Longitudinal course of lung function in myotonic dystrophy type 1.
BACKGROUND
Quality of life and prognosis among patients with myotonic dystrophy type 1 (DM1) depend on the degree of respiratory impairment. However, the changes over time in pulmonary function in DM1 have not been clearly described.


METHODS
We retrospectively reviewed pulmonary function tests of 80 DM1 patients followed for at least 5 years.


RESULTS
During 9.02 ± 3.4 years of follow-up, the average annual changes were: forced vital capacity (FVC) -0.034 ± 0.06 L (-0.72 ± 1.7% predicted); forced expiratory volume in 1 second (FEV1) -0.043 ± 0.05 L (-1.07 ± 1.7% predicted); and total lung capacity (TLC) -0.047 ± 0.1 L (-1.15 ± 1.7% predicted).


CONCLUSIONS
These results suggest that, compared with other neuromuscular disorders, DM1 is, overall, associated with slowly progressive impairment of lung function. Muscle Nerve 56: 816-818, 2017.
Department of Pulmonary Function Testing and Exercise Physiology, CHRU Nancy, Nancy, F-54000, France.
Catherine
C
Thil
INSERM CIC-1433, CHRU Nancy, Nancy, F-54000, France.
Nelly
N
Agrinier
Department of Pulmonary Function Testing and Exercise Physiology, CHRU Nancy, Nancy, F-54000, France.
Bruno
B
Chenuel
Department of Pulmonary Function Testing and Exercise Physiology, CHRU Nancy, Nancy, F-54000, France.|EA 3450, Development, Adaptation and Disadvantage, Cardiorespiratory Regulations and Motor Control, Université de Lorraine, Nancy, France.
Mathias
http://orcid.org/0000-0002-4386-8606
M
Poussel
D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008137:Longitudinal Studies; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000175:diagnosis* / Q000503:physiopathology*; D012129:Respiratory Function Tests / Q000639:trends; D012189:Retrospective Studies
hypercapnia; hypoxemia; lung function; lung restriction; myotonic dystrophy; neuromuscular disorder