• Chingching Foocharoen, Ratanavadee Nanagara, Songsak Kiatchoosakun, Siraphop Suwannaroj, and Ajanee Mahakkanukrauh.
• Division of Allergy-Immunology-Rheumatology, Department of Medicine, Khon Kaen University, Thailand. fching@kku.ac.th
• Int J Rheum Dis. 2011 Aug 1;14(3):282-9.

AimsPulmonary 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.MethodsAn 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.ResultsPulmonary 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%).ConclusionUp 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.© 2011 The Authors. International Journal of Rheumatic Diseases © 2011 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd.

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