FRI0258 CUMULATIVE INCIDENCE, SURVIVAL AND PREDICTORS OF PULMONARY HYPERTENSION IN SYSTEMIC SCLEROSIS SUBSETS: PAH IS NOT INCREASED IN LIMITED VS DIFFUSE PATIENTS BY ADJUSTED COMPETING RISK ANALYSIS Conference Paper uri icon

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abstract

  • Background:Pulmonary hypertension (PH) is a life-threatening complication of systemic sclerosis (SSc), thought to be more commonly found in limited cutaneous (lcSSc) compared to diffuse (dcSSc) subset. Since lcSSc has a better prognosis, it is unclear whether a higher occurrence of PH in lcSSc reflects survival bias.Objectives:To compare the cumulative PH incidence in disease subsets, after accounting for death as a competing event, in a large multi-center SSc cohort.Methods:Cumulative incidence of PH was studied in 1431 Canadian Scleroderma Research Group (CSRG) database patients (57% lcSSc; follow-up 3.5±2.9 years, range 1-14) by Fine-Gray analysis, unadjusted and adjusted for sex, age and SSc-related autoantibodies (SAS 9.4). Survival curves, predictors of PH development and survival were analyzed by Kaplan-Meier and Cox proportional hazards analyses (SPSS 25.0). Subgroup analysis was performed for PAH.Results:157 SSc patients had PH (including 117 PAH), either confirmed by RHC or postmortem. Compared to those without PH, lcSSc-PH patients had longer disease and older age at SSc diagnosis, while dcSSc-PH patients - more severe peripheral vascular and gastrointestinal involvement. The cumulative incidences of PH/PAH were similar in dcSSc and lcSSc after accounting for death in the adjusted competitive risk model (Table 1; Fig.1). 47% of PH- and 42% of PAH-patients died over a FU period. Male gender (p<0.0001) and anti-Scl-70 (p<0.001) were associated with earlier PH development, while older age (p=0.006) - with PAH (Table 2). ACA-negativity and older age predicted worse PH prognosis.Figure 1.Cumulative incidence curves for PH (A) and PAH (B).Conclusion:Cumulative incidence of PH, after accounting for death as competing event, was comparable in SSc subsets. Vigilance should be considered in males, Scl-70 positive and late age-onset SSc.Table 1.Sub-distribution Hazard ratio of incident PH and PAH.PHPAHHazard ratio (95% CIs)P valuesHazard ratio (95% CIs)P valuesCrude ModelDcSSc vs lcSSc2.03 (1.13, 3.66)0.01861.60 (0.82, 3.16)0.1710Adjusted modelDcSSc vs lcSSc1.82 (0.93, 3.57)0.08181.57 (0.69, 3.59)0.2812Female vs male0.98 (0.42, 2.32)0.96602.10 (0.51, 8.65)0.3040Age1.00 (0.99, 1.02)0.70411.01 (0.98, 1.03)0.5498AntibodiesACA vs negative0.95 (0.46, 1.96)0.89911.08 (0.50, 2.35)0.8391ATA vs negative1.93 (0.84, 4.42)0.11980.59 (0.13, 2.73)0.4970Anti-RNAP vs negative1.24 (0.45, 3.43)0.68411.77 (0.58, 5.44)0.3181Disclosure of Interests:Tatiana Nevskaya: None declared, Yuxuan Jiang: None declared, Mianbo Wang: None declared, Murray Baron: None declared, Janet Pope Grant/research support from: AbbVie, Bristol-Myers Squibb, Eli Lilly & Company, Merck, Roche, Seattle Genetics, UCB, Consultant of: AbbVie, Actelion, Amgen, Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Eicos Sciences, Eli Lilly & Company, Emerald, Gilead Sciences, Inc., Janssen, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi, UCB, Speakers bureau: UCB

authors

  • Nevskaya, T
  • Jiang, Y
  • Wang, M
  • Baron, M
  • Pope, J
  • Pope, Janet E
  • Baron, Murray
  • Hudson, Marie
  • Gyger, Geneviève
  • Larche, Margaret
  • Khalidi, Nader A
  • Masetto, Ariel
  • Sutton, Evelyn
  • Robinson, David
  • Rodriguez-Reyna, Tatiana S
  • Smith, Doug
  • Thorne, Carter
  • Fortin, Paul R
  • Fritzler, Marvin J

publication date

  • June 2020