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Juvenile dermatomyositis calcifications selectively displayed markers of bone formation
Arthritis Care and Research , 04/02/09

Urganus AL et al. – Study demonstrates that the disorganized juvenile dermatomyositis (DM) calcifications differ in structure, composition, and protein content from bone, suggesting that they may not form through an osteogenic pathway. Osteoclasts at the deposit surface represent an attempt to initiate its resolution.

• Aim was to determine the presence of small integrin-binding ligand N-linked glycoprotein (SIBLING), and bone components in juvenile DM pathologic calcifications
• Calcifications were removed from 4 girls with juvenile DM symptoms
• They were stained for SIBLING proteins, bone markers, and the mineral regulators
• The deposit center, periphery, adjacent connective tissue, and vascular endothelial cells were examined

• Alizarin red stained calcified deposits that did not localize with collagen, like bone, under polarized light
• Hematoxylin and eosin stain revealed a paucity of connective tissue and absence of bone-like structures
• The deposits, connective tissue, and vascular endothelial cells were positive for:
  • bone sialoprotein (BSP)
  • dentin phosphoprotein (DPP)
  • dentin matrix protein 1 (DMP1)
  • and alkaline phosphatase (AP)
• Matrix extracellular phosphoglycoprotein (MEPE) was not detected
• Osteocalcin (OC), osteonectin (ON), and matrix Gla protein (MGP) were present in the deposits and vascular endothelial cells
• Osteopontin (OPN) and core-binding factor -α1 (CBF-α1) were present in deposits and connective tissue
• Tartrate-resistant acid phosphatase (TRAP)-positive osteoclasts were localized to the calcification periphery

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