A 62-year-old woman presented to the emergency department with malaise, lethargy and proximal muscle weakness. She had a similar presentation in childhood. [ncbi.nlm.nih.gov]

Some patients can present calcinosis3 and the gastrointestinal tract, kidney and myocardium can be involve4,5,6. [scielo.br]

The juvenile IIM also may be classified based on the presence of certain autoantibodies that are present specifically in myositis patients (myositis-specific autoantibodies), and others that are present in myositis patients as well as patients with other [obgynkey.com]

Part IV (Overview) summarizes the issues that may not have been clear and highlights differing and unsettled views or present available data. [books.google.de]

• Fever

  The disease often begins with nonspecific, general symptoms (such as fever, fatigue and muscle weakness). [childrenshospital.org]

  […] a) fluctuation of the rash with twice daily fevers b) blue color changes to the hands with cold exposure c) oral ulcers d) new difficulty keeping up with peers in soccer Answer 2. [pediatrics.mc.vanderbilt.edu]

  […] the neck, shoulders, trunk, back or hips) Difficulty with everyday activities, such as climbing stairs, standing up, riding a bicycle, getting dressed, lifting the head Frequently falling down Weak voice (dysphonia) Difficulty swallowing ( dysphagia ) Fever [my.clevelandclinic.org]

• Fatigue

  The disease often begins with nonspecific, general symptoms (such as fever, fatigue and muscle weakness). [childrenshospital.org]

  When a child becomes irritable, fatigued, reluctant to socialize, and the face becomes flushed easily, physicians refer to this constellation of symptoms as "misery." The speed of the progression of JDMS is highly variable. [en.wikipedia.org]

  The inflammation of the muscles results into weakness of the muscles, clumsiness, fatigue, and even physical inactivity. [web.archive.org]

  Fatigue and fatigue along with muscle weakness are common symptoms Muscle pain may occur, but not at all Thigh muscles are most commonly attacked Symptoms may be trouble getting up from the floor or the chair, climbing stairs and hills Skin symptoms are [bindevevssykdommer.no]

  In addition to the classical symptoms of proximal symmetric muscle weakness further symptoms such as arthralgia, fatigue, and dysphagia can occur in DM. [thieme-connect.com]

• Weight Loss

  We describe a case of juvenile dermatomyositis associated with hepatocarcinoma.A 14-year-old boy with a weight loss of 15 kg over a three-month period presented with clinical signs of dermatomyositis, including typical edematous rash, muscular weakness [nejm.org]

  Dysphagia, myalgia, fever and weight loss are other features 7. There is cell-mediated injury targeted at striated muscle with resultant atrophy, edema, coagulation necrosis, fibrosis and calcification. Markers elevated muscle enzymes (e.g. [radiopaedia.org]

  Constitutional symptoms or signs, as used in these listings, means severe fatigue, fever, malaise, or involuntary weight loss. [ssa.gov]

  About half of children with JDMS also have pain in their muscles.Other symptoms may include irritability, weight loss, and mouth ulcers. [en.wikipedia.org]

  If the muscles in your esophagus are affected, you can have problems swallowing (dysphagia), which can cause weight loss and malnutrition. Aspiration pneumonia. [mayoclinic.org]

• Disability

  Disability can be measured using the Childhood Health Assessment Questionnaire. Originally developed for evaluating disability in children with arthritis, this tool has also been shown to be a valid and responsive tool in these patients, he said. [mdedge.com]

  After a mean follow-up of 4.9 years, 28 (78%) of the patients were well without functional impairment; five patients had inactive disease but with persisting disabilities; and three patients had active disease despite several years of treatment. [ncbi.nlm.nih.gov]

  The trial is aimed to find out the treatment regimen associated with the lowest occurrence of flare and the lowest drug related toxicity Scientific objectives: The proposed project is aimed to improve treatment approaches for rare, severe and disabling [clinicaltrials.gov]

  Please enable JS and disable any ad blocker [rheumatologynetwork.com]

  Juvenile Dermatomyositis Treatment The goal of JDM treatment is to reduce inflammation, improve function and prevent disability. This is accomplished through a combination of medications, physical and speech therapy and self-care methods. [arthritis.org]

• Malaise

  A 62-year-old woman presented to the emergency department with malaise, lethargy and proximal muscle weakness. She had a similar presentation in childhood. [ncbi.nlm.nih.gov]

  […] symptoms differently, but the most common signs are: fever rash around the eyelids (known as heliotrope rash ) and/or knuckles and finger joints (called Gottren’s papules ); a rash may also occur on the elbows, knees and ankles muscle weakness fatigue malaise [childrenshospital.org]

  Some individuals with dermatomyositis may also develop symptoms such as low-grade fever, a general feeling of ill health (malaise), shortness of breath (dyspnea), pain in multiple joints (polyarthralgia), weight loss, and/or other abnormalities. [rarediseases.org]

  Some patients display constitutional symptoms including fever, weight loss, anorexia, and malaise. [rheumaknowledgy.com]

• Dysphagia

  Dysphagia, myositis-specific or -related antibodies, factor VIII-related (von Willebrand) antigen, myalgia, muscle ultrasound and ulcerations had a median score of 4. [doi.org]

  The signs could include dysphonia, falling or dysphagia. Due to these symptoms, JDM is often misdiagnosed as muscular dystrophy or any other disease of the muscle. A few patients develop contractures (the muscle retracts and the joints remain bent). [web.archive.org]

  Five children developed dysphagia necessitating tube feeding and more aggressive therapy. Dysphagia due to weakness of the pharyngeal musculature is associated with severe forms of JDM. [indianpediatrics.net]

  In addition to the classical symptoms of proximal symmetric muscle weakness further symptoms such as arthralgia, fatigue, and dysphagia can occur in DM. [thieme-connect.com]

• Muscle Weakness

  A muscle biopsy was inconclusive, however, she was treated for a presumptive diagnosis of inflammatory myositis. In a second flare in childhood, she developed proximal muscle weakness and calcinosis cutis. [ncbi.nlm.nih.gov]

  All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. [doi.org]

  The muscle weakness often causes a medical misdiagnosis of muscular dystrophy or other muscle disease. [en.wikipedia.org]

• Myopathy

  […] with lung involvement Juvenile dermatopolymyositis with myopathy Clinical Information A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. [icd9data.com]

  New perspectives on the idio-pathic inflammatory myopathies of childhood. Curr Opin Rheumatol 1994; 6(6):575–582 PubMed CrossRef Google Scholar Rider LG. Outcome assessment in the adult and juvenile idiopathic inflam-matory myopathies. [link.springer.com]

  Mononuclear cells in myopathies: quantification of functionally distinct subsets, recognition of antigen-specific cell-mediated cytotoxicity in some diseases, and implications for the pathogenesis of the different inflammatory myopathies. [scielo.br]

  Juvenile dermatomyositis, the most common inflammatory myopathy of childhood, is a rare systemic autoimmune vasculopathy that is characterised by weakness in proximal muscles and pathognomonic skin rashes. [ncbi.nlm.nih.gov]

• Proximal Muscle Weakness

  A 62-year-old woman presented to the emergency department with malaise, lethargy and proximal muscle weakness. She had a similar presentation in childhood. [ncbi.nlm.nih.gov]

  All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. [doi.org]

  He considers that this may be dermatomyositis and tests for proximal muscle weakness. The proximal muscles are not found to be weak on physical exam although a subsequent blood test showed a mildly elevated CK and AST. [mhmedical.com]

• Myalgia

  Dysphagia, myositis-specific or -related antibodies, factor VIII-related (von Willebrand) antigen, myalgia, muscle ultrasound and ulcerations had a median score of 4. [doi.org]

  Dysphagia, myalgia, fever and weight loss are other features 7. There is cell-mediated injury targeted at striated muscle with resultant atrophy, edema, coagulation necrosis, fibrosis and calcification. Markers elevated muscle enzymes (e.g. [radiopaedia.org]

  Fever, weight loss, fatigue, myalgias, arthritis, lymphadenopathy, and abdominal pain are constitutional symptoms known to be associated with JDM. [5], [7] Gastrointestinal system involvement may lead to gut vasculitis and perforation; similarly, dyspnea [ijpd.in]

  Less than half of patients complain of myalgia at the onset. Pharyngeal skeletal muscle may be involved, causing upper pharyngeal dysphagia, dysphonia, nasal regurgitation, and the risk of aspiration. [rheumaknowledgy.com]

• Muscle Tenderness

  Clinical criteria Muscle biopsy (definitive) Autoimmune myositis should be suspected in patients with proximal muscle weakness with or without muscle tenderness. [merckmanuals.com]

• Skin Rash

  All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. [doi.org]

  The skin rash generally starts in the first few weeks after the onset of muscle symptoms. However, the skin rash may be absent early on and many physicians may fail to recognize this disease during its early stages. [cincinnatichildrens.org]

  The main symptoms of juvenile dermatomyositis are muscle weakness and skin rashes. This is caused by inflammation in the small blood vessels ( vasculitis ) in skin and muscle and also by inflammation of the muscle cells. [my.clevelandclinic.org]

• Skin Lesion

  The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. [ebi.ac.uk]

  Skin ulcerations reflect significant vasculopathy of the skin and may be a sign of internal organ vasculopathy. Ulcerative skin lesions are associated with more severe disease and worse prognosis. [clinicalgate.com]

  […] and chloroquine Mainstay adequate antibody levels to improved muscle function and for skin lesions in patients whom corticosteroids and immunosupressives have failed: Intravenous immune globulin Monoclonal antibodies (destruction of B cells): Rituximab [physio-pedia.com]

  Skin lesions Heliotrope: red-purple edematous erythema on the upper palpebra Gottron's sign: red-purple keratotic, atrophic erythema or macules on the extensor surface of finger joints Erythema on the extensor surface of extremity joints, slight raised [aafp.org]

• Suggestibility

  Observations suggest that genetic influence on host susceptibility may play a role in childhood dermatomyositis and that a combination of host and environmental factors is most likely necessary to produce the disease. [ncbi.nlm.nih.gov]

  Access to MRI was reported by 70.6% of respondents, 58% using it routinely to detect abnormalities suggestive of inflammatory myositis. [doi.org]

• Denial

  Three of 26 (13%) respondents did not use biologics due to insurance denial. One of 26 (4%) PR did not use a biologic due to parent denial, 15/26 (64%) due to not being sure a biologic would work, and 2/26 (9%) due to cost of therapy. [ped-rheum.biomedcentral.com]

• Malar Rash

  The classic heliotrope rash is a violaceous discoloration of the eyelids with associated eyelid edema; this is present in approximately 80% of children at diagnosis. About 40% of children also have a prominent erythematous malar rash. [clinicalgate.com]

  Malar and facial erythema, and erythematous rashes involving both sun-exposed and covered areas, are also common. [obgynkey.com]

• Confusion

  Enzymatic dissection into the right lower quadrant produced confusing clinical and radiographic signs and extensive retroperitoneal necrosis. [ncbi.nlm.nih.gov]

  Gottron’s papules are frequently confused with severe eczema if JDMS is not suspected. [clinicalgate.com]

  Muscle pain can be mistaken for growing pains or overuse, and weakness can be confused with laziness or sleepiness. There are several hallmark symptoms of juvenile dermatomyositis, often present at the time of diagnosis. [jointhealth.org]

  However, the presence of prominent vacuoles similar to that seen in glycogen storage disease (GSD) within the cytoplasm of myofibers [Figure 1] confused the histopathologic diagnosis. [ijdvl.com]

  It is thought that these extra antibodies confuse and interfere with the body’s immune system. [mda.org.au]

• Asthenia

  Prognosis Poor prognostic indicators include recalcitrant disease, delay in diagnosis, older age, malignancy, fever, asthenia-anorexia, pulmonary interstitial fibrosis, dysphagia and leukocytosis. [aafp.org]

• Leukocytosis

  Prognosis Poor prognostic indicators include recalcitrant disease, delay in diagnosis, older age, malignancy, fever, asthenia-anorexia, pulmonary interstitial fibrosis, dysphagia and leukocytosis. [aafp.org]

Treatment often includes combinations of corticosteroids, methotrexate, and other immunosuppressive agents. Disease outcome, if treatment is initiated early, is generally good. [ncbi.nlm.nih.gov]

To reach these goals, doctors work with families to find a treatment plan that works best for them. Treatment usually includes medication, physical therapy, and education. As your child's symptoms change, the treatment plan may also be changed. [cincinnatichildrens.org]

Diet is also an important component of treatment for any disease, and this is especially true for one requiring steroid treatment. [jointhealth.org]

Future research in the treatment of juvenile dermatomyositis should focus on improving the understanding of disease course and its predictors such that treatment protocols can be developed to provide the most benefit and least amount of medication toxicity [journals.lww.com]

The technique of nailfold capillaroscopy may prove to be a clinically useful, noninvasive means of early prognosis. [ncbi.nlm.nih.gov]

Ulcerative skin lesions are associated with more severe disease and worse prognosis. Buy Membership for Pediatrics Category to continue reading. Learn more here Netters Pediatrics [clinicalgate.com]

Differential diagnosis is important because treatment and prognosis is different. [kjim.org]

Prior to the widespread use of corticosteroids, the prognosis was poor. [emedicine.medscape.com]

Part II (Etiology and Mechanisms) covers advances in the immunopathology and viral etiology of PM/DM along with a frequently recognized entity: inclusion body myositis. [books.google.de]

Published on May 23, 2012 Dermatomiositis juvenil Abstract 13 Background: Juvenile dermatomyositis is a multisystemic disease of unknown etiology, characterized by non-suppurated inflamma- t... [issuu.com]

The etiology of jDM is unknown and the underlying immune mechanism poorly understood which is why current studies address the question of environmental triggers, genetic predisposition, or maternal microchimerism. [thieme-connect.com]

In patients with overlap syndrome, the myositis tends to respond better to treatment with corticosteroids than it does in patients with an idiopathic etiology. [physio-pedia.com]

Genetic factors and environmental triggers such as enterovirus and group B streptococcus infection and ultraviolet radiation exposure are likely to play an important role in the etiology. [2] JDM has been associated with significant mortality and morbidity [ijpd.in]

Understanding the epidemiology and treatment outcomes of this disease in children is important for better planning of appropriate diagnostic and treatment interventions. [ajol.info]

Clinical Classification of the Juvenile Idiopathic Inflammatory Myopathies (IIM) EPIDEMIOLOGY The annual incidence of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) ranges from 1.5 to 5 cases per 1 million children in various countries [obgynkey.com]

The epidemiology of polymyositis. Am J Med 1970; 48: 715-23. [ Links ] 2. Bridges BF. The rashes of dermatomyositis in a black patient. Am J Med 1991; 91: 661-2. [ Links ] 3. Pachman LM. Juvenile dermatomyositis. [scielo.br]

Further research examining the mechanisms of action of UVR in the pathogenesis of juvenile IIM is needed. [52, 53] Epidemiology In the United States, the annual incidence of JDM ranges from 2.5-4.1 cases per million population. [54] In the United Kingdom [emedicine.medscape.com]

Idiopathic inflammatory myopathies: epidemiology, classification, and diagnostic criteria. Rheum Dis Clin North Am. 2002;28(4):723-41. Fagan KA, Badesch DB. Pulmonary hypertension associated with connective tissue disease. [malattierare.regione.veneto.it]

The length of time before the initiation of treatment affects presenting symptoms, laboratory measures, and pathophysiology. [ncbi.nlm.nih.gov]

GENETICS AND PATHOPHYSIOLOGY The pathogenesis of JDM appears to involve chronic immune activation in genetically susceptible individuals following exposure to specific environmental triggers. [obgynkey.com]

Environmental and genetic factors affect the child's susceptibility to these conditions and also alter the inflammatory response, adding heterogeneity to disease pathophysiology. [link.springer.com]

Further understanding of the pathophysiology, role of cytokines in inflammatory myopathies, and pharmacogenomics should guide future clinical trials in patients with juvenile DM. [onlinelibrary.wiley.com]

In contrast, the main pathophysiologic abnormality in polymyositis is direct T cell-mediated muscle injury. [merckmanuals.com]

Delay in the diagnosis of FOP is common, although early recognition of FOP prevents a child from accidental or iatrogenic injury, which can precipitate ectopic ossification. [ncbi.nlm.nih.gov]

Early diagnosis and sticking to the treatment plan are important to prevent permanent muscle weakness. Children experience JDM differently. [rheumatology.org]

Physical therapy is directed at preventing muscle wasting, stiffness and contractures. Speech therapy. If the throat muscles become weak, speech therapy can help improve speech and swallowing. Skin protection. [arthritis.org]

Children with juvenile dermatomyositis should eat a diet low in sodium and high in calcium, to help prevent the brittle bones that are a potential side-effect of steroid treatment. [jointhealth.org]

Frequent exercise can prevent contractures. The first muscles that are first to be affected tend to be the proximal muscles such as those that are located on the shoulders, neck, back and abdomen. [web.archive.org]