Prognosis of patients with alpha1-antitrypsine deficiency on long-term oxygen therapy

TY - JOUR

T1 - Prognosis of patients with alpha1-antitrypsine deficiency on long-term oxygen therapy

AU - Ringbaek, Thomas J

AU - Seersholm, Niels

AU - Perch, Michael

AU - Iversen, Martin

AU - Lange, Peter

N1 - Copyright © 2014 Elsevier Ltd. All rights reserved.

PY - 2014/8

Y1 - 2014/8

N2 - Introduction Data on patients with alpha1-antitrypsine deficiency (AATD) on long-term oxygen therapy (LTOT) is sparse. The aim of this study was to present the incidence of patients with AATD on LTOT, and compare their characteristics, comorbidities and prognosis (lung transplantation, termination of LTOT, and survival) with COPD patients without AATD. Methods A National prospective study of all COPD patients who started LTOT for the first time in the period 01.11.1994 to 31.12.2010. Results Among the 21,964 patients on LTOT, 234 patients had AATD. AATD patients were more often males and were on average about 17 years younger than patients without AATD. Cardio-vascular diseases and diabetes mellitus were significantly less prevalent among patients with AATD (60.4% versus 70.3% (P < 0.001) and 4.7% versus 12.2% (P < 0.001)), whereas osteoporosis was more frequent (28.5% versus 20.4%, p = 0.002. Eighty-nine (38.0%) AATD patients and 173 (0.8%) non-AATD patients were lung transplanted in the study period. Median survival was 8.7 years in AATD patients with lung transplantation, 3.3 years in AATD patients without lung transplantation, 6.3 years in non-AATD patients with lung transplantation, and 1.6 years in non-AATD without lung transplantation. Even after adjustment for gender, age, comorbidities, and the time between start of LTOT and lung transplantation, patients with AATD had a lower risk of death compared to non-AATD patients (Hazard ratio 0.73 (95% CI: 0.62-0.86; P < 0.001). Conclusions Compared with COPD without AATD, AATD patients are younger, more often males, have a lower prevalence of cardio-vascular diseases and diabetes mellitus, and higher prevalence of osteoporosis. Moreover, they have better prognosis, partly due to greater chance of receiving a lung transplantation.

AB - Introduction Data on patients with alpha1-antitrypsine deficiency (AATD) on long-term oxygen therapy (LTOT) is sparse. The aim of this study was to present the incidence of patients with AATD on LTOT, and compare their characteristics, comorbidities and prognosis (lung transplantation, termination of LTOT, and survival) with COPD patients without AATD. Methods A National prospective study of all COPD patients who started LTOT for the first time in the period 01.11.1994 to 31.12.2010. Results Among the 21,964 patients on LTOT, 234 patients had AATD. AATD patients were more often males and were on average about 17 years younger than patients without AATD. Cardio-vascular diseases and diabetes mellitus were significantly less prevalent among patients with AATD (60.4% versus 70.3% (P < 0.001) and 4.7% versus 12.2% (P < 0.001)), whereas osteoporosis was more frequent (28.5% versus 20.4%, p = 0.002. Eighty-nine (38.0%) AATD patients and 173 (0.8%) non-AATD patients were lung transplanted in the study period. Median survival was 8.7 years in AATD patients with lung transplantation, 3.3 years in AATD patients without lung transplantation, 6.3 years in non-AATD patients with lung transplantation, and 1.6 years in non-AATD without lung transplantation. Even after adjustment for gender, age, comorbidities, and the time between start of LTOT and lung transplantation, patients with AATD had a lower risk of death compared to non-AATD patients (Hazard ratio 0.73 (95% CI: 0.62-0.86; P < 0.001). Conclusions Compared with COPD without AATD, AATD patients are younger, more often males, have a lower prevalence of cardio-vascular diseases and diabetes mellitus, and higher prevalence of osteoporosis. Moreover, they have better prognosis, partly due to greater chance of receiving a lung transplantation.

KW - Cardiovascular Diseases

KW - Diabetes Complications

KW - Female

KW - Humans

KW - Long-Term Care

KW - Lung Transplantation

KW - Male

KW - Middle Aged

KW - Osteoporosis

KW - Oxygen

KW - Prognosis

KW - Prospective Studies

KW - Pulmonary Disease, Chronic Obstructive

KW - Sex Factors

KW - alpha 1-Antitrypsin Deficiency

U2 - 10.1016/j.rmed.2014.05.001

DO - 10.1016/j.rmed.2014.05.001

M3 - Journal article

C2 - 24906692

SN - 0954-6111

VL - 108

SP - 1189

EP - 1194

JO - Respiratory Medicine

JF - Respiratory Medicine

IS - 8

ER -