Inclusion body myositis — a case based clinicopathological update

Open Medicine ◽  
2014 ◽  
Vol 9 (1) ◽  
pp. 80-85
Author(s):  
Levente Bodoki ◽  
Melinda Vincze ◽  
Zoltán Griger ◽  
Tamás Csonka ◽  
Balázs Murnyák ◽  
...  
Keyword(s):  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Treatment Options ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Older Patient ◽  
Close Collaboration ◽  
Systemic Autoimmune Diseases ◽  
Case Based

AbstractInclusion body myositis is a slowly progressive myopathy affecting predominantly the middle-aged and older patient population. It is a major form of the idiopathic inflammatory myopathies which are chronic systemic autoimmune diseases characterized by symmetrical proximal muscle weakness. Unfortunately, there is no effective therapy yet; however, the early diagnosis is essential to provide treatment options which may significantly slow the progression of the disease. In our case-based clinicopathological study the importance of the close collaboration between the clinician and the neuropathologist is emphasised.

Idiopathic inflammatory myopathies: polymyositis and dermatomyositis

2011 ◽  
pp. 385-404
Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq
Keyword(s):  
Inclusion Body ◽  
Clinical Features ◽  
Muscle Weakness ◽  
Inclusion Body Myositis ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Breakdown

Epidemiology and diagnosis 386 Clinical features of polymyositis and dermatomyositis 388 Investigation of polymyositis and dermatomyositis 392 Autoantibodies in myositis 394 Treatment of polymyositis and dermatomyositis 398 Inclusion-body myositis 401 Polymyositis and dermatomyositis in children 402 • The idiopathic inflammatory myopathies are characterized by proximal muscle weakness and evidence of autoimmune-mediated muscle breakdown. These disorders include:...

Immunopathogenesis of Inflammatory Myopathies

2019 ◽  
pp. 177-192
Author(s):  
Marinos C. Dalakas
Keyword(s):  
Inclusion Body ◽  
Clinical Features ◽  
Muscle Tissue ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Treatment Options ◽  
Inflammatory Myopathies ◽  
Antisynthetase Syndrome ◽  
Autoimmune Myositis ◽  
Overlap Myositis

This chapter looks at inflammatory myopathies (IM), which constitute a heterogeneous group of acquired myopathies that have in common the presence of inflammation in the muscle tissue. The chapter looks at specific clinical features such as dermatomyositis, polymyositis, necrotizing autoimmune myositis, antisynthetase syndrome-overlap myositis, and inclusion body myositis. It then considers diagnosis, which can be made through muscle biopsy and the detection of autoantibodies. Finally, it looks at treatment options.

Necrotizing autoimmune myopathy

2012 ◽  
Vol 153 (38) ◽  
pp. 1502-1507 ◽  
Author(s):  
Levente Bodoki ◽  
Melinda Vincze ◽  
Tibor Hortobágyi ◽  
Zoltán Griger ◽  
Karolina Cseri ◽  
...  
Keyword(s):  
Muscle Weakness ◽  
Rare Entity ◽  
Separate Entity ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Systemic Autoimmune Diseases ◽  
Necrotizing Autoimmune Myopathy ◽  
Autoimmune Myopathy ◽  
Very High

Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by symmetrical proximal muscle weakness. One of them is the subgroup of necrotizing autoimmune myopathy, which has recently been recognized as a separate entity. In addition to the typical symmetrical muscle weakness, it is characterized by very high creatine kinase levels, myopathic triad in the electromyography, and myocyte necrosis without significant inflammation. The paper aims to review this rare entity, which has to be diagnosed and treated quickly in every case. Orv. Hetil., 2012, 153, 1502–1507.

scholarly journals Amyloid myopathy: a diagnostic challenge

2009 ◽  
Vol 1 (1) ◽  
pp. 7 ◽  
Author(s):  
Heli Tuomaala ◽  
Mikko Kärppä ◽  
Hannu Tuominen ◽  
Anne M. Remes
Keyword(s):  
Inclusion Body ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Initial Symptom ◽  
Inflammatory Myopathies ◽  
Proximal Muscle ◽  
Diagnostic Challenge ◽  
Amyloid Accumulation ◽  
Rare Manifestation ◽  
Congo Red Staining

Amyloid myopathy (AM) is a rare manifestation of primary systemic amyloidosis (AL). Like inflammatory myopathies, it presents with proximal muscle weakness and an increased creatine kinase level. We describe a case of AL with severe, rapidly progressive myopathy as the initial symptom. The clinical manifestation and muscle biopsy were suggestive of inclusion body myositis. AM was not suspected until amyloidosis was seen in the gastric mucosal biopsy. The muscle biopsy was then re-examined more specifically, and Congo red staining eventually showed vascular and interstitial amyloid accumulation, which led to a diagnosis of AM. The present case illustrates the fact that the clinical picture of AM can mimic that of inclusion body myositis.

scholarly journals In Pursuit of an Effective Treatment: the Past, Present and Future of Clinical Trials in Inclusion Body Myositis

2021 ◽  
Author(s):  
A. M. Snedden ◽  
J. B. Lilleker ◽  
H. Chinoy
Keyword(s):  
Clinical Practice ◽  
Effective Treatment ◽  
Inclusion Body ◽  
Therapeutic Effect ◽  
Outcome Measures ◽  
Inclusion Body Myositis ◽  
Cytotoxic T Cells ◽  
Treatment Options ◽  
Trial Methodology ◽  
Short Treatment

Abstract Purpose of review No clinical trial in sporadic inclusion body myositis (IBM) thus far has shown a clear and sustained therapeutic effect. We review previous trial methodology, explore why results have not translated into clinical practice, and suggest improvements for future IBM trials. Recent findings Early trials primarily assessed immunosuppressive medications, with no significant clinical responses observed. Many of these studies had methodological issues, including small participant numbers, nonspecific diagnostic criteria, short treatment and/or assessment periods and insensitive outcome measures. Most recent IBM trials have instead focused on nonimmunosuppressive therapies, but there is mounting evidence supporting a primary autoimmune aetiology, including the discovery of immunosuppression-resistant clones of cytotoxic T cells and anti-CN-1A autoantibodies which could potentially be used to stratify patients into different cohorts. The latest trials have had mixed results. For example, bimagrumab, a myostatin blocker, did not affect the 6-min timed walk distance, whereas sirolimus, a promotor of autophagy, did. Larger studies are planned to evaluate the efficacy of sirolimus and arimoclomol. Summary Thus far, no treatment for IBM has demonstrated a definite therapeutic effect, and effective treatment options in clinical practice are lacking. Trial design and ineffective therapies are likely to have contributed to these failures. Identification of potential therapeutic targets should be followed by future studies using a stratified approach and sensitive and relevant outcome measures.

Histopathological features of the idiopathic inflammatory myopathies

2018 ◽  
Author(s):  
Marianne de Visser and Eleonora M.A. Aronica
Keyword(s):  
Inclusion Body ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Inflammatory Myopathy ◽  
Adult Patients ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Histopathological Features ◽  
Muscle Biopsies

In adult patients with presumed idipathic inflammatory myopathy (IIM) without a characteristic and diagnostic dermatomyositis rash, muscle biopsy is mandatory to confirm the IIM diagnosis and to exclude a myopathy which would not respond to glucocorticoids or other immunosuppressants, including inclusion body myositis. This chapter discusses when, where, and how to undertake muscle biopsies, when to repeat them, how to interpret their results, and how these relate to IIM subtypes and disease processes.

Idiopathic Inflammatory Myopathies

2015 ◽  
Author(s):  
Frederick W Miller ◽  
Adam Schiffenbauer
Keyword(s):  
Differential Diagnosis ◽  
Connective Tissue ◽  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Clinical Laboratory ◽  
Connective Tissue Diseases ◽  
Muscle Pathology ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Pathologic Evaluation

The idiopathic inflammatory myopathies (IIMs), also known as myositis syndromes, are a collection of heterogeneous disorders that share the common feature of chronic muscle inflammation of unknown cause. These disorders may occur in adults or children and are sometimes associated with other connective tissue disorders and a variety of cancers. A combined clinical, laboratory, and pathologic evaluation is needed to establish the diagnosis of these acquired systemic connective tissue diseases to rule out the many disorders that mimic IIMs. This module reviews the classification of IIMs, including polymyositis, dermatomyositis, inclusion body myositis, myositis associated with other connective tissue diseases and cancer, and antisynthetase syndrome. The epidemiology; etiology, genetics, and environmental factors; pathophysiology and pathogenesis; diagnosis; differential diagnosis; treatment; and prognosis of IIMs are discussed. Tables describe the criteria for polymyositis, dermatomyositis, and inclusion body myositis; well-characterized subgroups of the IIMs in adults and children; presentation of polymyositis; differential diagnosis of muscle weakness or pain; features that assist in discriminating IIMs from other myopathies; goals for managing IIMs; and key factors for achieving adequate corticosteroid response in IIMs. Figures demonstrate skin findings in IIMs, muscle pathology of IIMs, magnetic resonance imaging of three patients with different IIMs, and treatment approaches to the management of myositis patients. This review contains 4 highly rendered figures, 8 tables, and 80 references.

Acquired Muscle Disorders

2021 ◽  
pp. 779-784
Author(s):  
Teerin Liewluck ◽  
Margherita Milone
Keyword(s):  
Skeletal Muscle ◽  
Inclusion Body Myositis ◽  
Inflammatory Diseases ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Overlap Syndrome ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Muscle Disorders ◽  
Diagnostic Pathways

Muscle diseases consist of various disorders that primarily affect skeletal muscle, but they can also affect cardiac or smooth muscle. The disorders may be inherited or acquired. The pathophysiology, clinical manifestations, diagnostic pathways, and treatment options will vary for each disorder. This chapter reviews common acquired muscle disorders. Inflammatory myopathies are characterized by muscle weakness and inflammatory reaction in muscle, and often, but not always, are accompanied by elevated creatine kinase values. They can occur with infections, systemic inflammatory diseases, or they may be idiopathic. On the basis of clinical, histologic, and immunopathologic criteria, idiopathic inflammatory myopathies can be distinguished as dermatomyositis, polymyositis, or inclusion body myositis. When dermatomyositis or polymyositis occurs in association with a connective tissue disease, the disorder is called overlap syndrome.

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