title: Increased plasma soluble CD40 ligand concentrations in systemic sclerosis and association with pulmonary arterial hypertension and digital ulcers.
abstract: BACKGROUND
  Increased expression of CD40L has been reported on activated CD4+ T lymphocytes in systemic sclerosis. CD40L can be expressed in soluble form (sCD40L).
  
  
  OBJECTIVE
  To compare sCD40L concentrations in patients with systemic sclerosis and healthy controls.
  
  
  METHODS
  Quantitative sandwich ELISA was used to measure plasma sCD40L in systemic sclerosis (n = 50) and matched healthy controls (n = 20). Patients with systemic sclerosis had limited cutaneous disease (29), digital ulcers (14), pulmonary arterial hypertension (PAH) (10), pulmonary fibrosis on CT (23), positive anti-Scl70 (14), and anti-centromere antibodies (10). Calcium channel blockers were discontinued 72 hours before measurements.
  
  
  RESULTS
  Median (range) sCD40L concentration (pg/ml) was higher in systemic sclerosis than in controls (495 (10 to 7720) v 79 (50 to 118); p = 0.003), in limited cutaneous disease v diffuse disease (620 (20 to 7720) v 250 (10 to 2690); p = 0.005), in patients with digital ulcers v those without (1430 (36 to 7720) v 370 (10 to 2320); p = 0.002), and in those with PAH v those without (995 (15 to 3850) v 400 (10 to 7720); p = 0.048). sCD40L correlated with pulmonary arterial pressure estimated by Doppler echocardiography (r = 0.41; p = 0.005).
  
  
  CONCLUSIONS
  The soluble form of CD40L is increased in plasma in systemic sclerosis and may be associated with vascular complications of the disease.
authors.affiliation: Rheumatology A, Paris V University, Assistance Publique Hôpitaux de Paris, France.
authors.forename: Y
authors.initials: Y
authors.lastname: Allanore
authors.forename: D
authors.initials: D
authors.lastname: Borderie
authors.forename: C
authors.initials: C
authors.lastname: Meune
authors.forename: H
authors.initials: H
authors.lastname: Lemaréchal
authors.forename: S
authors.initials: S
authors.lastname: Weber
authors.forename: O G
authors.initials: OG
authors.lastname: Ekindjian
authors.forename: A
authors.initials: A
authors.lastname: Kahan
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D023201:CD40 Ligand / Q000097:blood*; D016022:Case-Control Studies; D005260:Female; D005385:Fingers; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology*; D012883:Skin Ulcer / Q000209:etiology / Q000276:immunology*; D012995:Solubility; D018709:Statistics, Nonparametric
chemical_list: D023201:CD40 Ligand