Myositis (Oxford Rheumatology Library)

2018 ◽  
Keyword(s):  
Risk Factors ◽  
Skeletal Muscle ◽  
Environmental Risk ◽  
Inclusion Body Myositis ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Histopathological Changes ◽  
Muscle Enzymes ◽  
Health Significance ◽  
Laboratory Investigations

Myositis, or the idiopathic inflammatory myopathies (IMM) are a group of rare autoimmune diseases of considerable health significance. Features include muscle weakness, raised skeletal muscle enzymes, and characteristic histopathological changes. IIM can be split into polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM). If diagnosed promptly PM and DM can be treated with immunosuppression, but IBM currently cannot be treated. Summarizing the current understanding of the epidemiology, genetic and environmental risk factors, and clinical features, this handbook gives practical strategies for laboratory investigations and treatment paradigms. Future strategies and ongoing trials are also discussed.

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.

scholarly journals FRI0248 PROGNOSTIC FACTORS FOR STEROID-FREE REMISSION IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: IMPORTANCE OF ANTHROPOMETRIC MEASUREMENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 708.1-708
Author(s):  
J. S. Lee ◽  
S. H. Nam ◽  
S. J. Choi ◽  
W. J. Seo ◽  
S. Hong ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Cox Regression ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Immunosuppressant Therapy ◽  
Muscle Enzymes ◽  
Cox Regression Analysis ◽  
Factors Associated ◽  
Early Use

Background:Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIM), but few studies have assessed prognostic factors for steroid-free remission in IIM.Objectives:We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes.Methods:Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Steroid-free remission was defined as at least three months of normalisation of muscle enzymes and no detectable clinical disease activity. The factors associated with steroid-free remission were evaluated by a Cox regression analysis.Results:Of the 106 IIM patients, 35 displayed steroid-free remission during follow-up periods. In the multivariable Cox regression analyses, immunosuppressants’ early use within one month after diagnosis [hazard ratio (HR) 6.21, 95% confidence interval (CI) 2.61–14.74, p < 0.001] and sex-specific height quartiles (second and third quartiles versus first quartile, HR 3.65, 95% CI 1.40–9.51, p = 0.008 and HR 2.88, 95% CI 1.13–7.32, p = 0.027, respectively) were positively associated with steroid-free remission. Polymyositis versus dermatomyositis (HR 0.21, 95% CI 0.09–0.53, p = 0.001), presence of dysphagia (HR 0.15, CI 0.05–0.50, p = 0.002) and highest versus lowest quartile of waist circumference (WC) (HR 0.24, 95% CI 0.07–0.85, p = 0.027) were negatively associated with steroid-free remission.Conclusion:The early initiation of immunosuppressant therapy, type of myositis and presence of dysphagia are strong predictors of steroid-free remission in IIM; moreover, height and WC measurements at baseline may provide additional important prognostic value.Disclosure of Interests:None declared

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.

Longterm Survival and Associated Risk Factors in Patients with Adult-onset Idiopathic Inflammatory Myopathies and Amyopathic Dermatomyositis: Experience in a Single Institute in Japan

2011 ◽  
Vol 38 (8) ◽  
pp. 1636-1643 ◽  
Author(s):  
YOSHIOKI YAMASAKI ◽  
HIDEHIRO YAMADA ◽  
MICHIKO OHKUBO ◽  
MASAOMI YAMASAKI ◽  
KOHEI AZUMA ◽  
...  
Keyword(s):  
Risk Factors ◽  
Therapeutic Strategy ◽  
Causes Of Death ◽  
Idiopathic Inflammatory Myopathies ◽  
Adult Onset ◽  
Inflammatory Myopathies ◽  
Skin Ulcers ◽  
Independent Risk Factors ◽  
Longterm Survival ◽  
Associated Risk Factors

Objective.To analyze clinical characteristics, survival, causes of death, and risk factors associated with mortality in patients with adult-onset idiopathic inflammatory myopathies (IIM) in Japan.Methods.We retrospectively investigated 197 patients diagnosed with adult-onset IIM at our hospital from 1984 to 2009 according to Bohan and Peter criteria for polymyositis (PM)/dermatomyositis (DM) and modified Sontheimer’s criteria for clinically amyopathic DM (ADM).Results.Survival in the whole group at 1, 5, and 10 years was 85%, 75%, and 67%, respectively. Mortality in cancer-associated myositis was the worst (25% at 5 yrs), followed by clinically ADM (61% at 5 yrs) and primary DM (77% at 5 yrs). Primary DM had significantly low survival compared to primary PM (91% at 5 yrs; p = 0.0427). Among the 53 patients who died were 6 patients with ADM (11%) and 20 patients with primary DM (38%). Interstitial lung disease (ILD) was the main cause of death in clinically ADM (71%) and primary DM (60%), most of which occurred within the first few months. Fewer patients died in primary PM (9%) and overlap myositis (13%). Independent risk factors for death were older age (HR 1.031; 95% CI 1.009–1.053) and skin ulcers (HR 3.018; 95% CI 1.340–6.796) in the whole group and ILD with mild serum creatine kinase levels (< 500 IU/l; HR 3.537; 95% CI 1.260–9.928) in primary DM.Conclusion.Survival of clinically ADM and primary DM was low, mainly due to fatal ILD, compared to primary PM. Establishing therapeutic strategy for ILD may improve the survival in our patient population.

scholarly journals A study on demographic pattern of idiopathic inflammatory myopathies in a tertiary care hospital

2019 ◽  
Vol 6 (2) ◽  
pp. 236
Author(s):  
Srujith C. H. ◽  
Kavitha Mohanasundaram ◽  
Jagadeesan M. ◽  
Halleys Kumar E. ◽  
Kannan R. ◽  
...  
Keyword(s):  
Tertiary Care ◽  
Lactic Dehydrogenase ◽  
Idiopathic Inflammatory Myopathies ◽  
Care Hospital ◽  
Inflammatory Myopathies ◽  
Muscle Enzymes ◽  
Cross Sectional ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic systemic autoimmune diseases characterized by proximal muscle weakness and elevated muscle enzymes. Aim and Objective was to analyze the demographic profile of patients with idiopathic inflammatory myopathies (IIM).Methods: This was a cross sectional observational study conducted over a period of two years (2016-2018). After obtaining institutional ethical committee clearance, informed consent from patients. 16 patients who fulfilled the criteria were included in the study. The demographic and the clinical data were analysed.Results: The mean age was 47.3±11.2 years. The study showed female predominance. ANA was positive in 11(68.7%) patients. Among the 16 patients, 5 (31.25%) had polymyositis and 11 (68.7%) had dermatomyositis. The median enzymes levels were creatinine kinase 1134 U/L, lactic dehydrogenase 477U/L, ALT (alanine aminotransferase) 154 IU/L, AST (aspartate aminotransferase) 236IU/L. Raynaud's phenomenon was seen in 37.5%. In our study, 31.25% had hypothyroidism and 6.25% had diabetic mellitus. On follow up 37.5% developed interstitial lung disease (ILD) and 18.75% were found to have malignancy.Conclusions: Steroids and immunomodulators are the mainstay of treatment in patients with idiopathic inflammatory myositis. All our patients improved with steroids. It is important to evaluate these patients during early stages and follow up to prevent complications.

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.

Sign in / Sign up

Export Citation Format

Share Document