title: Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody.
abstract: OBJECTIVE
  Pulmonary arterial hypertension (PAH) and severe pulmonary fibrosis (SPF) are the most common causes of death in scleroderma. Our study focuses on lung disease in patients with a nucleolar antibody in comparison to other scleroderma-specific autoantibodies.
  
  
  METHODS
  Patients initially seen between 1972 and 1995 (and followed through 2004) with [systolic pulmonary artery pressure (sPAH) (PASP > 50 mm Hg] or SPF [forced vital capacity (FVC%) < 55% predicted) were grouped by the presence of anticentromere antibody (ACA), an isolated antinucleolar antibody (ANoA), or an antitopoisomerase antibody-I (TOPO).
  
  
  RESULTS
  Twenty percent of ACA, 23% of TOPO, and 32% of ANoA patients had severe lung disease (p < 0.005). In ANoA patients with PAH without severe fibrosis, the FVC was lower (71% predicted) than in ACA patients, suggesting they had some interstitial fibrosis. However, they had a higher FVC%/diffusing capacity for carbon monoxide (DLCO)% ratio than the ACA patients (2.4 vs 1.8). pulmonary hypertension in TOPO patients was associated with a lower FVC%/DLCO% ratio and lower levels of PAP than either the PAH in ACA or ANoA patients.
  
  
  CONCLUSIONS
  Scleroderma-specific autoantibodies are associated with characteristic subgroups of lung disease. The ANoA patients have a unique mixture of PAH and SPF subgroups of lung disease. Scleroderma-specific autoantibodies and the FVC%/DLCO% ratio are helpful in determining whether a patient has PAH alone, PAH along with pulmonary fibrosis, or secondary PAH from chronic hypoxia with SPF.
authors.affiliation: Department of Medicine, Division of Rheumatology, Allergy and Immunology, Georgetown University, 3800 Reservoir Road, Washington, DC 20007, USA.
authors.forename: Virginia D
authors.initials: VD
authors.lastname: Steen
authors.forename: Mary
authors.initials: M
authors.lastname: Lucas
authors.forename: Noreen
authors.initials: N
authors.lastname: Fertig
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Medsger
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood*; D002466:Cell Nucleolus / Q000276:immunology; D002503:Centromere / Q000276:immunology; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000276:immunology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000209:etiology* / Q000276:immunology* / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000276:immunology* / Q000503:physiopathology; D014797:Vital Capacity
chemical_list: D000974:Antibodies, Antinuclear; D004264:DNA Topoisomerases, Type I