title: Antiendothelial cell antibodies in scleroderma correlate with severe digital ischemia and pulmonary arterial hypertension.
abstract: OBJECTIVE
  To determine the prevalence of IgG antiendothelial cell antibodies (AECA) in patients with scleroderma (systemic sclerosis, SSc) and to correlate it with clinical spectrum and autoantibody profile.
  
  
  METHODS
  Seventy-six patients with SSc and 50 matched healthy controls were studied. Immunological variables were antinuclear antibody (ANA), rheumatoid factor (RF), and Scl-70. IgG-AECA was measured by cellular ELISA.
  
  
  RESULTS
  The prevalence of IgG-AECA was 27.6% in patients with SSc compared to 6% in controls (p < 0.01). Forty percent of patients with diffuse disease had this antibody, versus 13.5% of those with limited cutaneous involvement (p < 0.05). Patients with AECA had significantly higher incidence of digital infarcts and gangrene (p < 0.01) and pulmonary arterial hypertension (p < 0.001) than those without. In the AECA positive group, mean IgG-AECA levels (measured by absorbance values) were significantly higher in patients with digital infarcts (0.91+/-0.31 vs 0.60+/-0.05; p < 0.01) and pulmonary arterial hypertension (1.14+/-0.37 vs 0.68+/-0.13; p < 0.001) compared to those without these features.
  
  
  CONCLUSIONS
  IgG-AECA appears to be an important marker for disease severity in scleroderma.
authors.affiliation: Department of Clinical Immunology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.
authors.forename: V S
authors.initials: VS
authors.lastname: Negi
authors.forename: N K
authors.initials: NK
authors.lastname: Tripathy
authors.forename: R
authors.initials: R
authors.lastname: Misra
authors.forename: S
authors.initials: S
authors.lastname: Nityanand
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D004730:Endothelium, Vascular / Q000276:immunology*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000150:complications / Q000276:immunology*; D007074:Immunoglobulin G / Q000097:blood*; D007511:Ischemia / Q000097:blood / Q000150:complications / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000276:immunology; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications / Q000276:immunology*; D013223:Statistics as Topic
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D007074:Immunoglobulin G; C087589:anti-endothelial cell antibody