Presentation
Understanding the varied scope of presentation of these diseases is essential to providing optimal patient care. [jhu.pure.elsevier.com]
It is frequently present at the onset of disease and it may even precede muscular manifestations of IIM. [ncbi.nlm.nih.gov]
Entire Body System
- Weakness
BACKGROUND: The purpose of this case series was to quantify different strategies used to compensate in gait for hip muscle weakness. [ncbi.nlm.nih.gov]
People who have this type of disease experience severe muscle weakness that may make it difficult for them to stand or walk. [myphiladelphiadisabilitylawyer.com]
In addition to the proximal weakness in the group of inflammatory myopathy, 14/61 (22.95%) patients had distal weakness. Nearly 50% of patients [8/18] with PM had demonstrable distal weakness. [neurologyindia.com]
- Inflammation
Although rare, cases of focal/localized myositis in which polymyositis-like muscle inflammation is present in only one muscle or extremity have also been reported. [ncbi.nlm.nih.gov]
Diagnosis is confirmed by elevated serum muscle-derived enzymes, typical electromyography (EMG) findings, and inflammation on muscle biopsy. Corticosteroids are first-line therapy. [bestpractice.bmj.com]
Laboratory tests Muscle Biopsy Histologic confirmation of muscle inflammation is required in many, but not all, cases of inflammatory muscle disease. [what-when-how.com]
- Swelling
Associated features Pain, Fever, Swelling, Rash, Weight loss, Fatigue, Malignancy Laboratory Investigations CK, ESR, ANA, dsDNA, other serological markers as indicated Histopathology Fiber size variability, Hypertrophic fibers, Basophilic/Rounded fibers [neurologyindia.com]
The predominant manifestations at onset are increased dyspnoea, cervical or facial swelling, subcutaneous emphysema, cervical pain and cough [ 3, 140 ]. Pneumothorax coexists in 40% of cases [ 3 ]. [err.ersjournals.com]
- Pathologist
Tissue samples obtained with the closed-needle approach can provide sufficient diagnostic information for interpretation by the muscle pathologist. [what-when-how.com]
Speech pathologists can provide advice on preventing choking episodes and reducing the anxiety of an immanent aspiration for both patients and carers. [4] Epidemiology [ edit ] Every year between 2.18 and 7.7 people per million receive a diagnosis of [en.wikipedia.org]
Gastrointestinal
- Dysphagia
Four to six weeks after IVMP, dysphagia had resolved in 2 and improved in 3. [ncbi.nlm.nih.gov]
May be associated with dysphagia, fatigue, difficulties with breathing, and skin lesions. Diagnosis is confirmed by elevated serum muscle-derived enzymes, typical electromyography (EMG) findings, and inflammation on muscle biopsy. [bestpractice.bmj.com]
Quadriceps and hamstrings, adducters and abducters of hip) Distal weakness Dysphagia Weakness in the neck flexor group of muscles. [neurologyindia.com]
- Nausea
• Side Effects : Nausea, Loose stools, fever, and Liver toxicity 63. [slideshare.net]
Skin
- Erythema
The most common cutaneous lesions endorsed were periungual capillary loop changes (63%), Gottron's papules/sign (53%), heliotrope rash (49%) and malar/facial erythema (49%). [ncbi.nlm.nih.gov]
Hallmarks of the disease are myositis with necrosis, regeneration and perifascicular atrophy accompanied by a typical skin rash with heliotrope erythema, Gottron’s sign, Gottron’s papules and nail fold changes with splinter hemorrhage. [infona.pl]
Clinical Features DM : Dermatologic Manifestations Shawl Sign • Macular violaceous erythema over Nape,back and shoulders. • May show photosensitivity. 13. [slideshare.net]
The risk is maximal within the first year of follow-up and in Asian populations, and is higher in cases of severe dermatomyositis rash suggestive of intense vasculitis (cutaneous ulcers and periungual erythema), older age, male sex, higher C-reactive [err.ersjournals.com]
Musculoskeletal
- Muscle Weakness
BACKGROUND: The purpose of this case series was to quantify different strategies used to compensate in gait for hip muscle weakness. [ncbi.nlm.nih.gov]
Inclusion body myositis causes severe muscle weakness. Loss of coordination is experienced as the muscles are damaged. Small holes appear in the fiber of the muscles. [myphiladelphiadisabilitylawyer.com]
Discussion Myasthenia, or simply muscle weakness, is a common admitting diagnosis in the inpatient setting with a broad differential. [f1000research.com]
Definition Idiopathic inflammatory myopathies constitute a heterogeneous group of sub-acute, chronic, and, rarely, acute diseases of skeletal muscle that have in common the presence of moderate-to-severe proximal muscle weakness and inflammation on muscle [bestpractice.bmj.com]
- Arthritis
We have confirmed a strong association of arthritis with the presence of the anti-Jo1 antibody. [ncbi.nlm.nih.gov]
Arthritis in Idiopathic Inflammatory Myopathy: Clinical Features and Autoantibody Associations. [opus.bath.ac.uk]
Arthritis Rheum 1992, 35 :821–830. PubMed CrossRef Google Scholar 5. [link.springer.com]
- Proximal Muscle Weakness
[…] to 2 hospitals within our academic system in calendar year 2014 with acute/subacute onset of profound proximal muscle weakness and markedly elevated creatine kinase levels. [ncbi.nlm.nih.gov]
Our patient met three out of the four criteria for polymyositis per the American College of Rheumatology including symmetric proximal muscle weakness, elevation of skeletal muscle enzymes, and an abnormal EMG showing polyphasic, short, small motor unit [f1000research.com]
- Muscular Atrophy
Muscular atrophy occurs with preservation of tendon reflexes, flexor plantar response and normal sensation. Muscles may be tender on palpation and may have a nodular grainy feel. Investigations Creatine kinase can be up to 50 times normal. [patient.info]
Neurologic
- Confusion
Myalgia syndromes, such as polymyalgia rheu-matica, in which stiffness is a predominant complaint, may confuse the diagnosis in some patients. [what-when-how.com]
Differentiation between acute PM and LGMD does not pose a difficulty, but when patients present for the first time with duration of illness longer than one year, there is a strong likelihood of confusion. [neurologyindia.com]
[…] incidence ratios (SIR) of 2.4 (1.2-4.9), but a more recent study failed to show a significant association with SIR of 1.4 (0.8-2.3). [26] Data on ADM is even more scarce, as, in part, it is a relatively uncommon entity and its definition is still somewhat confusing [najms.org]
Workup
Bauer said the Swedish study is unlikely to alter U.S. practice and in fact supports the current approach, which involves a thorough malignancy and pulmonary workup at time of diagnosis. [medpagetoday.com]
We recommend that a muscle biopsy is performed in the workup of a patient suspected of having IIM whenever possible as there are reported cases of non-inflammatory myopathies that can mimic IIM. 19 One factor contributing to the low rate of muscle biopsy [nzma.org.nz]
NAM has been associated with malignancy and statin use. [6] See Etiology, Presentation, and Workup. Polymyositis and dermatomyositis have many shared clinical features. Both present as symmetrical muscle weakness that develops over weeks to months. [emedicine.medscape.com]
Treatment
Patients with inflammatory myositis of recent onset who had not received treatment were evaluated for associated myocarditis by magnetic resonance imaging (MRI) and reinvestigated after treatment with high dose corticosteroids and immunosuppressors. [ncbi.nlm.nih.gov]
Prognosis
SUMMARY: Myositis-specific autoantibodies are useful markers for clinical diagnosis, classification and predicting prognosis of idiopathic inflammatory myopathy. [ncbi.nlm.nih.gov]
Etiology
DRB1*0301 was a common genetic risk factor for familial and sporadic IIM, but contributed less to the genetic risk of familial IIM (etiologic fraction 0.35 versus 0.51 in sporadic IIM). [ncbi.nlm.nih.gov]
Epidemiology
Author information 1 Department of Epidemiology, Flinders University, Adelaide, South Australia, Australia. 2 Clinical Epidemiology Unit, Flinders Medical Centre, Adelaide, South Australia, Australia. 3 Rheumatology Department, Royal Adelaide Hospital [ncbi.nlm.nih.gov]
Clinical and epidemiological research Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study Gerd Cecilie Dobloug 1, John Svensson 2, Ingrid E Lundberg 2, Marie Holmqvist 2, 3 1 Department of Rheumatology [ard.bmj.com]
Moghadam-Kia, Siamak (2015) Epidemiology of idiopathic inflammatory myopathies and statin myopathy. Master Essay, University of Pittsburgh. Abstract Myopathies are generally divided into acquired and inherited forms. [d-scholarship.pitt.edu]
However, several epidemiologic features make this a particularly difficult diagnosis in this patient. Foremost, polymyositis is a rare disease with incidence rates occurring in about 1 per 100,000 people annually 4 – 7. [f1000research.com]
Pathophysiology
This review examines recent progress in myositis-specific autoantibodies, particularly in their clinical significance and pathophysiological roles. [ncbi.nlm.nih.gov]
[…] cytoxic mechanisms in polymyositis, to a complement-mediated vasculopathy of the small vessels in muscle tissues in dermatomyositis, to a primarily macrophage driven degeneration in immune-mediated necrotizing myopathy have all been ascribed as the pathophysiology [f1000research.com]
However, in the recent years, several new studies were published allowing us to better understand the clinical characteristics and pathophysiology of cancer-associated IIMs. [najms.org]
Further studies are needed to better understand the pathophysiology of ILD related to dermatomyositis/polymyositis, and to investigate the effectiveness and tolerance of targeted therapies in randomised controlled trials. [err.ersjournals.com]
Prevention
Exercise training could be one way to prevent or delay the negative effects of the disease and the impairments seen in patients with an IIM. The objective was to examine whether exercise training is safe and effective in patients with an IIM. [ncbi.nlm.nih.gov]
Collaborative Meta-Analysis of Randomised Trials of Antiplatelet Therapy for Prevention of Death, Myocardial Infarction, and Stroke in High Risk Patients. [books.google.es]
Because patients taking prednisone are at risk for osteoporosis, they should receive proper treatment to prevent it. [rheumatology.org]
During the phase of active inflammatory disease, passive range-of-motion exercises are necessary to prevent contractures. [what-when-how.com]