in the LMNA gene Yes (e38)