title: The association of antiphospholipid antibodies with cardiopulmonary manifestations of systemic sclerosis.
abstract: OBJECTIVE
  To determine the prevalence and correlates of antiphospholipid antibodies (APLA) in systemic sclerosis (SSc).
  
  
  METHODS
  Nine hundred and forty SSc patients were tested for APLA using an ELISA assay at recruitment. Clinical manifestations were defined as present, if ever present from SSc diagnosis. Logistic regression analysis was used to determine the associations of APLA.
  
  
  RESULTS
  One or more types of APLA were present in 226 (24.0%) patients. Anticardiolipin (ACA) IgG (ACA-IgG) antibodies were associated with right heart catheter-diagnosed pulmonary arterial hypertension (PAH), with higher titres corresponding with a higher likelihood of PAH (moderate titre (20-39 U/ml) ACA-IgG odds ratio [OR] 1.70, 95% CI: 1.01-2.93, p=0.047; high titre (>40 U/ml) ACA-IgG OR 4.60, 95% CI:1.02-20.8, p=0.047). Both ACA-IgM (OR 2.04, 95% CI: 1.4-3.0, p<0.0001) and ACA-IgG (OR 1.84, 95% CI: 1.2-2.8, p=0.005) were associated with interstitial lung disease (ILD). Increasing ACA-IgM and IgG titres were associated with increased likelihood of ILD. ACA-IgG was a marker of coexistent pulmonary hypertension and ILD (ILD-PH) (OR 2.10, 95% CI: 1.1-4.2, p=0.036). We also found an association between ACA-IgG and digital ulcers (OR 1.76, 95% CI: 1.16-2.67, p=0.008) and ACA-IgM and Raynaud's phenomenon (OR 2.39, 95% CI: 1.08-5.27, p=0.031). There was no association between APLA and SSc disease subtype, peak skin score, presence of other autoantibodies, mortality or other disease manifestations.
  
  
  CONCLUSIONS
  The association of APLA with PAH, ILD, ILD-PH, Raynaud's phenomenon and digital ulcers suggests that endothelial abnormalities and small vessel thrombosis may be important in the pathogenesis of these disease features.
authors.affiliation: Department of Rheumatology, St. Vincent's Hospital, Melbourne, Australia. kbmorrisroe@gmail.com.
authors.forename: K B
authors.initials: KB
authors.lastname: Morrisroe
authors.forename: W
authors.initials: W
authors.lastname: Stevens
authors.forename: H
authors.initials: H
authors.lastname: Nandurkar
authors.forename: D
authors.initials: D
authors.lastname: Prior
authors.forename: V
authors.initials: V
authors.lastname: Thakkar
authors.forename: J
authors.initials: J
authors.lastname: Roddy
authors.forename: J
authors.initials: J
authors.lastname: Zochling
authors.forename: J
authors.initials: J
authors.lastname: Sahhar
authors.forename: K
authors.initials: K
authors.lastname: Tymms
authors.forename: A
authors.initials: A
authors.lastname: Sturgess
authors.forename: G
authors.initials: G
authors.lastname: Major
authors.forename: F
authors.initials: F
authors.lastname: Kermeen
authors.forename: C
authors.initials: C
authors.lastname: Hill
authors.forename: J
authors.initials: J
authors.lastname: Walker
authors.forename: P
authors.initials: P
authors.lastname: Nash
authors.forename: E
authors.initials: E
authors.lastname: Gabbay
authors.forename: P
authors.initials: P
authors.lastname: Youssef
authors.forename: S M
authors.initials: SM
authors.lastname: Proudman
authors.forename: M
authors.initials: M
authors.lastname: Nikpour
mesh_terms: D000368:Aged; D017153:Antibodies, Anticardiolipin / Q000276:immunology*; D017152:Antibodies, Antiphospholipid / Q000276:immunology; D015331:Cohort Studies; D005260:Female; D006229:Hand Dermatoses / Q000209:etiology / Q000276:immunology; D006331:Heart Diseases / Q000209:etiology / Q000276:immunology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000276:immunology*; D007074:Immunoglobulin G / Q000276:immunology; D007075:Immunoglobulin M / Q000276:immunology; D016015:Logistic Models; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011928:Raynaud Disease / Q000209:etiology / Q000276:immunology; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology*; D012883:Skin Ulcer / Q000209:etiology / Q000276:immunology
chemical_list: D017153:Antibodies, Anticardiolipin; D017152:Antibodies, Antiphospholipid; D007074:Immunoglobulin G; D007075:Immunoglobulin M