title: Prognostic factors of mortality and 2-year survival analysis of systemic sclerosis with pulmonary arterial hypertension in Thailand.
abstract: OBJECTIVE
  Pulmonary arterial hypertension (PAH) is a major complication and cause of death in systemic sclerosis (SSc). Our objective was to identify the predictive factors of mortality and the 2-year survival rate among Thai sufferers of PAH-SSc.
  
  
  METHODS
  An historical cohort study was performed among PAH-SSc patients followed up at Srinagarind Hospital, Thailand, between January 2005 and December 2008. Kaplan-Meier and Cox regression analyses were used to estimate the probability of survival and to assess the significant factors associated with death.
  
  
  RESULTS
  Pulmonary arterial hypertension was recognized in 60 patients using ECHO criteria, right ventricular systolic pressure (RVSP) > 35 mmHg. Two-thirds of the patients were female, > 50 years of age, with the dSSc subtype. Twenty patients (33.3%) died: the mortality rate was 15.6% per 100 person-years. The respective 1-, 2-, 3- and 4-year survival rate was 86.1%, 71.3%, 64.6% and 53.9%. The majority (85%) died without any specific treatment for PAH. Using univariate analysis, the mortality risk was associated with: the WHO functional class (FC) III (HR = 27.82), visceral organ involvement (HR = 5.14), myositis (HR = 3.14), esophageal dysmotility (HR = 3.08) and pericarditis (HR = 2.84). Using Cox regression, the only predictor of death was FCIII. The causes of death in PAH-SSc were related to PAH (60%), infection (30%) and acute renal failure (10%).
  
  
  CONCLUSIONS
  Up to one-third of Thai sufferers of PAH-SSc died within 2 years of PAH diagnosis, without any specific treatment being given. Increased mortality risk was found in SSc patients who had FCIII and visceral organ involvement.
authors.affiliation: Division of Allergy-Immunology-Rheumatology, Department of Medicine, Khon Kaen University, Thailand. fching@kku.ac.th
authors.forename: Chingching
authors.initials: C
authors.lastname: Foocharoen
authors.forename: Ratanavadee
authors.initials: R
authors.lastname: Nanagara
authors.forename: Songsak
authors.initials: S
authors.lastname: Kiatchoosakun
authors.forename: Siraphop
authors.initials: S
authors.lastname: Suwannaroj
authors.forename: Ajanee
authors.initials: A
authors.lastname: Mahakkanukrauh
mesh_terms: D000328:Adult; D000368:Aged; D002423:Cause of Death; D015331:Cohort Studies; D015897:Comorbidity; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000401:mortality*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis / Q000401:mortality*; D015996:Survival Rate; D013785:Thailand / Q000453:epidemiology; D013997:Time Factors