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Soluble thrombomodulin concentration is raised in scleroderma associated pulmonary hypertension
  1. Richard J Strattona,
  2. Lucie Pompona,
  3. John G Coghlanb,
  4. Jeremy D Pearsonc,
  5. Carol M Blacka
  1. aAcademic Rheumatology and Connective Tissue Disease Unit, The Royal Free Hospital, Rowland Hill Street, London NW3 2PF, bDepartment of Cardiology, The Royal Free Hospital, cVascular Biology Research Centre, Kings College, London
  1. Professor C M Black

Abstract

OBJECTIVE To investigate the expression of thrombomodulin in scleroderma associated pulmonary hypertension.

METHODS Soluble thrombomodulin (sTM), was measured in plasma samples from 34 scleroderma patients shown to have pulmonary hypertension at echocardiogram, and comparison drawn against samples from 38 scleroderma control patients, and 20 healthy controls. Serial measurements of sTM were performed in the 34 patients with scleroderma associated pulmonary hypertension to investigate possible changes in sTM concentration with progression of the condition.

RESULTS Mean sTM was raised in scleroderma associated pulmonary hypertension when compared with scleroderma controls (mean sTM 65.4 ng/mlv 43.3 ng/ml, p<0.05), and when compared with healthy controls (mean sTM 38.1 ng/ml, p<0.05). There was no significant difference between mean sTM in scleroderma controls and healthy controls. Mean sTM concentration did not change with progression of pulmonary hypertension.

CONCLUSION Plasma sTM is raised in scleroderma associated pulmonary hypertension. The pathogenesis of scleroderma associated pulmonary hypertension may be distinct from the pathogenesis of other forms of pulmonary vascular disease.

  • scleroderma
  • pulmonary hypertension
  • thrombomodulin

https://doi.org/10.1136/ard.59.2.132

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