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]
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]
Skin
- Papule
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 Gottron Papules • Violaceous flat topped papules and plaques over dorsal aspect of interphalengeal and MCP joints • Pathognomic of DM, seen in > 80% patients with DM 11. [slideshare.net]
If heliotrope (purple) rash or Gottron's papules are also present, then the diagnosis is DM. [en.wikipedia.org]
Inverse Gottron's papules: an unusual cutaneous manifestation of juvenile dermatomyositis. Pediatr Dermatol 2012; 29 (5) 641-644 6 Schmeling H, Stephens S, Goia C, et al. [thieme-connect.com]
Eyes
- Periorbital Edema
In the present study, this group was relatively homogenous, patients presenting with skin rash, or periorbital edema, myalgia and fatigue. [neurologyindia.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]
- 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]
- Leg Weakness
(distal arm and proximal leg weakness), in inclusion body myositis (IBM). 1,2 Common features in IBM, dermatomyositis, polymyositis, and necrotizing autoimmune myopathy, frequently include serum creatine kinase (CK) elevation and myopathy with muscle [neurology.org]
Neurologic
- Asthenia
Clinical presentations Usual presentation The signs and symptoms related to ILD in IIM do not differ from idiopathic ILD, and include dyspnoea on exertion, cough, decreased exercise tolerance, digital clubbing and asthenia. [err.ersjournals.com]
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]
Other Pathologies
- Lymphocytic Infiltrate
Necrotizing Myopathy Scattered necrotic myofibres with myophagocytosis in the paucity of T-lymphocytic infiltration. 51. [slideshare.net]
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]