497. Follistatin Gene Therapy Improves Six Minute Walk Distance in Sporadic Inclusion Body Myositis (sIBM)

Abstract Aims Patients with heart failure with preserved ejection fraction (HFpEF) are usually older and multi-morbid and diagnosis can be challenging. The aims of this cohort study were to confirm diagnosis of HFpEF in patients with possible HFpEF recruited from primary care, to compare characteristics and health status between those with and without HFpEF, and to determine factors associated with health status in patients with HFpEF. Methods Patients with presumed HFpEF were recruited from primary care practices and underwent clinical assessment and diagnostic evaluation as part of a longitudinal cohort study. Health status was measured by Montreal Cognitive Assessment (MOCA), 6-minute walk test, symptoms, and the Kansas City Cardiomyopathy Questionnaire (KCCQ), and quality of life (QoL) by EQ-5D-5L visual analogue scale (VAS). Results 151 patients (mean age 78.5±8.6 years, 40% women, mean EF 56% + 9.4) were recruited and 93 (61.6%) were confirmed HFpEF (those without HFpEF had other HF and cardiac diagnoses). Patients with and without HFpEF did not differ by age, MOCA, blood pressure, heart rate, NYHA class, proportion with atrial fibrillation, Charlson Comorbidity Index, or NT-ProBNP levels. Patients with HFpEF were more likely to be women, overweight or obese, frail, and to be more functionally impaired by 6 minute walk distance and gait speed than those without. Although not statistically significant, patients with HFpEF had clinically significant differences (>5 points) on the physical limitations, symptom burden and clinical summary subscales of the KCCQ, but did not differ by other subscales or by EQ-5D-5L VAS (70±17 vs 73±19, p=0.385). More patients with HFpEF reported daytime dyspnoea (63% vs 46%, p=0.035) and fatigue (81% vs 61%, p=0.008), but not other symptoms compared to those without HFpEF. For both groups BMI was moderately negatively correlated with KCCQ subscale scores, and 6 minute walk distance was positively correlated with KCCQ subscales. Conclusions Nearly 40% were not confirmed as HFpEF indicating the challenges of diagnosis. Patients with confirmed HFpEF differed by sex, overweight/obesity, frailty, functional impairment, and symptoms but not by age or comorbidities from those without HFpEF. These differences were reflected in some subscale scores of the KCCQ, but not how patients reported their quality of life on the KCCQ QoL subscale and EQ-5D-5L VAS. Older patients with HFpEF reported relatively high QoL despite poor health status by functional impairment, frailty and symptoms. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): National Institute of Health Research School of Primary Care Research

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Predictors of reduced 6-minute walk distance after COVID-19: a cohort study in Mexico

Pulmonology ◽

10.1016/j.pulmoe.2021.03.004 ◽

2021 ◽

Author(s):

Alyson W. Wong ◽

Stephanie López-Romero ◽

Esperanza Figueroa-Hurtado ◽

Saul Vazquez-Lopez ◽

Kathryn M. Milne ◽

...

Keyword(s):

Cohort Study ◽

Walk Distance ◽

Minute Walk Distance ◽

Minute Walk

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SAT0332 ANTIBODIES AGAINST CYTOSOLIC 5’-NUCLEOTIDASE 1A IN SPORADIC INCLUSION BODY MYOSITIS: ASSOCIATION WITH CLINICAL AND MRI FEATURES

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.3399 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1112.1-1112

Author(s):

R. Dejthevaporn ◽

S. Shah ◽

S. Wastling ◽

J. Thornton ◽

T. Yousry ◽

...

Keyword(s):

Inclusion Body ◽

Inclusion Body Myositis ◽

Rating Scale ◽

Fatty Infiltration ◽

Connective Tissue Diseases ◽

Diagnostic Process ◽

Antibody Testing ◽

Muscle Mri ◽

Mri Features ◽

Sporadic Inclusion Body Myositis

Background:Autoantibodies directed against cytosolic 5´-nucleotidase 1A (cN1A) have been identified in sporadic inclusion body myositis (sIBM) and other connective tissue diseases. Anti-cN1A antibodies may support the diagnostic process for sIBM as well as potentially provide clues for disease pathogenesis. Nevertheless, the utility of anti-cN1A autoantibody testing in clinical practice remains unclear and requires validation.Objectives:To investigate the association between anti-cN1A antibody status and clinical and MRI features in patients with sIBM.Methods:Data for patients fulfilling European Neuromuscular Centre (ENMC) 2011 criteria for sIBM were obtained from a natural history study database. Demographic, clinical, functional assessment, and muscle MRI data in patients with sIBM who had anti-cN1A autoantibody testing were collected and analysed. Comparisons between subgroups with anti-cN1A antibody status were performed with the Mann-Whitney or Fisher’s exact tests, as appropriate.Results:Forty-nine patients with sIBM had anti-cN1A autoantibody testing, of whom 17 (34.7%) were positive. Twelve patients had muscle MRI performed (seropositivity=5). Demographics, disease duration at antibody testing and overall disease pattern were closely matched in antibody positive and negative cohorts. Dysphagia was more common in the seropositive subgroup (77% vs 47%, p=0.070). Antibody positive patients were more severely affected with a trend to lower IBM functional rating scale (IBMFRS) scores (22.4±8.4 vs 26.7±6.4, p=0.09) with significantly worse ability to climb stairs (0.9±0.9, 1.7±1.1, p=0.02). On T1-weighted MRI more fatty infiltration was found in seropositive patients (Mercuri score: 3.0±0.8 vs 1.7±0.7, p=0.03). Short tau inversion recovery (STIR) hyperintensity was more conspicuous in seropositive patients (STIR extent score: 2.4±0.6 vs 1.4±0.7, p=0.04).Conclusion:There was a trend for more dysphagia and severity of dysphagia in seropositive patients. Differences in upper limb involvement were not seen according to IBMFRS and Medical Research Council (MRC) strength grades. Seropositive patients were more severely affected at the lower limb level, in terms of muscle weakness, physical function, MRI fatty infiltration and muscle inflammation. These results suggest positive antibody status is associated with a worse phenotype. These results have potential implications in clinical trials: whether antibody status influences treatment response should be assessed.Disclosure of Interests:Revadee Dejthevaporn: None declared, Sachit Shah: None declared, Stephen Wastling: None declared, John Thornton: None declared, Tarek Yousry: None declared, Jasper M Morrow: None declared, Pedro M Machado Consultant of: PMM: Abbvie, Celgene, Janssen, Lilly, MSD, Novartis, Pfizer, Roche and UCB, Speakers bureau: PMM: Abbvie, BMS, Lilly, MSD, Novartis, Pfizer, Roche and UCB

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42 Is Mitochondrial Function Measured by 31P Magnetic Resonance Spectroscopy Associated with Physical Performance in Older People with Functional Impairment?

Age and Ageing ◽

10.1093/ageing/afab030.03 ◽

2021 ◽

Vol 50 (Supplement_1) ◽

pp. i12-i42

Author(s):

R R Chungath ◽

M D Witham ◽

C L Clarke ◽

A Hutcheon ◽

S Gandy ◽

...

Keyword(s):

Magnetic Resonance ◽

Magnetic Resonance Spectroscopy ◽

Older People ◽

Physical Performance ◽

Functional Impairment ◽

Resonance Spectroscopy ◽

Walk Distance ◽

31P Magnetic Resonance Spectroscopy ◽

Minute Walk Distance ◽

Minute Walk

Abstract Background Mitochondrial dysfunction has been proposed as a therapeutic target to improve muscle strength and endurance, but the contribution that mitochondrial dysfunction makes to impaired skeletal muscle performance in older people remains unclear. We studied the relationship between phosphocreatine recovery rate (a measure of skeletal muscle mitochondrial function) and physical performance in older people. Methods We analysed data from the Allopurinol in Functional Impairment (ALFIE) trial. Participants aged 65 and over, who were unable to walk 400 m in six minutes, underwent 31P magnetic resonance spectroscopy of the calf after exercise at baseline and at 20 weeks follow up. The phosphocreatine recovery half-life time (t-half) was derived as a measure of mitochondrial function. Participants also undertook the 6-minute walk distance, the Short Physical Performance Battery test (SPPB), and had muscle mass measured using bio-impedance analysis. Bivariate correlations and multivariable regression analyses were conducted to determine associations between t-half and baseline factors. Results One hundred and seventeen people underwent baseline 31P magnetic resonance spectroscopy, mean age 80.4 years (SD 6.0); 56 (48%) were female. Mean 6-minute walk was 291 m (SD 80) and mean SPPB score was 8.4 (SD 1.9). T-half was significantly correlated with SPPB score (r = 0.22, p = 0.02) but not with 6-minute walk distance (r = 0.10, p = 0.29). In multivariable linear regression, muscle mass and weight, but not t-half, were independently associated with SPPB score and with 6-minute walk distance. The change in t-half between baseline and 20 weeks was not significantly associated with the change in SPPB (r = 0.03, p = 0.79) or with the change in 6-minute walk distance (r = −0.11, p = 0.28). Conclusion Muscle mass, but not phosphocreatine recovery time, was associated with Short Physical Performance Battery score and 6-minute walk distance in this cohort of older people with functional impairment.

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