Idiopathic Inflammatory Myopathies

2015 ◽  
Author(s):  
Frederick W Miller
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.

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.

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.

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.

Inflammatory myopathies

2020 ◽  
pp. 4537-4546
Author(s):  
Ingrid E. Lundberg ◽  
Hector Chinoy ◽  
Robert Cooper
Keyword(s):  
Gastrointestinal Tract ◽  
Connective Tissue ◽  
Muscle Fibre ◽  
Inclusion Body Myositis ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Heterogenous Group ◽  
Muscle Biopsies

The idiopathic inflammatory myopathies are a heterogenous group of disorders characterized by muscle weakness, inflammation in muscle tissue, and with frequent extramuscular involvement. Autoantibodies are common, supporting the notion of these disorders being autoimmune. Typically, inflammatory cell infiltrates are found in muscle biopsies. Other organs are frequently involved such as skin, lungs, joints, gastrointestinal tract, and the heart. These heterogenous disorders can be subclassified based on clinical and histopathological features, or by autoantibody specificities. The idiopathic inflammatory myopathies have traditionally comprised polymyositis (PM), dermatomyositis (DM), juvenile DM, PM/DM overlapping with another connective tissue disease, and inclusion body myositis. More recently a subgroup with similar clinical features but with no or scarce inflammation and with pronounced muscle fibre necrosis has been identified and termed immune-mediated necrotizing myopathy.

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:...

scholarly journals Clinical types of lung disease in polymyositis and dermatomyositis

2021 ◽  
Vol 2 (4) ◽  
pp. 34-39
Author(s):  
Inna B. Bondarenko ◽  
◽  
Liubov A. Ponomareva ◽  
Elena N. Popova ◽  
◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inclusion Body ◽  
Lung Tissue ◽  
Inclusion Body Myositis ◽  
Rapid Development ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Immune Mediated ◽  
Observed Damage

The idiopathic inflammatory myopathies are a group of rare, heterogeneous connective tissue disorders characterized by skeletal muscle inflammation. The four main forms of idiopathic inflammatory myopathies are dermatomyositis, polymyositis, inclusion body myositis, and necrotizing immune-mediated myopathy. Each form of myositis, other than inclusion body myositis, can be associated with damage to many organs, including the lungs, heart, joints, and skin. The most often observed damage to the lung tissue with the development of interstitial lung disease, which occurs with or without myositis. The severity of the course varies from mild to severe, with rapid development of respiratory failure. Interstitial lung disease can be fatal in patients with myositis, therefore, it is necessary to assess the damage to the lung tissue in the early stages of the disease.

Idiopathic inflammatory myopathies including polymyositis and dermatomyositis

2018 ◽  
pp. 425-446
Author(s):  
Gavin Clunie ◽  
Nick Wilkinson ◽  
Elena Nikiphorou ◽  
Deepak R. Jadon
Keyword(s):  
Young People ◽  
Diagnostic Criteria ◽  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Juvenile Dermatomyositis ◽  
Heterogeneous Group ◽  
Therapeutic Options ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Clinical Presentations

This chapter explores the epidemiology and pathophysiology of idiopathic inflammatory myopathies. The chapter takes a focus on polymyositis and dermatomyositis and describes relevant diagnostic criteria, clinical presentations, investigations, and therapeutic options. Other inflammatory myopathies discussed include inclusion-body myositis and other (adult) inflammatory myopathies such as those induced by drugs. The chapter closes with a description of juvenile idiopathic inflammatory myopathies, a heterogeneous group of inflammatory muscle conditions affecting young people and consisting of juvenile dermatomyositis and amyopathic juvenile dermatomyositis among others.

scholarly journals The Correlation of Muscle Biopsy Scores with the Clinical Variables in Idiopathic Inflammatory Myopathies

2016 ◽  
Vol 10 (1) ◽  
pp. 141-149 ◽  
Author(s):  
Suparaporn Wangkaew ◽  
Songkiet Suwansirikul ◽  
Kantawut Aroonrungwichian ◽  
Nuntana Kasitanon ◽  
Worawit Louthrenoo
Keyword(s):  
Connective Tissue ◽  
Muscle Biopsy ◽  
Muscle Power ◽  
Demographic Data ◽  
Muscle Pathology ◽  
Idiopathic Inflammatory Myopathies ◽  
Muscle Degeneration ◽  
Inflammatory Myopathies ◽  
Muscle Biopsies ◽  
Modest Correlation

Objectives: To compare the muscle pathology findings among subgroups of idiopathic inflammatory myopathies (IIM) patients, and to determine the correlations of muscle biopsy scores with muscle power and creatine kinase (CK). Methods: The medical records of IIM patients consisting of the demographic data, clinical parameters and laboratory conducted were retrospectively reviewed. Their initial muscle biopsies were reviewed, and four domains were scored: inflammation, vascular, muscle, and connective tissue. Results: Ninety-five IIM patients (28 patients with idiopathic polymyositis (PM) 9 idiopathic dermatomyositis (DM), 5 DM associated with malignancy, and 53 PM/DM associated with connective tissue disease) with median (IQR: Q1, Q3) disease duration of 1.2 (0.5, 3.1) months were included. No significant differences in initial muscle pathology findings and muscle pathology score among the subgroups were found. Muscle degeneration and endomysial fibrosis scores were negatively correlated with muscle power (r=-0.23 and-0.24, respectively, p<0.05) and positively correlated with CK (r=0.27 and 0.39, respectively, p<0.01). No significant correlation was detected either inflammation or vasculitis scores with muscle power and CK levels. Conclusion: In this study, muscle biopsy cannot be used to differentiate among subgroups of IIM patients. In addition, we found only modest correlation of muscle biopsy scores with muscle power and CK. Further study is necessary to confirm our findings.

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