PT  - JOURNAL ARTICLE
AU  - Clara Hjalmarsson
AU  - Göran Rådegran
AU  - David Kylhammar
AU  - Bengt Rundqvist
AU  - Jonas Multing
AU  - Magnus D. Nisell
AU  - Barbro Kjellström
ED  - ,
TI  - IMPACT OF AGE AND COMORBIDITY ON RISK STRATIFICATION IN IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION
AID  - 10.1183/13993003.02310-2017
DP  - 2018 Jan 01
TA  - European Respiratory Journal
PG  - 1702310
4099  - http://erj.ersjournals.com/content/early/2018/03/21/13993003.02310-2017.short
4100  - http://erj.ersjournals.com/content/early/2018/03/21/13993003.02310-2017.full
AB  - Background. Recent reports from worldwide pulmonary hypertension registries show a new demographic picture of the idiopathic pulmonary arterial hypertension (IPAH) population, with an increasing prevalence among the elderly.Aim. To investigate the effect of age and comorbidity on risk stratification and outcome of patients with incident IPAH.Methods and results. The study population (n=264) was categorized in four age groups; 18–45, 46–64, 65–74, and ≥75 years. Individual risk profile was determined according to a risk assessment instrument, based on the ESC/ERS guidelines. Change in risk group from baseline to follow-up (median 5 months), and survival were compared across age groups. In the two youngest age groups, a significant number of patients improved (18–45 years; Z = −4.613, p <0.001 and 46–64 years; Z= −2.125, p=0.034), but no significant improvement was found in the older patients. Five-year survival was highest in patients 18–45 years (88%), while the survival rates were 63%, 56%, and 36% for patients in the groups 46–64, 65–74 and ≥75 years, respectively (p<0.001). Ischaemic heart disease and kidney dysfunction independently predicted survival.Conclusion. These findings highlight the importance of age and specific comorbidities as prognostic markers of outcome, in addition to established risk assessment algorithms.Change in risk category at follow-up and specific comorbidity predict survival in IPAH across age groupsFootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Hjalmarsson reports personal fees from Actelion Pharmaceuticals Sweden, outside the submitted work.Conflict of interest: Dr. Rådegran reports grants from Actelion Pharmaceuticals Sweden AB, grants from GlaxoSmith-Kline, personal fees from Actelion Pharmaceuticals Sweden AB, personal fees from GlaxoSmith-Kline, personal fees from Bayer Health Care, personal fees from Actelion Pharmaceuticals Sweden AB, personal fees from GlaxoSmith-Kline, personal fees from Bayer Health Care, non-financial support from Actelion Pharmaceuticals Sweden AB, non-financial support from GlaxoSmith-Kline, non-financial support from Bayer Health Care, non-financial support from Pfizer, non-financial support from United Therapeutics, non-financial support from Novartis, outside the submitted work.Conflict of interest: Dr. Kylhammar reports personal fees from Actelion Pharmaceuticals Sweden, personal fees from GlaxoSmith-Kline, grants from Actelion Pharmaceuticals Sweden, grants from Pfizer, grants from Bayer Health Care, outside the submitted work.Conflict of interest: Dr. Rundqvist reports non-financial support from Actelion Pharmaceuticals Sweden AB, non-financial support from GlaxoSmith-Kline, non-financial support from Bayer Health Care, non-financial support from United Therapeutics, outside the submitted work.Conflict of interest: Mr. Multing has nothing to disclose.Conflict of interest: M.N. reports personal lecture fees from Actelion Pharmaceuticals Sweden AB, Pfizer, Bayer Health Care, NordicInfu Care, Novartis, Astra Zeneca, Glaxo-SmithKline, Boehringer Ingelheim, and Takeda. M.N. has been the primary or co-investigator in clinical trials for Boehringer Ingelheim, Astra Zeneca, Takeda, Novartis, and United Therapeutics and has been involved in research advisory boards for Actelion Pharmaceuticals Sweden AB, Bayer Health Care, NordicInfu Care, and Glaxo-SmithKlineConflict of interest: Dr. Kjellström has nothing to disclose.