title: Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry.
abstract: OBJECTIVE
  To determine the association between serum autoantibodies and survival in patients with incident systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry.
  
  
  METHODS
  Patients with definite PAH diagnosed by right heart catheterization within 6 months of registry enrollment were studied. Serum autoantibodies were assayed at each participating institution's clinical laboratory. Mortality data were collected from electronic medical records and/or the Social Security Death Index. Kaplan-Meier survival estimates were reported for five autoantibody groups (anticentromere/AC, nucleolar ANA/NUC, anti-topoisomerase/Scl-70, overlapping or non-specific autoantibodies/other, and a combined group with similar survival consisting of RNA polymerase III, U1RNP, and autoantibody-negative patients). Cox proportional hazards models permitted examination of the association between autoantibody groups and overall survival, controlling for age, sex, race, and SSc disease duration.
  
  
  RESULTS
  In all, 162 subjects had PAH, and serum autoantibody and survival information; 60 (37%) had AC, 39 (24%) NUC, 11 (7%) Scl-70, 28 (17%) had other, 9 (6%) RNA pol, 8 (5%) U1RNP autoantibodies, and 7 (4%) had negative antibodies; 32 (20%) subjects died over a median follow-up time of 2.1 years (range: 0.01-6.8); 1- and 3-year survival estimates were, respectively, 94% and 78% for AC, 94% and 72% for NUC, 89% and 63% for Scl-70, 92% and 79% for the other group, and 100% and 93% for the combined group. Unadjusted and adjusted hazard ratios revealed no statistically significant association between risk of death and autoantibodies.
  
  
  CONCLUSIONS
  Anticentromere and NUC autoantibodies are prevalent in SSc-PAH patients. An association between serum autoantibodies and survival in patients with SSc-PAH was not identified in the PHAROS cohort.
authors.affiliation: Department of Medicine, Northwestern University Feinberg School of Medicine, 240 E Huron St, McGaw Pavilion Suite M300, Chicago, IL 60611; Institute for Public Health and Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL. Electronic address: m-hinchcliff@northwestern.edu.
authors.forename: Monique
authors.initials: M
authors.lastname: Hinchcliff
authors.affiliation: Harvard University, Cambridge, MA.
authors.forename: Saira
authors.initials: S
authors.lastname: Khanna
authors.affiliation: Department of Medicine, Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ.
authors.forename: Vivien M
authors.initials: VM
authors.lastname: Hsu
authors.affiliation: Institute for Public Health and Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL; Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL.
authors.forename: Jungwha
authors.initials: J
authors.lastname: Lee
authors.affiliation: Department of Medicine, Northwestern University Feinberg School of Medicine, 240 E Huron St, McGaw Pavilion Suite M300, Chicago, IL 60611.
authors.forename: Orit
authors.initials: O
authors.lastname: Almagor
authors.affiliation: Department of Medicine, Northwestern University Feinberg School of Medicine, 240 E Huron St, McGaw Pavilion Suite M300, Chicago, IL 60611; Institute for Public Health and Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL; Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL.
authors.forename: Rowland W
authors.initials: RW
authors.lastname: Chang
authors.affiliation: Department of Medicine, Georgetown University, Washington, DC.
authors.forename: Virginia
authors.initials: V
authors.lastname: Steen
authors.affiliation: Department of Medicine, Stanford University and Palo Alto VA Health Care System, Palo Alto, CA.
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000150:complications / Q000276:immunology / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012042:Registries; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications / Q000276:immunology / Q000401:mortality*
chemical_list: D001323:Autoantibodies
keywords: Mortality; Pulmonary arterial hypertension; Pulmonary hypertension; Risk factors; Scleroderma; Serum autoantibody; Systemic sclerosis