Takayasu's arteritis concurrent with Marfan syndrome--a case report

Angiology. 2000 May;51(5):435-9. doi: 10.1177/000331970005100512.

Abstract

Marfan syndrome (MS) is a dominantly inherited connective tissue disorder characterized by arachnodactyly, tall stature, the presence of aortic aneurysm, and lens dislocation. Takayasu's arteritis (TA) is a chronic vasculitis that primarily affects the aorta and its branches. The authors report the first case of TA in a patient with MS. The simultaneous presence of TA and MS could be a coincidence, however; the pathogenesis of TA might be linked with autoimmunity induced by abnormal extracellular matrix protein derived from the genetic mutations in MS.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aorta, Thoracic / pathology
  • Aortic Aneurysm, Thoracic / complications
  • Aortic Aneurysm, Thoracic / genetics
  • Aortic Aneurysm, Thoracic / immunology
  • Aortography
  • Autoantibodies / blood
  • Chromosomes, Human, Pair 15
  • Extracellular Matrix Proteins / genetics
  • Extracellular Matrix Proteins / immunology
  • Female
  • Fibrillins
  • Humans
  • Magnetic Resonance Imaging
  • Marfan Syndrome / complications*
  • Marfan Syndrome / genetics
  • Marfan Syndrome / immunology
  • Microfilament Proteins / genetics
  • Microfilament Proteins / immunology
  • Phenotype
  • Takayasu Arteritis / complications*
  • Takayasu Arteritis / genetics
  • Takayasu Arteritis / immunology

Substances

  • Autoantibodies
  • Extracellular Matrix Proteins
  • Fibrillins
  • Microfilament Proteins