OBJECTIVES: Previous studies into the survival differences between individuals with idiopathic pulmonary fibrosis and those with connective tissue disease associated pulmonary fibrosis (CTD-PF) have yielded mixed results. The aim of this study is to compare the survival of individuals with CTD-PF to those with idiopathic pulmonary fibrosis clinical syndrome (IPF-CS) using data derived from The Health Improvement network, a large primary care database in the UK. METHODS: Incident cases of CTD-PF and IPF-CS between the years 2000-2009 were identified. Survival analysis was performed using Kaplan-Meier methods, stratified by type of connective tissue disease. Cox regression was then used to compare mortality rates between the groups, adjusting for age, gender and year of diagnosis. RESULTS: A total of 324 cases of CTD-PF and 2209 cases of IPF-CS were followed up over a mean period of 2.3 years. During this period, 113 (34.9%) cases of CTD-PF and 1073 (48.6%) cases of IPF-CS died. The mortality rates for cases with CTD-PF and IPF-CS were 123.6 per 1000 person years (95%CI: 102.8-148.9) and 229.8 per 1000 person years (95% CI: 216.4-244.0) respectively. After adjusting for age, sex and year of diagnosis, cases with CTD-PF had a better prognosis compared to those with IPF-CS (HR 0.76,95%CI: 0.62-0.92). CONCLUSION: The prognosis of individuals with CTD-PF appears to be significantly better than those with IPF-CS, but remains an important cause of death in patients with connective tissue disease, and requires more effective treatment options.
OBJECTIVES: Previous studies into the survival differences between individuals with idiopathic pulmonary fibrosis and those with connective tissue disease associated pulmonary fibrosis (CTD-PF) have yielded mixed results. The aim of this study is to compare the survival of individuals with CTD-PF to those with idiopathic pulmonary fibrosis clinical syndrome (IPF-CS) using data derived from The Health Improvement network, a large primary care database in the UK. METHODS: Incident cases of CTD-PF and IPF-CS between the years 2000-2009 were identified. Survival analysis was performed using Kaplan-Meier methods, stratified by type of connective tissue disease. Cox regression was then used to compare mortality rates between the groups, adjusting for age, gender and year of diagnosis. RESULTS: A total of 324 cases of CTD-PF and 2209 cases of IPF-CS were followed up over a mean period of 2.3 years. During this period, 113 (34.9%) cases of CTD-PF and 1073 (48.6%) cases of IPF-CS died. The mortality rates for cases with CTD-PF and IPF-CS were 123.6 per 1000 person years (95%CI: 102.8-148.9) and 229.8 per 1000 person years (95% CI: 216.4-244.0) respectively. After adjusting for age, sex and year of diagnosis, cases with CTD-PF had a better prognosis compared to those with IPF-CS (HR 0.76,95%CI: 0.62-0.92). CONCLUSION: The prognosis of individuals with CTD-PF appears to be significantly better than those with IPF-CS, but remains an important cause of death in patients with connective tissue disease, and requires more effective treatment options.
Authors: Justin M Oldham; Cathryn Lee; Eleanor Valenzi; Leah J Witt; Ayodeji Adegunsoye; Scully Hsu; Lena Chen; Steven Montner; Jonathan H Chung; Imre Noth; Rekha Vij; Mary E Strek Journal: Respir Med Date: 2016-11-04 Impact factor: 3.415
Authors: Liesbeth ten Klooster; George D Nossent; Johanna M Kwakkel-van Erp; Diana A van Kessel; Erik J Oudijk; Ed A van de Graaf; Bart Luijk; Rogier A Hoek; Bernt van den Blink; Peter Th van Hal; Erik A Verschuuren; Wim van der Bij; Coline H van Moorsel; Jan C Grutters Journal: Lung Date: 2015-09-24 Impact factor: 2.584
Authors: Hanna M Nurmi; Minna K Purokivi; Miia S Kärkkäinen; Hannu-Pekka Kettunen; Tuomas A Selander; Riitta L Kaarteenaho Journal: BMC Pulm Med Date: 2017-01-13 Impact factor: 3.317
Authors: Julia Graham; Iazsmin Bauer Ventura; Chad A Newton; Cathryn Lee; Noelle Boctor; Janelle Vu Pugashetti; Claire Cutting; Elena Joerns; Habrinder Sandhu; Jonathan H Chung; Christine Kim Garcia; Michael Kadoch; Imre Noth; Ayodeji Adegunsoye; Mary E Strek; Justin M Oldham Journal: Eur Respir J Date: 2020-07-16 Impact factor: 16.671
Authors: Hanna M Nurmi; Minna K Purokivi; Miia S Kärkkäinen; Hannu-Pekka Kettunen; Tuomas A Selander; Riitta L Kaarteenaho Journal: BMC Pulm Med Date: 2016-07-27 Impact factor: 3.317
Authors: Paola Faverio; Federica De Giacomi; Luca Sardella; Giuseppe Fiorentino; Mauro Carone; Francesco Salerno; Jousel Ora; Paola Rogliani; Giulia Pellegrino; Giuseppe Francesco Sferrazza Papa; Francesco Bini; Bruno Dino Bodini; Grazia Messinesi; Alberto Pesci; Antonio Esquinas Journal: BMC Pulm Med Date: 2018-05-15 Impact factor: 3.317