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REVIEW ARTICLE
Ahead of print publication  

Sporadic inclusion body myositis in the rheumatology clinic


 Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, SP, Brazil (BR), Brazil

Correspondence Address:
Samuel Katsuyuki Shinjo,
Division of Rheumatology, Laboratório de Miopatias Inflamatórias, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, SP
Brazil
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_80_20

  Abstract 


Sporadic inclusion body myositis (sIBM) is a progressive and insidious skeletal muscle disorder characterized classically by the quadriceps, wrist, and finger flexor weakness. The main irreversible complications of sIBM are dysphagia and walking difficulties. The disease affects more men than women, and the symptom onset mainly occurs between 50 and 70 years of age. Due to its slow progression, sIBM diagnosis is frequently delayed and therefore misdiagnosed as other muscle diseases. sIBM remains refractory to treatment (e.g., glucocorticoid, and immunosuppressive/immunomodulatory/immunobiological drugs). Although there have been no robust clinical trials, training exercise/physiotherapy should be prescribed regularly in sIBM patients.

Keywords: Idiopathic inflammatory myopathies, inclusion body myositis, myopathies, myositis, review



How to cite this URL:
de Souza FH, Behrens Pinto GL, de Souza JM, Olivo Pallo PA, Hoff LS, Shinjo SK. Sporadic inclusion body myositis in the rheumatology clinic. Indian J Rheumatol [Epub ahead of print] [cited 2020 Oct 30]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=289182




  Introduction Top


Sporadic inclusion body myositis (sIBM) is a rare disorder characterized by late-onset muscle disease with distinct classical features, which include progressive muscle weakness and hypo/atrophy of the quadriceps and flexors of the fingers, histopathology with infiltration of cytotoxic T-cells.[1],[2]

sIBM is acquired inflammatory muscle disease and it should not be mistaken for hereditary inclusion body myopathies (not myositis). These later are hereditary diseases with autosomal recessive or dominant inheritance. To distinguish these groups, it is rather to define them as sporadic or hereditary.

The prevalence varies considerably depending on the region under analysis, and according to a recent meta-analysis, the prevalence is 45.6 cases/million habitants.[3] It affects more men than women at a ratio of 2~3:1.[4] The average age reported is 61–66 years, and sIBM is even less common in non-Caucasian individuals.[1],[4]

sIBM has been associated with different systemic autoimmune diseases, such as rheumatoid arthritis, Sjögren's syndrome, systemic lupus erythematosus, and others.[5],[6] HIV-infected patients may have histological abnormalities on muscle biopsy equal to sIBM.[2] A higher incidence of cancer has been found in some but not all population-based studies.[5],[7],[8] Furthermore, patients with sIBM have more comorbidities, such as hypertension, dyslipidemia, diabetes mellitus, neoplasia, and osteoporosis.[5],[9],[10],[11]

Although the mechanisms for the development of disease are still incompletely understood, there are two main theories for the pathophysiology of sIBM: one involving autoimmunity and another with a neurodegenerative perspective.[2]

The postulate of autoimmunity is reinforced by the increased incidence of other autoimmune diseases in these patients. An important finding related to the immune system was the identification of cytotoxic CD8+ T-cell endomysial infiltrates capable of surrounding and invading nonnecrotic muscle fibers.[1],[4] Another evidence of autoimmunity was the discovery of the antibody anti-cytosolic 5'-nucleotidase 1A (cN1A) in 2011.[12]

The neurodegenerative perspective of the disease is built on the lack of response to glucocorticoids and immunosuppressive drugs, and the finding of protein aggregates in muscle fibers, frequently associated with other neurodegenerative diseases.[4] Aggregates of β-amyloid, hyperphosphorylated tau, heavy chain neurofilament, and Tar-DNA-binding protein-43 (TDP-43) ubiquitin, among others, have been described in muscle immunohistochemical studies of patients with sIBM.[1],[13],[14] Important data to corroborate this hypothesis is the degeneration of myocyte nuclei, which generates rimmed vacuoles and leads to anomalous displacement of DNA-binding proteins to the cytoplasm of muscle fibers.[1]

It is important to emphasize that these two hypotheses are not mutually exclusive, and possible link mechanisms between these two main theories are still being elucidated. There is also evidence about genetic defects in autophagy and mitochondrial dysfunction, as possible mechanisms involved in the pathophysiology of sIBM.[2],[15]


  Methods Top


The present study was performed by searching the sentence “inclusion body myositis” for research works available on the Medline (PubMed) from 2000 to 2020. The following articles were selected for our revision: English version, reviews, original articles, controlled clinical trials, and case series.

Development

Clinical manifestations

The most common clinical manifestation in sIBM is asymmetric proximal and distal muscle weakness, of slow progression toward disability, generally of >12 months of evolution.[13]

Patients initially report symptoms related to muscle groups that are particularly affected in sIBM: quadriceps (difficulty rising from a chair and difficulty in climbing stairs and falls), finger flexors and ankle dorsiflexors (falls and foot drop).[1],[2],[4],[9],[10],[16] On physical examination, muscle weakness is typically accompanied by atrophy in the quadriceps muscles and impaired grip strength in finger flexors [Figure 1].
Figure 1: Clinical features of patients with inclusion body myositis. (a) Atrophy of finger flexors; (b) thigh atrophy. Figures from the author's personal archive

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Dysphagia is found in 42%–65% of patients, which is associated with high morbidity and mortality because of nutritional deficiency, weight loss, aspiration pneumonia, and reduction of quality of life.[17],[18],[19]

Finally, other findings on physical examination in individuals with sIBM, which may be useful at the time of differential diagnosis, show greater weakness of elbow flexion than shoulder abduction; greater muscle weakness in the quadriceps than the hips, and finger flexion is ever weaker than shoulder abduction.[1],[2],[20],[21]

Classification criteria

As the recognition of sIBM is a subtype of systemic autoimmune myopathies, many diagnostic criteria have been developed and suggested.[22] However, Griggs et al. criteria and the European Neuromuscular Centre (ENMC) IBM research diagnostic criteria of 2011 are used widely in clinical research and practice.[23],[24]

Griggs et al. criteria[23] are based on the clinical and laboratory data [Table 1] and consider definite sIBM if all three pathological features (mononuclear cell invasion of nonnecrotic muscle fibers, rimmed vacuoles, and either amyloid deposits or 15–18 nm tubule filaments by electronic microscopy) are present and independent of the clinical features. They also define possible sIBM if clinical features exist, with inflammatory myopathy but without rimmed vacuoles and amyloid or tubule filaments.
Table 1: Griggs et al. criteria for sporadic inclusion body myositis

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The ENMC research diagnostic criteria of 2011,[24] which are based in the clinical, laboratory, and pathological features [Table 2], recognize three main groups of patients: those with classical clinical and pathological features (clinic and pathologically defined sIBM), those with classical clinical features but limited pathological changes (clinically defined sIBM), and patients with characteristic lower and upper and limb patterns of weakness but not both (probable sIBM).
Table 2: European Neuromuscular Centre 2011 sporadic inclusion body myositis diagnostic criteria

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Subsequently, Lloyd et al. assessed the sensitivity and specificity of all available diagnostic criteria for sIBM and showed that all diagnostic groups had high specificity (≥97%) but varied substantially in their sensitivities (11%–84%). The probable IBM group from the ENMC 2011 criteria was the best performing category with a sensitivity of 84%.[25]

Complementary exams

Muscle biopsy

Before the muscle for biopsy, it is relevant not to choose either a muscle which is so involved by the disease process (e.g., atrophic muscles) or a muscle which is so little affected or submitted to electromyogram (EMG) recently.

A classical biopsy finding in sIBM is characterized by atrophic muscle fibers, infiltration of nonnecrotic myofibers by predominant of CD8+ T-cells in an endomysial and perivascular pattern, and rimmed vacuoles [Figure 2]. Increased numbers of cytochrome c oxidase-negative fibers are also frequently noted in the muscle biopsies of individuals with sIBM. Moreover, the major histocompatibility complex I is upregulated on immunostaining [Figure 2]. Cytoplasmic inclusions of β-amyloid may be visualized using Congo red and polarized light.[26]
Figure 2: Inclusion body myositis – typical histological finding in muscle biopsy legends: *Infiltration of muscle endomysium by immune cells – hematoxylin and eosin staining; **Deep invasion of non-necrotic myofiber by immune cells – hematoxylin and eosin staining; ***Rimmed vacuoles in Gomori trichrome stained

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In addition, abnormal sarcoplasmic deposition of TDP-43 or p62 immunostaining may help to discriminate individuals with polymyositis that are going to convert sIBM, but there has been no study to elucidate p62 utility in inflammatory myopathies.[27]

Electroneuromyography

Electroneuromyography can be useful in distinguishing neuropathy from myopathy weakness. Needle electromyography typically shows an irritative myopathy that is related to fibrillation potentials.[4],[28] EMG findings of sIBM include irritability of the muscle fibers at rest and during needle insertion and myopathic motor unit potentials during contraction.[4],[28] However, neuropathic features can also be found in approximately one-third of the IBM patients caused by reinnervation of denervated.[4],[28] In some cases, the neurogenic motor unit action potentials in sIBM may be appropriately dense to minimize the myopathic changes, leading to a misdiagnosis of motor neuron disorders.[28]

Magnetic resonance imaging

The evaluation of the muscle condition has many benefits in sIBM. It is one of the tools used to guide diagnosis because the pattern of involvement may suggest the clinical hypothesis and might be used to guide biopsy and the acquisition of images may serve as a monitoring method. Up to date, magnetic resonance imaging (MRI) is the cornerstone for muscle assessment in systemic autoimmune myopathies in general.[29],[30]

The pattern of muscular involvement in sIBM as seen by MRI has been studied extensively in many cross-sectional studies, most of them depicting it as a disease that most often affects the anterior compartment of the thighs.[29],[30],[31] Frequently, there is an important degree of atrophy and fatty infiltration, mainly in the quadriceps femoris muscles and relatively sparing the rectus femoris and the posterior compartment.[29],[31] Some studies also reported a high degree of fatty infiltration and atrophy in the gastrocnemius muscles.[29],[30] The girdle muscles are affected less frequently, and in the forearm, there is a striking predilection for the flexor digitorum profundus (forearm).[29] In general, the pattern of involvement seems to be so solid that an MRI-based criterion was already proposed, with good specificity and moderate sensibility.[30] [Figure 3] shows a typical thigh MRI from a patient with sIBM.
Figure 3: Magnetic resonance in a patient with inclusion body myositis T1 – axial section of thigh (a: Proximal; b: Medium; c: Distal). A significant “melted” appearance with fatty infiltration/fibrous tissue and atrophy is observed in the distal part of the thigh

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Autoantibodies

Anti-cN1A (or anti-NT5C1A) is a highly specific antibody detected in sIBM patients: studies have shown a sensitivity of 33%–76% and a specificity of 87%–100%.[32] Interestingly, anti-cN1A antibodies are also detected in 20%–30% of individuals with juvenile dermatomyositis, systemic lupus erythematosus, and Sjögren's syndrome but not in adults with other systemic autoimmune myopathies.[33],[34] Therefore, anti-cN1A antibody testing may be useful to differentiate an atypical case of sIBM from other forms of adult myopathies and should not be used as a screening test for sIBM (given the low-moderate sensitivity). Anti-cN1A does not seem to related to disease severity or to a specific clinic pathological phenotype of sIBM.[32] They also have not been comprised as diagnostic criteria for sIBM.

Other autoantibodies detected less frequently are antinuclear antibodies, anti-Ro, anti-La, and rheumatoid factor. Myositis-specific autoantibodies are usually negative in individuals with sIBM.[6],[35]

Laboratory tests

Levels of creatine phosphokinase serum are usually <10 times the normal limit.[6],[36] Erythrocyte sedimentation rate is frequently normal.[6] All patients with suspected sIBM should also have a complete blood workup to rule out other causes of myopathy, including endocrine and electrolyte disorders.

Drug treatment

The management of sIBM remains the main challenge because of the coexistence of inflammatory and degenerative courses. No effective treatment has been known so far, and the standard regimens used for other types of inflammatory myopathies do not stabilize sIBM.[1],[2]

Nonimmunobiological treatment

Glucocorticosteroids, azathioprine, methotrexate, or β-interferon-1a are not effective in sIBM.[37],[38],[39] Moreover, Benveniste et al.. demonstrated that 71 individuals who received prednisone associated with intravenous human immunoglobulins, azathioprine, or methotrexate (median duration of 40.8 months) were more affected on disability measures than controls.[40]

A double-blinded, placebo-controlled study with methotrexate (5–20 mg/week) showed in 44 patients no difference in development and muscle strength at 48 weeks but did show decreased serum creatine phosphokinase activity.[38]

In a pilot study, interferon was well tolerated at low doses, and the adverse event profile was similar to placebo. However, no differences were shown in muscle strength or mass at 6 months.[37]

Similar results were found in biopsies from a placebo-controlled study with intravenous human immunoglobulin and glucocorticoids that down-modulated inflammatory mediators but miscarried to block nitric oxide stress in the muscle and in an analogous in-vitro model.[39] It can be related to stabilization or even temporary, mild upgrading of muscle strength and upgraded dysphagia in several individuals with sIBM.[41]

A small clinical study demonstrated that oxandrolone had a doubtful substantial effect in improving whole-body strength and a relevant effect in refining upper-extremity strength.[42] In 10 patients who completed a trial of simvastatin, the management appeared safe and well tolerated, but none of them showed a substantial clinical upgrading.[43]

In a prospective, randomized, double-blind, placebo-controlled trial, 22 individuals received oral rapamycin and 22 received placebos over 12 months. No difference in the main outcome (quadriceps strength) was recognized, but significantly less fatty replacement was found in the quadriceps and hamstrings. Improvements in sIBM weakness composite index and the forced vital capacity were noted in the dynamically treated limb.[44]

Immunobiological treatment

Etanercept and anakinra failed to demonstrate improvement.[45],[46] Barohn et al. used etanercept (25 mg two times a week) for 17.0 months, and there was no alteration between the treated group and the placebo group at 6 and 12 months when observing at elbow flexors or at 6 months when observing at handgrip. In the treated individuals, there was a minor but important improvement in handgrip at 12 months.[45]

In a minor pilot study with anakinra, four individuals with biopsy-proven sIBM were treated for 7.7 months. However, no upgrading in muscle strength or steadying was observed in any of the individuals based on grip strength and medical research council dimensions.[46]

Alemtuzumab (0.3 mg/kg/day for 4 days) led to a slowdown of disease development up to 6 months, improved the strength of some individuals, and reduced endomysial inflammation and stressor molecules.[47] In a post hoc analysis, a downregulation of inflammatory markers was noted without an effect on numerous crucial markers of cell stress and degeneration, which could possibly explain the inadequate long-term effect of this treatment.[48]

A global, randomized, double-blind placebo-controlled trial with bimagrumab (anti-activin type II receptor antibody) showed no improvement in the 6-min walking distance test after 52 weeks of treatment.[49]

A long-term open-label study with canakinumab (150 mg subcutaneous injection, bi-monthly for 4 months, and then monthly) showed minor but not clinically considerable, stabilizing profits in two of five individuals with sIBM.[50]

Other treatments

Physical exercise

The rationale behind attempting to treat IBM with physical exercise is the notion that this entity is a complex and multifactorial disease that should be tackled in a multidisciplinary manner. Despite the promising results reported in case studies and even clinical experience, the evidence sustaining physical training in sIBM is still fragile. Most studies are not controlled or randomized, and the heterogeneity of the training protocols makes it difficult to grasp the ideal intervention. Currently, there is limited evidence to funding the safety of physical exercise in sIBM. Based on the conflicting and low-quality evidence, physical exercise might slightly improve muscle strength or, at least, reduce the speed of decline.

Many previous studies addressed home-based programs, especially resistive training or combined (resistive and aerobic) exercises.[51],[52],[53] These protocols established the safety of exercise training in sIBM and some of them presented gains in strength and functionality. One study also evaluated aerobic exercise in community-based facilities and, although underpowered to grasp differences between groups, was able to find strong size effects of the intervention regarding improvement in VO2 peak.[54]

Moreover, one randomized clinical trial evaluated the effect of blood-flow restricted resistance training over physical function and was unable to reach the primary endpoint. Even still, the authors emphasize that they could find differences regarding the amount of loss of knee extensor strength between groups, suggesting that the exercise had a protective role in the subjects.[55] In summary, there is a scientific penumbra regarding physical exercise in sIBM that calls for high-quality randomized controlled trials. Efforts to homogenize the interventions are welcome for these trials, but from what science indicates so far, an individual approach might be more suitable in the real-life clinical practice.

Nutrition and supplementation

Considering the mechanisms underlying the pathogenesis of sIBM, some clinicians empirically prescribe combinations of L-carnitine, coenzyme Q10, B complex vitamins, and creatine for patients.[56] Although those substances may participate in one or more pathways involved in the muscle metabolism and mitochondrial respiratory chain, we could not find evidence for benefits in the literature, not even considering those with low quality. At present, no ongoing trials were found to be registered in ClinicalTrials.gov concerning supplementation in sIBM.

Differential diagnosis

The classical sIBM manifestations are characterized by proximal leg or distal arm weakness. The most important differential diagnoses are the other inflammatory myopathies, neuromuscular diseases, and neurological diseases that can affect the lower motor neuron [Table 3].
Table 3: Differential diagnosis of patients with inclusion body myositis

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Some important demographic considerations and clinical features can help to differentiate sIBM from other diseases. For instance, the age at onset of sIBM is generally 50 years and older, is more prevalent in men than women, and its evolution is slow and chronic, particularly over 12 months, unlike other subgroups of systemic autoimmune myopathies with an acute or subacute course.[21],[22] Hereditary inclusion body myopathies, for instance, usually occurs at the age between 30 and 40 years. Asymmetric distribution and arrangement of proximal and distal muscle weakness observed in sIBM is particularly uncommon in other groups of myopathies, and a greater atrophy of quadriceps (especially vastus medialis), flexor muscle of the fingers, and thenar and hypothenar muscles also are frequently found only in sIBM.[21],[22] On the other hand, sparing of hypothenar, thenar, and finger extensor muscles sets sIBM besides a myotomal diseases, such as amyotrophic lateral sclerosis.[4] On physical examination, the presence of cramps, fasciculation, hyperreflexia, and/or neurogenic signs suggests neuromuscular disease.[22] Finally, individuals with sIBM usually do not respond to glucocorticoid therapies.[2],[22]

In hereditary inclusion body myopathies, there is sparing of the quadriceps muscles, even in the progressive disease phases. Weakness and atrophy of the foot extensors show as reduced foot dorsiflexion at an early phase of the disease, presenting as walk complications. Afterward, girdle and axial muscles convert more complicated.

Therefore, when considering differential diagnoses, multidisciplinary discussion with an expert in neuromuscular disease is crucial for the correct diagnosis and treatment.

A suggested diagnostic chart built on clinical outcomes is presented in [Figure 4].
Figure 4: Suggested diagnostic approach to muscle weakness in the Rheumatology setting. *Present in physical examination. If relevant family history is present, consider first an inherited disease. Metabolic myopathies include: glycogenosis, mitochondrial, and lipid storage myopathies.Systemic connective tissues diseases.Depending on the context, relevant different diagnosis may include muscular dystrophies, motor neuron disease, infectious myopathies, and toxic myopathies

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Prognosis

The natural progression of the sIBM consists of slowly advanced weakness of the lower and upper limbs, with a yearly decline of 10% for pinch strength and of 4% for knee extension.[57] Younger age at diagnosis may be related to a rapid deterioration of grip strength, while an older age at diagnosis may be related to a rapid deterioration of knee extension strength.[57]

Most patients may experience walking difficulties and up to a half will be bound to a wheelchair as the disease progress. Dysphagia is also a common complication of the disease, contributing to cachexia and muscle wasting.[36],[40]

Mortality in sIBM is a matter of controversy. A population-based study showed an increased mortality risk for patients with sIBM, while two cohort studies did not show differences in life expectancy.[7],[36],[40] Dysphagia and aspiration pneumonia contribute to premature death.[36]

Financial support and sponsorship

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) #303379/2018-9 and Faculdade de Medicina da USP - SP to SKS.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Greenberg SA. Inclusion body myositis. Contin Lifelong Learn Neurol 2016;22:1871-88.  Back to cited text no. 1
    
2.
Greenberg SA. Inclusion body myositis: Clinical features and pathogenesis. Nat Rev Rheumatol 2019;15:257-72.  Back to cited text no. 2
    
3.
Callan A, Capkun G, Vasanthaprasad V, Freitas R, Needham M. A systematic review and meta-analysis of prevalence studies of sporadic inclusion body myositis. J Neuromuscul Dis 2017;4:127-37.  Back to cited text no. 3
    
4.
Dimachkie MM, Barohn RJ. Inclusion body myositis. Neurol Clin 2014;32:629-46.  Back to cited text no. 4
    
5.
Keshishian A, Greenberg SA, Agashivala N, Baser O, Johnson K. Health care costs and comorbidities for patients with inclusion body myositis. Curr Med Res Opin 2018;34:1679-85.  Back to cited text no. 5
    
6.
Dobloug GC, Antal EA, Sveberg L, Garen T, Bitter H, Stiarne J, et al. High prevalence of inclusion body myositis in Norway; a population-based clinical epidemiology study. Eur J Neurol 2015;22:672-e41.  Back to cited text no. 6
    
7.
Dobloug GC, Garen T, Brunborg C, Gran JT, Molberg Ø. Survival and cancer risk in an unselected and complete Norwegian idiopathic inflammatory myopathy cohort. Semin Arthritis Rheum 2015;45:301-8.  Back to cited text no. 7
    
8.
Limaye V, Luke C, Tucker G, Hill C, Lester S, Blumbergs P, et al. The incidence and associations of malignancy in a large cohort of patients with biopsy-determined idiopathic inflammatory myositis. Rheumatol Int 2013;33:965-71.  Back to cited text no. 8
    
9.
de Camargo LV, de Carvalho MS, Shinjo SK, de Oliveira AS, Zanoteli E. Clinical, Histological, and Immunohistochemical Findings in Inclusion Body Myositis. Biomed Res Int 2018;2018:5069042.  Back to cited text no. 9
    
10.
Alverne AR, Marie SK, Levy-Neto M, de Souza FH, de Carvalho MS, Shinjo SK. Inclusion body myositis: Series of 30 cases from a Brazilian tertiary center. Acta Reumatol Port 2013;38:179-85.  Back to cited text no. 10
    
11.
Danckworth F, Karabul N, Posa A, Hanisch F. Risk factors for osteoporosis, falls and fractures in hereditary myopathies and sporadic inclusion body myositis – A cross sectional survey. Mol Genet Metab Rep 2014;1:85-97.  Back to cited text no. 11
    
12.
Salajegheh M, Lam T, Greenberg SA. Autoantibodies against a 43 KDa muscle protein in inclusion body myositis. PLoS One 2011;6:e20266.  Back to cited text no. 12
    
13.
Askanas V, Engel WK, Nogalska A. Sporadic inclusion body myositis: A degenerative muscle disease associated with aging, impaired muscle protein homeostasis and abnormal mitophagy. Biochim Biophys Acta Mol Basis Dis 2015;1852:633-43.  Back to cited text no. 13
    
14.
Huntley ML, Gao J, Termsarasab P, Wang L, Zeng S, Thammongkolchai T, et al. Association between TDP-43 and mitochondria in inclusion body myositis. Lab Investig 2019;99:1041-8.  Back to cited text no. 14
    
15.
Joshi PR, Vetterke M, Hauburger A, Tacik P, Stoltenburg G, Hanisch F. Functional relevance of mitochondrial abnormalities in sporadic inclusion body myositis. J Clin Neurosci 2014;21:1959-63.  Back to cited text no. 15
    
16.
Paltiel AD, Ingvarsson E, Lee DK, Leff RL, Nowak RJ, Petschke KD, et al. Demographic and clinical features of inclusion body myositis in North America. Muscle Nerve 2015;52:527-33.  Back to cited text no. 16
    
17.
Oh TH, Brumfield KA, Hoskin TL, Stolp KA, Murray JA, Bassford JR. Dysphagia in inflammatory myopathy: Clinical characteristics, treatment strategies, and outcome in 62 patients. Mayo Clin Proc 2007;82:441-7.  Back to cited text no. 17
    
18.
Cox FM, Verschuuren JJ, Verbist BM, Niks EH, Wintzen AR, Badrising UA. Detecting dysphagia in inclusion body myositis. J Neurol 2009;256:2009-13.  Back to cited text no. 18
    
19.
Mohannak N, Pattison G, Hird K, Needham M. Dysphagia in patients with sporadic inclusion body myositis: Management challenges. Int J Gen Med 2019;12:465-74.  Back to cited text no. 19
    
20.
Allenbach Y, Benveniste O, Decostre V, Canal A, Eymard B, Herson S, et al. Quadriceps strength is a sensitive marker of disease progression in sporadic inclusion body myositis. Neuromuscul Disord 2012;22:980-6.  Back to cited text no. 20
    
21.
Badrising UA, Maat-Schieman MLC, Van Houwelingen JC, Van Doorn PA, Van Duinem SG, Van Engelen BG, et al. Inclusion body myositis: Clinical features and clinical course of the disease in 64 patients. J Neurol 2005;252:1448-54.  Back to cited text no. 21
    
22.
Catalán M, Selva-O'Callaghan A, Grau JM. Diagnosis and classification of sporadic inclusion body myositis (sIBM). Autoimmun Rev 2014;13:363-6.  Back to cited text no. 22
    
23.
Griggs RC, Askanas V, DiMauro S, Engel A, Karpati G, Mendell JR, et al. Inclusion body myositis and myopathies. Ann Neurol 1995;38:705-13.  Back to cited text no. 23
    
24.
Rose MR. 188th ENMC International Workshop: Inclusion nody myositis, 2-4 December 2011, Naarden, The Netherlands. Neuromuscul Disord 2013;23:1044-55.  Back to cited text no. 24
    
25.
Lloyd TE, Mammen AL, Amato AA, Weiss MD, Needham M, Greenberg SA. Evaluation and construction of diagnostic criteria for inclusion body myositis. Neurology 2014;83:426-33.  Back to cited text no. 25
    
26.
Naddaf E, Barohn RJ, Dimachkie MM. Inclusion body myositis: Update on pathogenesis and treatment. Neurotherapeutics 2018;15:995-1005.  Back to cited text no. 26
    
27.
Milisenda JC, García AM, Jou C, Pinal-Fernandez I, O'Callaghan AS, Grau JM. Sporadic inclusion body myositis: Diagnostic value of p62 immunostaining. Med Clin (Barc) 2019;153:437-40.  Back to cited text no. 27
    
28.
Nojszewska M, Gawel M, Kierdaszuk B, Sierdzinski J, Szmidt-Salkwska E, Seroka A, et al. Electromyographic findings in sporadic inclusion body myositis. J Electromyogr Kinesiol 2018;39:114-9.  Back to cited text no. 28
    
29.
Cox FM, Reijnierse M, van Rijswijk CS, Wintzen AR, Verschuuren JJ, Badrising UA. Magnetic resonance imaging of skeletal muscles in sporadic inclusion body myositis. Rheumatology (Oxford) 2011;50:1153-61.  Back to cited text no. 29
    
30.
Tasca G, Monforte M, De Fino C, Kley RA, Ricci E, Mirabella M. Magnetic resonance imaging pattern recognition in sporadic inclusion body myositis. Muscle Nerve 2015;52:956-62.  Back to cited text no. 30
    
31.
Dion E, Cherin P, Payan C, Fournet JC, Papo T, Maisonobe T, et al. Magnetic resonance imaging criteria for distinguishing between inclusion body myositis and polymyositis. J Rheumatol 2002;29:1897-906.  Back to cited text no. 31
    
32.
Felice KJ, Whitaker CH, Wu Q, Larose DT, Shen G, Metzger AL, et al. Sensitivity and clinical utility of the anti-cytosolic 5′-nucleotidase 1A (cN1A) antibody test in sporadic inclusion body myositis: Report of 40 patients from a single neuromuscular center. Neuromuscul Disord 2018;28:660-4.  Back to cited text no. 32
    
33.
Herbert MK, Stammen-Vogelzangs J, Verbeek MM, Rietveld A, Lundberg IE, Chinoy H, et al. Disease specificity of autoantibodies to cytosolic 5'-nucleotidase 1A in sporadic inclusion body myositis versus known autoimmune diseases. Ann Rheum Dis 2016;75:696-701.  Back to cited text no. 33
    
34.
Yeker RM, Pinal-Fernandez I, Kishi T, Pak K, Targoff IN, Miller FW, et al. Anti-NT5C1A autoantibodies are associated with more severe disease in patients with juvenile myositis. Ann Rheum Dis 2018;77:714-9.  Back to cited text no. 34
    
35.
Chahin N, Engel AG. Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM. Neurology 2008;70:418-24.  Back to cited text no. 35
    
36.
Cox FM, Titulaer MJ, Sont JK, Wintzen AR, Verschuuren JJ, Badrising UA. A 12-year follow-up in sporadic inclusion body myositis: An end stage with major disabilities. Brain 2011;134:3167-75.  Back to cited text no. 36
    
37.
Muscle Study Group. Randomized pilot trial of high-dose INF-1a in patients with inclusion body myositis. Neurology 2004;63:718-20.  Back to cited text no. 37
    
38.
Badrising UA, Maat-Schieman ML, Ferrari MD, Zwinderman AH, Wessels JA, Breedveld FC, et al. Comparison of weakness progression in inclusion body myositis during treatment with methotrexate or placebo. Ann Neurol 2002;51:369-72.  Back to cited text no. 38
    
39.
Zschüntzsch J, Voss J, Creus K, Sehmisch S, Raju R, Dalakas MC, et al. Provision of an explanation for the inefficacy of immunotherapy in sporadic inclusion body myositis: Quantitative assessment of inflammation and β-amyloid in the muscle. Arthritis Rheum 2012;64:4094-103.  Back to cited text no. 39
    
40.
Benveniste O, Guiguet M, Freebody J, Dubourg O, Squier W, Maisonobe T, et al. Long-term observational study of sporadic inclusion body myositis. Brain 2011;134:3176-84.  Back to cited text no. 40
    
41.
Cherin P, Delain JC, De Jaeger C, Crave JC. Subcutaneous immunoglobulin use in inclusion body myositis: A review of 6 cases. Case Rep Neurol 2015;7:227-32.  Back to cited text no. 41
    
42.
Rutkove SB, Parker RA, Nardin RA, Connolly CE, Felice KJ, Raynor EM. A pilot randomized trial of oxandrolone in inclusion body myositis. Neurology 2002;58:1081-7.  Back to cited text no. 42
    
43.
Sancricca C, Mora M, Ricci E, Tonali PA, Mantegazza R, Mirabella M. Pilot trial of simvastatin in the treatment of sporadic inclusion body myositis. Neurol Sci 2011;32:841-7.  Back to cited text no. 43
    
44.
Benveniste O, Hogrel JY, Annoussamy M, Bachasson D, Rigolet A, Servais L, et al. Rapamycin vs. placebo for the treatment of inclusion body myositis: Improvement of the 6 min walking distance, a functional scale, the FVC and muscle quantitative MRI [abstract]. Arthritis Rheumatol 2017;69 (Suppl 10):5L.  Back to cited text no. 44
    
45.
Barohn RJ, Herbelin L, Kissel JT, King W, McVey AL, Saperstein DS, et al. Pilot trial of etanercept in the treatment of inclusion body myositis. Neurology 2006;66:S123-4.  Back to cited text no. 45
    
46.
Kosmidis ML, Alexopoulos H, Tzioufas AG, Dalakas MC. The effect of anakinra, an IL1 receptor antagonist, in patients with sporadic inclusion body myositis (sIBM): A small pilot study. J Neurol Sci 2013;334:123-5.  Back to cited text no. 46
    
47.
Dalakas MC, Rakocevic G, Schmidt J, Salajegheh M, McElroy B, Harris-Love MO, et al. Effect of Alemtuzumab (CAMPATH 1-H) in patients with inclusion body myositis. Brain 2009;132:1536-44.  Back to cited text no. 47
    
48.
Schmidt K, Kleinschnitz K, Rakocevic G, Dalakas MC, Schmidt J. Molecular treatment effects of alemtuzumab in skeletal muscles of patients with IBM. BMC Neurol 2016;16:48.  Back to cited text no. 48
    
49.
Hanna MG, Badrising UA, Benveniste O, Lloyd TE, Needham M, Chinoy H, et al. Safety and efficacy of intravenous bimagrumab in inclusion body myositis (RESILIENT): A randomised, double-blind, placebo-controlled phase 2b trial. Lancet Neurol 2019;18:834-44.  Back to cited text no. 49
    
50.
Kosmidis ML, Pikazis D, Vlachoyiannopoulos P, Tzioufas AG, Dalakas MC. Trial of canakinumab, an IL-1β receptor antagonist, in patients with inclusion body myositis. Neurol Neuroimmunol Neuroinflamm 2019;6:e581.  Back to cited text no. 50
    
51.
Arnardottir S, Alexanderson H, Lundberg IE, Borg K. Sporadic inclusion body myositis: Pilot study on the effects of a home exercise program on muscle function, histopathology and inflammatory reaction. J Rehabil Med 2003;35:31-5.  Back to cited text no. 51
    
52.
Johnson LG, Sci Hons B, Edwards DJ, Walters S, Thickbroom GW, Mastaglia FL, et al. The effectiveness of an individualized, home-based functional exercise program for patients with sporadic inclusion body myositis. J Clin Neuromuscul Dis 2007;8:187-94.  Back to cited text no. 52
    
53.
Johnson LG, Collier KE, Edwards DJ, Walters S, Thickbroom GW, Mastaglia FL. Improvement in aerobic capacity after an exercise program in sporadic inclusion body myositis. J Clin Neuromuscul Dis 2009;10:178-84.  Back to cited text no. 53
    
54.
Wallace A, Pietrusz A, Dewar E, Dudziec M, Jones K, Hennis P, et al. Community exercise is feasible for neuromuscular diseases and can improve aerobic capacity. Neurology 2019;92:e1773-85.  Back to cited text no. 54
    
55.
Jørgensen AN, Aagaard P, Frandsen U, Boyle E, Diederichsen LP. Blood-flow restricted resistance training in patients with sporadic inclusion body myositis: A randomized controlled trial. Scand J Rheumatol 2018;47:400-9.  Back to cited text no. 55
    
56.
De Paepe B. Sporadic inclusion body myositis: An acquired mitochondrial disease with extras. Biomolecules 2019;9. pii: e15.  Back to cited text no. 56
    
57.
Oldroyd AGS, Lilleker JB, Williams J, Chinoy H, Miller JA. Long-term strength and functional status in inclusion body myositis and identification of trajectory subgroups. Muscle Nerve 2020. doi: 10.1002/mus.26859.  Back to cited text no. 57
    


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