Longitudinal observational study of sporadic inclusion body myositis: Implications for clinical trials

Cortese, Andrea; Machado, Pedro; Morrow, Jasper M.; Dewar, L.; Hiscock, A.; Miller, A.; Brady, Stefen; Hilton‐Jones, David; Parton, M.; Hanna, Michael G.

doi:10.1016/j.nmd.2013.02.010

Cited by 72 publications

(83 citation statements)

References 24 publications

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“…Ankle dorsiflexion weakness, facial weakness, and dysphagia can also be seen at early or late stages of the disease, although a small percentage may have dysphagia as the sole presenting symptom [7, 8•, 9, 11, 12]. IBM is rarely associated with malignancy or extra-muscular symptoms, although a high prevalence of autoimmune disorders has been demonstrated in IBM populations [1,9]. Additionally supporting its distinction from PM/DM is the fact that IBM exhibits little response to immunosuppressive therapy [8•, 13-17].…”

Section: Inclusion Body Myositismentioning

confidence: 99%

Myositis Mimics

Michelle

Mammen

2015

Curr Rheumatol Rep

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Patients with autoimmune myositis typically present with muscle weakness, elevated serum levels of muscle enzymes, and abnormal muscle biopsies. However, patients with other acquired myopathies or genetic muscle diseases may have remarkably similar presentations. Making the correct diagnosis of another muscle disease can prevent these patients from being exposed to the risks of immunosuppressive medications, which benefit those with myositis, but not those with other types of muscle disease. Here, we review some of the most common acquired and inherited muscle diseases that can mimic autoimmune myositis, including inclusion body myositis, limb girdle muscular dystrophies, metabolic myopathies, mitochondrial myopathies, and endocrine myopathies. We emphasize aspects of the medical history, physical exam, laboratory evaluation, and muscle biopsy analysis that can help clinicians distinguish myositis mimics from true autoimmune myositis.

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Section: Inclusion Body Myositismentioning

confidence: 99%

Myositis Mimics

Michelle

Mammen

2015

Curr Rheumatol Rep

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“…Quadriceps strength was also found to be a sensitive indicator of disease progression in a French patient cohort (Allenbach et al, 2012). In the UK-based IBM-Net Project to identify outcome measures for clinical trials, a 27.9% decline in strength was found with QMT at one-year follow-up in a group of 23 patients and a 13.8% annual decline in the 10-point IBM Functional Rating Scale (IBM-FRS) and it was concluded that QMT and the IBM-FRS are the best indicators of disease progression (Jackson et al, 2008, Cortese et al, 2013. A longitudinal study of 136 IBM patients from two European Centres used a composite clinical weakness index (ICWI) to assess progression in a subgroup of 71 patients, and found a good correlation with other measures of strength and walking handicap (Benveniste et al, 2011).…”

Section: Natural Historymentioning

confidence: 99%

“…Although initial studies have shown that these therapies may have some mild short-term benefits, targeting the immune response alone does not appear to be sufficient to prevent progression of the disease and as yet there are no effective therapies to counter the abnormal protein homeostasis and degenerative aspects of the disease (Breithaupt and Schmidt 2013). However a phase IIa trial of ariclomol, which increases expression of cytoprotective heat shock proteins in muscle, was shown to slow the rate of deterioration and warrants further investigation (Machado et al, 2013).…”

Section: Treatmentmentioning

confidence: 99%

Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment

Needham

Mastaglia

2016

Clinical Neurophysiology

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Sporadic inclusion body myositis is the most frequent acquired myopathy of middle and later life and is distinguished from other inflammatory myopathies by its selective pattern of muscle involvement and slowly progressive course, and by the combination of inflammatory and degenerative muscle pathology and multi-protein deposits in muscle tissue. This review summarises the findings of recent studies that provide a more complete picture of the clinical phenotype and natural history of the disease and its global prevalence and genetic predisposition. Current diagnostic criteria, including the role of electrophysiological and muscle imaging studies and the recently identified anti-5'-nucleotidase (anti-cN1A) antibody in diagnosis are also discussed as well as current trends in the treatment of the disease.

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“…Másodlagos aerob metabolikus diszfunkció -amelyet a hypoxia talaján kialakuló proinflammatorikus környe-zet indukál -vezet a kóros izomfunkciókhoz [3,5]. A metabolikus diszfunkció, amely károsodott aerob anyagcseréhez vezet, befolyásolja az intrinszik izomfunkciókat és korai izomkifáradást okoz.…”

Section: Kóros Izomteljesítményt Okozó Mechanizmusok Iim-benunclassified

Betekintés az idiopathiás inflammatorikus myopathiában szenvedő betegek gyógytornájába

Váncsa

2016

Orvosi Hetilap

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Idiopathiás inflammatorikus myopathiában szenvedő betegeknél a jelenleg alkalmazott gyógyszeres kezelés mellett izomkárosodás és egészségromlás alakul ki. A gyógytornának az izomteljesítményre, az aerob kapacitásra és az egész-ségre gyakorolt jótékony hatásait vizsgálták krónikus polymyositises és dermatomyositises betegeknél, és a kisebb mértékben aktív betegségben, inclusion body myositisben szenvedő betegeknél. Randomizált vizsgálatok arra utalnak, hogy a fokozott aerob kapacitás egészségjavulással és a klinikai betegségaktivitás csökkenéssel jár együtt. Jelenleg kutatják azokat a mechanizmusokat, amelyek a gyógytornának a vázizomzatra kifejtett hatásai mögött állnak (fokozott mitokondriális kapacitás és kapillárisdenzitás, csökkent laktátszint, aerob fenotípusú génaktiváció, izomnöveke-dési programok, gyulladáshoz kapcsolható gének csökkent kifejeződése). Összességében a gyógytorna hozzájárul mind a szisztémás, mind az izmon belüli adaptációhoz. Aktív betegségben és inclusion body myositisben szenvedő betegeknél a gyógytorna hatásának tanulmányozására randomizált klinikai vizsgálatok szükségesek. Orv. Hetil., 2016, 157(39), 1557-1562.Kulcsszavak: idiopathiás inflammatorikus myopathiák, polymyositis, dermatomyositis, inclusion body myositis, aerob kapacitás, aerob metabolizmus, adaptáció, gyógytorna, vázizomzat, betegségaktivitás, izomfunkció Insight into the training of patients with idiopathic inflammatory myopathyUsing current recommended treatment, a majority of patients with idiopathic inflammatory myopathy develop muscle impairment and poor health. Beneficial effects of exercise have been reported on muscle performance, aerobic capacity and health in chronic polymyositis and dermatomyositis, as well as in active disease and inclusion body myositis to some extent. Importantly, randomized controlled trials indicate that improved health and decreased clinical disease activity could be mediated through increased aerobic capacity. Recently, reports seeking pathomechanisms of the underlying effects of exercise on skeletal muscle indicate increased aerobic capacity (i.e. increased mitochondrial capacity and capillary density, reduced lactate levels), activation of genes of aerobic phenotype and muscle growth programs and down regulation of genes related to inflammation. Exercise contributes to both systemic and withinmuscle adaptations demonstrating that it is fundamental for improving muscle performance and health in patients with idiopathic inflammatory myopathy. There is a need for randomized controlled trials to study the effects of exercise in patients with active disease and inclusion body myositis.Keywords: idiopathic inflammatory myopathies, polymyositis, dermatomyositis, inclusion body myositis, aerobic capacity, aerobic metabolism, exercise adaptations, physical exercise, skeletal muscle, disease activity, muscle function Váncsa, A. [Insight into the training of patients with idiopathic inflammatory myopathy]. Orv. Hetil., 2016, 157(39), 1557-1562. (Beérkezett: 2016 elfogadva: 2016. július

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Longitudinal observational study of sporadic inclusion body myositis: Implications for clinical trials

Cited by 72 publications

References 24 publications

Myositis Mimics

Myositis Mimics

Sporadic inclusion body myositis: A review of recent clinical advances and current approaches to diagnosis and treatment

Betekintés az idiopathiás inflammatorikus myopathiában szenvedő betegek gyógytornájába

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