title: HLA markers for poor prognosis in systemic sclerosis Brazilian patients.
abstract: OBJECTIVE
  The aim of this study was to evaluate human leukocyte antigen (HLA) involvement in the disease expression and poor prognostic clinical features (pulmonary fibrosis and pulmonary arterial hypertension) in patients diagnosed with systemic sclerosis (SSc) in a multiethnic population.
  
  
  METHODS
  SSc patients followed up between 2008 and 2011 were included, and clinical data were obtained through records review. Molecular HLA typing was performed (polymerase chain reaction amplification technique using specific primer sequences). The statistical analysis involved Fisher's exact test and Pearson's corrected chi-square test. P (values) ≤ 0.05 were considered significant. The delta method was used to estimate the variance of the prevalence ratio (PR).
  
  
  RESULTS
  A total of 141 patients (120 women and 21 men) with SSc were studied, including 33.3% with diffuse cutaneous SSc (dcSSc), 62.4% with limited cutaneous SSc (lcSSc), and 4.3% with sine scleroderma. Pulmonary fibrosis was present in 61 patients (43.3%), and the HLA-A∗30 and DQB1∗04 alleles were related to susceptibility. In contrast, the HLA-DRB1∗01 and DQB1∗05 alleles were protective. Pulmonary arterial hypertension was diagnosed in 19 patients (13.5%) and was associated with HLA-B∗35 and C∗04; in contrast, C∗03 seemed to be protective.
  
  
  CONCLUSIONS
  Our current study documents the association of some classes I and II HLA alleles with the most severe clinical manifestations in a multiethnic case series. Our findings differed slightly from the previous data in other populations.
authors.affiliation: Unit of Rheumatology, Department of Internal Medicine, Faculty of Medical Sciences, State University of Campinas (UNICAMP), Campinas, SP, Brazil.
authors.forename: Ana Paula Toledo
authors.initials: AP
authors.lastname: Del Rio
authors.forename: Zoraida
authors.initials: Z
authors.lastname: Sachetto
authors.forename: Percival Degrava
authors.initials: PD
authors.lastname: Sampaio-Barros
authors.forename: João Francisco
authors.initials: JF
authors.lastname: Marques-Neto
authors.forename: Ana Carolina Santos
authors.initials: AC
authors.lastname: Londe
authors.forename: Manoel Barros
authors.initials: MB
authors.lastname: Bertolo
mesh_terms: D001938:Brazil; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005787:Gene Frequency; D056726:Genetic Association Studies; D005819:Genetic Markers; D015234:HLA-A Antigens / Q000235:genetics*; D015235:HLA-B Antigens / Q000235:genetics; D015236:HLA-C Antigens / Q000235:genetics; D059866:HLA-DQ beta-Chains / Q000235:genetics*; D059811:HLA-DRB1 Chains / Q000235:genetics*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000235:genetics; D008297:Male; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000235:genetics; D012595:Scleroderma, Systemic / Q000235:genetics*
chemical_list: D005819:Genetic Markers; D015234:HLA-A Antigens; C069526:HLA-A*30 antigen; D015235:HLA-B Antigens; D015236:HLA-C Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D059811:HLA-DRB1 Chains; C497841:HLA-DRB1*01 antigen