Sameer Desai1, Hubert Wong1, Jenna Sykes2,3, Anne L Stephenson2,3, Joel Singer1,4, Bradley S Quon5,6. 1. 1 School of Population and Public Health, and. 2. 2 Division of Respirology, University of Toronto, Toronto, Ontario, Canada. 3. 3 Adult Cystic Fibrosis Program, St. Michael's Hospital, Toronto, Ontario, Canada; and. 4. 4 Canadian HIV Trials Network and. 5. 6 Division of Respiratory Medicine, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada. 6. 5 Centre for Heart Lung Innovation, St. Paul's Hospital and University of British Columbia, Vancouver, British Columbia, Canada.
Abstract
RATIONALE: Individuals diagnosed with cystic fibrosis (CF) as adults represent a growing subpopulation of CF cases, but there are limited studies describing their characteristics and prognosis. OBJECTIVES: The objectives of this study were to describe the clinical characteristics of individuals with adult-diagnosed CF, estimate survival rates in this population, and identify clinical predictors of reduced survival at baseline. METHODS: The Canadian CF Patient Registry was used to identify patients with CF who were ≥18 years of age at diagnosis and received a diagnosis between 1990 and 2014. Clinical characteristics were described and the Kaplan-Meier method was used to estimate lung-transplant-free survival. Multivariable Cox regression analysis and adjusted survival curves were employed to identify important predictors of reduced survival at the time of diagnosis (i.e., baseline) and to produce adjusted effects. RESULTS: A total of 362 adults were diagnosed with CF during the study period. The median follow-up time was 7.7 years and 48 individuals experienced an event (15 transplants, 33 deaths without transplant). The median age at diagnosis was 34.3 years, with the majority of individuals presenting with pulmonary and/or gastrointestinal symptoms (71%). Lung-transplant-free survival was 88% at 10 years and 86% at 15 years. Age at diagnosis (hazard ratio [HR], 1.24 per 5-year increase, 95% confidence interval [CI], 1.09-1.43), diabetes (HR, 4.19; 95% CI, 1.35-13.01), and lung function (HR, 1.35 per 5% decrease in forced expiratory volume in 1 second % predicted; 95% CI, 1.24-1.48) at baseline were important predictors of reduced survival. CONCLUSIONS: CF care providers can use this information to inform individuals who received a diagnosis of CF as adults about their prognosis and to guide the necessity of treatments, specifically with regard to those who are at high risk for a worse prognosis.
RATIONALE: Individuals diagnosed with cystic fibrosis (CF) as adults represent a growing subpopulation of CF cases, but there are limited studies describing their characteristics and prognosis. OBJECTIVES: The objectives of this study were to describe the clinical characteristics of individuals with adult-diagnosed CF, estimate survival rates in this population, and identify clinical predictors of reduced survival at baseline. METHODS: The Canadian CF Patient Registry was used to identify patients with CF who were ≥18 years of age at diagnosis and received a diagnosis between 1990 and 2014. Clinical characteristics were described and the Kaplan-Meier method was used to estimate lung-transplant-free survival. Multivariable Cox regression analysis and adjusted survival curves were employed to identify important predictors of reduced survival at the time of diagnosis (i.e., baseline) and to produce adjusted effects. RESULTS: A total of 362 adults were diagnosed with CF during the study period. The median follow-up time was 7.7 years and 48 individuals experienced an event (15 transplants, 33 deaths without transplant). The median age at diagnosis was 34.3 years, with the majority of individuals presenting with pulmonary and/or gastrointestinal symptoms (71%). Lung-transplant-free survival was 88% at 10 years and 86% at 15 years. Age at diagnosis (hazard ratio [HR], 1.24 per 5-year increase, 95% confidence interval [CI], 1.09-1.43), diabetes (HR, 4.19; 95% CI, 1.35-13.01), and lung function (HR, 1.35 per 5% decrease in forced expiratory volume in 1 second % predicted; 95% CI, 1.24-1.48) at baseline were important predictors of reduced survival. CONCLUSIONS: CF care providers can use this information to inform individuals who received a diagnosis of CF as adults about their prognosis and to guide the necessity of treatments, specifically with regard to those who are at high risk for a worse prognosis.
Authors: Scott C Bell; Marcus A Mall; Hector Gutierrez; Milan Macek; Susan Madge; Jane C Davies; Pierre-Régis Burgel; Elizabeth Tullis; Claudio Castaños; Carlo Castellani; Catherine A Byrnes; Fiona Cathcart; Sanjay H Chotirmall; Rebecca Cosgriff; Irmgard Eichler; Isabelle Fajac; Christopher H Goss; Pavel Drevinek; Philip M Farrell; Anna M Gravelle; Trudy Havermans; Nicole Mayer-Hamblett; Nataliya Kashirskaya; Eitan Kerem; Joseph L Mathew; Edward F McKone; Lutz Naehrlich; Samya Z Nasr; Gabriela R Oates; Ciaran O'Neill; Ulrike Pypops; Karen S Raraigh; Steven M Rowe; Kevin W Southern; Sheila Sivam; Anne L Stephenson; Marco Zampoli; Felix Ratjen Journal: Lancet Respir Med Date: 2019-09-27 Impact factor: 30.700
Authors: Matthew S McCravy; Nancy L Quinney; Deborah M Cholon; Susan E Boyles; Timothy J Jensen; Andrei A Aleksandrov; Scott H Donaldson; Peadar G Noone; Martina Gentzsch Journal: Eur Respir J Date: 2020-07-30 Impact factor: 16.671