title: Four-year longitudinal study of clinical and functional endpoints in sporadic inclusion body myositis: implications for therapeutic trials.
abstract: Natural history studies in sporadic inclusion body myositis are of fundamental interest for future therapeutic trials. Previous works have demonstrated the particular relevance of knee extension strength in the follow-up of this disease. This work aimed to extend a preceding natural history over 9 months to a four year period. Thirteen patients were assessed using clinical and functional scales and dynamometry. Except wrist extension torque and manual muscle testing composite score, all the measurements presented a significant decline. The most important changes were observed for knee extension and ankle flexion and extension. The relative change in knee extension strength correlated with the level of strength at baseline. A non-linear correlation was found between 6-minute walk distance and knee extension strength. This study confirms that knee extension strength is particularly relevant to follow patients with sporadic inclusion body myositis. It also shows that a strength loss does not have linear consequences on motor ability. Finally strength and motor ability are complementing each other in the understanding of disease progression.
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France. Electronic address: jy.hogrel@institut-myologie.org.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Hogrel
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France; Université Pierre et Marie Curie, Assistance Publique - Hôpitaux de Paris, GH Pitié-Salpêtrière, Service de Médecine Interne 1, Paris, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Allenbach
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Canal
authors.affiliation: Université Pierre et Marie Curie, Assistance Publique - Hôpitaux de Paris, GH Pitié-Salpêtrière, Service de Médecine Interne 1, Paris, France.
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Leroux
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France.
authors.forename: Gwenn
authors.initials: G
authors.lastname: Ollivier
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France; Université Pierre et Marie Curie, Assistance Publique - Hôpitaux de Paris, GH Pitié-Salpêtrière, Service de Médecine Interne 1, Paris, France.
authors.forename: Kuberaka
authors.initials: K
authors.lastname: Mariampillai
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Servais
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France; Université Pierre et Marie Curie, Assistance Publique - Hôpitaux de Paris, GH Pitié-Salpêtrière, Service de Médecine Interne 1, Paris, France.
authors.forename: Serge
authors.initials: S
authors.lastname: Herson
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Decostre
authors.affiliation: Institut de Myologie, GH Pitié-Salpêtrière, Paris, France; Université Pierre et Marie Curie, Assistance Publique - Hôpitaux de Paris, GH Pitié-Salpêtrière, Service de Médecine Interne 1, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Benveniste
mesh_terms: D000368:Aged; D000842:Ankle / Q000503:physiopathology; D018450:Disease Progression; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007717:Knee / Q000503:physiopathology; D008137:Longitudinal Studies; D008297:Male; D009043:Motor Activity / Q000502:physiology*; D053580:Muscle Strength / Q000502:physiology*; D053581:Muscle Strength Dynamometer; D018482:Muscle, Skeletal / Q000503:physiopathology*; D018979:Myositis, Inclusion Body / Q000503:physiopathology* / Q000628:therapy; D017711:Nonlinear Dynamics; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D016138:Walking / Q000502:physiology; D014953:Wrist / Q000503:physiopathology
keywords: Dynamometry; Inclusion body myositis; Natural history; Outcome measures; Strength