Increased plasma thrombospondin-1 (TSP-1) levels are associated with the TNF alpha-308A allele in children with juvenile dermatomyositis

Clin Immunol. 2002 Jun;103(3 Pt 1):260-3. doi: 10.1006/clim.2001.5212.


Vascular occlusion is more frequent in children with juvenile dermatomyositis (JDM) who have the TNF alpha-308A allele. One of the potent anti-angiogenic factors is thrombospondin-1 (TSP-1). This study investigated the association of the TNF alpha-308A allele with circulating levels of angiogenic mediators, TSP-1, and platelet factor 4 (PF4) using fresh, platelet-poor plasma (PPP). The TNF alpha-308A allele was characterized by PCR amplification and NcoI digestion. Concentrations of TSP-1 and PF4 in PPP from 31 JDM patients and 25 matched pediatric controls were determined by ELISA. The majority of the JDM children with the TNF alpha-308A allele (7/12) produced more TSP-1 than their TNF alpha-308G counterparts (P < 0.05), and their TSP-1 values were inversely related to those for PF4 (P < 0.0006). We conclude that the increased circulating concentrations of TSP-1 associated with the TNF alpha-308A allele suggest that this anti-angiogenic regulator may play a significant role in the augmented vascular occlusion observed in JDM children with this genetic marker.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Alleles*
  • Child
  • DNA / chemistry
  • DNA / genetics
  • Dermatomyositis / blood
  • Dermatomyositis / genetics
  • Dermatomyositis / immunology*
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Humans
  • Male
  • Platelet Factor 4 / analysis
  • Platelet Factor 4 / biosynthesis*
  • Platelet Factor 4 / immunology
  • Polymerase Chain Reaction
  • Thrombospondin 1 / biosynthesis*
  • Thrombospondin 1 / blood
  • Thrombospondin 1 / immunology
  • Tumor Necrosis Factor-alpha / genetics
  • Tumor Necrosis Factor-alpha / immunology*


  • Thrombospondin 1
  • Tumor Necrosis Factor-alpha
  • Platelet Factor 4
  • DNA