Assessment of respiratory function and need for non-invasive ventilation in a cohort of patients with myotonic dystrophy type 1 followed at one single expert centre.
Carola R Ferrari AggradiElisa FalcierAndrea LizioAlice PirolaJacopo CasiraghiAlice ZanoliniElena CarraroLuca MauroFabrizio RaoElisabetta RomaAntonino IannelloElisa De MattiaAndrea BarpSara LuponeValentina GattiCristina ItalianoValeria Ada SansonePublished in: The clinical respiratory journal (2022)
We provide additional data on the natural history of respiratory function decline and treatment adherence in a relatively large cohort of well-characterized patients with DM1. A high proportion (28%) were lost to follow-up. A minority (11%) required NIV, and only 25% were treatment adherent, irrespective of specific demographics and respiratory features. Our results also confirm previous findings showing that age, disease duration and higher BMIs are predisposing factors for respiratory impairment.