title: Risk factors associated with pulmonary arterial hypertension in Colombian patients with systemic sclerosis: review of the literature.
abstract: OBJECTIVE
  Considering the significant morbidity and mortality of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) and the lack of precise information on disease in Latin America, we investigated the clinical and laboratory characteristics associated with PAH in Colombian patients with SSc and review the literature.
  
  
  METHODS
  This multicenter study included patients followed at 5 rheumatology units that were systematically assessed using a pretested questionnaire on clinical and immunological variables, focusing on PAH. Conditional logistic regression was employed to assess association between PAH and specific clinical characteristics. A systematic review of the literature was performed through electronic databases.
  
  
  RESULTS
  Of a total of 349 patients with SSc, 61 (17%) met the criteria for PAH. Pulmonary fibrosis [adjusted odds ratio (AOR) 7.37, 95% CI 3.67-14.81, p < 0.0001], microstomia (AOR 3.3, 95% CI 1.70-6.28, p < 0.0001), gastroesophageal reflux (AOR 2.41, 95% CI 1.31-4.43, p = 0.005), dysphagia (AOR 2.7, 95% CI 1.49-4.77, p = 0.001), hyperpigmentation (AOR 2.15, 95% CI 1.11-4.16, p = 0.02), and hypopigmentation (AOR 2.4, 95% CI 1.26-4.64, p = 0.008) were the most prevalent clinical characteristics associated with PAH, while anemia (AOR 5.4, 95% CI 1.98-14.93, p = 0.001) was observed as the unique laboratory risk factor. Association between subtypes of SSc and PAH was not observed. Significant differences in both clinical and laboratory data were observed among different series.
  
  
  CONCLUSIONS
  PAH may be a frequent complication of SSc in the Colombian population regardless of disease subtype. The identified clinical and laboratory risk factors might assist earlier diagnosis and guide decisions on therapeutic interventions on this critical complication of SSc. The reasons underlying the reported divergences among patients from different ethnicities are not fully understood, but it is most likely that both genetic and environmental factors are responsible for them.
authors.affiliation: Rheumatology Unit, Universidad Nacional de Colombia, Bogota, Colombia.
authors.forename: Paola
authors.initials: P
authors.lastname: Coral-Alvarado
authors.forename: Adriana
authors.initials: A
authors.lastname: Rojas-Villarraga
authors.forename: María C
authors.initials: MC
authors.lastname: Latorre
authors.forename: Ruben D
authors.initials: RD
authors.lastname: Mantilla
authors.forename: José F
authors.initials: JF
authors.lastname: Restrepo
authors.forename: Aryce L
authors.initials: AL
authors.lastname: Pardo
authors.forename: Philippe
authors.initials: P
authors.lastname: Chalem
authors.forename: Federico
authors.initials: F
authors.lastname: Rondón
authors.forename: Edwin
authors.initials: E
authors.lastname: Jáuregui
authors.forename: Juan C
authors.initials: JC
authors.lastname: Rueda
authors.forename: Carlos
authors.initials: C
authors.lastname: Cañas
authors.forename: María E
authors.initials: ME
authors.lastname: Hincapie
authors.forename: Ricardo
authors.initials: R
authors.lastname: Pineda-Tamayo
authors.forename: Fausto
authors.initials: F
authors.lastname: Alvarez
authors.forename: Antonio
authors.initials: A
authors.lastname: Iglesias-Gamarra
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: Diaz
authors.forename: Juan-Manuel
authors.initials: JM
authors.lastname: Anaya
mesh_terms: D000328:Adult; D000368:Aged; D003105:Colombia / Q000453:epidemiology; D005260:Female; D005764:Gastroesophageal Reflux / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D011658:Pulmonary Fibrosis / Q000150:complications; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000453:epidemiology