title: Respiratory failure due to muscle weakness in inflammatory myopathies: maintenance therapy with home mechanical ventilation.
abstract: Polymyositis and dermatomyositis are idiopathic inflammatory myopathies. Respiratory complications are a common feature, but ventilatory insufficiency is rare in these patients. We describe here three patients diagnosed with inflammatory myopathy (polymyositis) with respiratory failure due to muscle weakness who did not respond to immunosuppressive therapy. Mechanical ventilation at home with nasal or tracheal intermittent positive pressure resulted in improved chronic hypoventilation. This treatment improves the quality of life of patients with inflammatory myopathies and can be lifesaving in some cases.
authors.affiliation: Department of Internal Medicine and. Pneumology Service, Vall D'Hebron General Hospital, Barcelona, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Selva-O'Callaghan
authors.forename: L
authors.initials: L
authors.lastname: Sanchez-Sitjes
authors.forename: X
authors.initials: X
authors.lastname: Muñoz-Gall
authors.forename: T
authors.initials: T
authors.lastname: Mijares-Boeckh-Behrens
authors.forename: R
authors.initials: R
authors.lastname: Solans-Laque
authors.forename: J
authors.initials: J
authors.lastname: Angel Bosch-Gil
authors.forename: F
authors.initials: F
authors.lastname: Morell-Brotad
authors.forename: M
authors.initials: M
authors.lastname: Vilardell-Tarrés
mesh_terms: D000280:Administration, Inhalation; D000328:Adult; D005260:Female; D006699:Home Care Services; D006801:Humans; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000150:complications*; D017285:Polymyositis / Q000150:complications* / Q000628:therapy*; D011175:Positive-Pressure Respiration; D011788:Quality of Life; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy*; D014132:Trachea