Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The inflammatory myopathies are a heterogeneous group of disorders with recent evidence demonstrating differences in clinical features, pathologic changes, pathogenesis, and response to therapy. The inflammatory myopathies generally produce predominantly proximal, symmetric muscle weakness and wasting. Additional criteria for diagnosis include elevated serum muscle enzymes, myopathic features on EMG, and muscle biopsy abnormalities, including muscle fiber necrosis, degeneration, and inflammatory infiltrates. Inclusion body myositis is distinctive in that distal weakness is most commonly equal to or greater than proximal weakness and muscle biopsy reveals rimmed, cytoplasmic vacuoles, eosinophilic inclusions in the cytoplasm, and nucleus and abnormal filamentous structures. Autoimmune mechanisms seem likely to be involved in the pathogenesis of these disorders and viral infection may be etiologically involved in some of these diseases. The differences in the site of immune-mediated damages suggest an angiography in dermatomyositis while direct muscle fiber involvement is more likely in polymyositis and inclusion body myositis. Therapy of these disorders is similar although some, particularly inclusion body myositis, may be particularly resistant to therapy. Prednisone is currently recommended as the first treatment with azathioprine or methotrexate added after 3 months if steroids are ineffective.
 ...
PMID:Inflammatory myopathies. 306

Three adults, 2 of whom had polymyositis and 1 with dermatomyositis, developed dysphagia during the course of their illness. Results of esophageal manometry supplemented with esophageal radiography indicated the presence of cricopharyngeal achalasia. Because of the severity of this disorder, which is associated with aspiration of esophageal contents into the airways, surgery to divide the cricopharyngeal musculature was performed in 2 patients, giving complete relief of their symptoms. Prednisone dosage was not increased to treat this condition since it arose not from weakness but from obstruction. A biopsy specimen taken from 1 patient demonstrated inflammatory changes in the obstructing muscle. A review of these 3 patients and 3 previously reported cases indicates that cricopharyngeal obstruction can be a dominant cause of dysphagia in patients with myositis. The recognition of this entity is important in the management of patients with myositis because: it has serious and potentially life-threatening implications; and in certain cases, it can be effectively treated with surgery.
 ...
PMID:Cricopharyngeal obstruction in inflammatory myopathy (polymyositis/dermatomyositis). Report of three cases and review of the literature. 400 73

Among the main concerns regarding the current therapy for the inflammatory myopathies are a lack of adequate controlled trials, a lack of objective means to reliably measure muscle strength, lack of natural history data, consideration of polymyositis, dermatomyositis, and inclusion-body myositis as a homogeneous group of inflammatory myopathies, and reliance on nonspecific markers for determining prognosis and assessing response to therapies. Prednisone remains the drug of choice in treating these disorders, although a controlled trial has never been undertaken to study its efficacy. Among the steroid-sparing agents, azathioprine, methotrexate, cyclosporine, and chlorambucil are used with invariably low to moderate success. There are no results of controlled trials to indicate whether one of these drugs is superior to another. Intravenous immunoglobulin, which is very expensive, was shown in a controlled trial to be effective in steroid-resistant dermatomyositis not only in dramatically improving muscle strength and skin rash but also in resolving the underlying immunopathology. Controlled trials of intravenous immunoglobulin in patients with polymyositis and inclusion-body myositis are under way. Inclusion-body myositis has emerged as a common inflammatory myopathy that is predictably disabling and resistant to most therapies.
 ...
PMID:Current treatment of the inflammatory myopathies. 786 79

Classical myopathic dermatomyositis (DM) is a chronic autoimmune disease characterized by an erythematous rash and severe, proximal muscle weakness. A disease variant, amyopathic DM, presents with the typical rash but without clinical evidence of muscle weakness. Prednisone and immunosuppressive drugs alleviate symptoms in many patients. Accurate longitudinal evaluations of patients are important to limit serious side effects of these drugs, including osteoporosis, cataracts, and growth inhibition. Metabolic abnormalities detected with 31P magnetic resonance spectroscopy (MRS) provide the best quantitative data for evaluating these patients. With 31P MRS, the levels of inorganic phosphate (Pi), phosphocreatine (PCr), ATP, and phosphodiesters (PDE) were determined in the quadricep muscles of patients during rest and exercise. Artificial neural network (ANN) analyses of these data were previously used for accurate classification of patients with myopathic or amyopathic DM and normal controls. In the present investigation, an artificial neural network was employed for further analysis of the 31P metabolite levels in quantitative, longitudinal evaluations of the extent (percent) of clinical improvement or deterioration during treatment with prednisone and immunosuppressive drugs. The ANN results showed that adult patients in a severe myopathic state could improve with treatment to a clinical status of amyopathic DM. In contrast, severely weak juvenile patients in the myopathic state recovered to normal status. One juvenile patient did not improve and remained in the myopathic state. Additionally, a serious clinical relapse in an amyopathic patient was predicted with serial ANN analyses well in advance of the actual clinical event. These network analyses show potential utility for clinical applications in muscle diseases.
 ...
PMID:Analysis of 31P MR spectroscopy data using artificial neural networks for longitudinal evaluation of muscle diseases: dermatomyositis. 971 79

Autosomal dominant polycystic kidney disease is a multisystem disorder characterized by multiple, bilateral renal cysts and is also associated with cysts in other organs, such as the liver, pancreas, and arachnoid membranes. Dermatomyositis is a disease which mainly involves the skin and muscles, although occasionally other organs are affected. In this report, a 56-year-old male patient with a four-year history of autosomal dominant polycystic kidney disease was presented. Renal failure was exacerbated by a coexisting dermato-polymyositis. Prednisone treatment with hemodialysis improved the situation. This is the first report renal failure in a patient with autosomal dominant polycystic kidney disease and dermato-polymyositis.
 ...
PMID:Renal failure in a patient with autosomal dominant polycystic kidney disease and coexisting dermato-polymyositis: first report in the literature. 1523 96

This is a 65-yr-old woman with dermatomyositis and extensive calcinosis. On physical examination subcutaneous nodules were widely distributed on her chest and extremities. Her current CPK is 163 IU/L (normal range 0-175); however, with a recent flare of her symptoms, the CPK peaked at 411 IU/L. Her current medications are Imuran 25 mg/d, Prednisone 4 mg/d, and conjugated equine estrogen 0.625 mg/d.
 ...
PMID:Pitfalls in interpretation: calcium that's not bone. 1530 89

A 64-year-old man was referred to our hospital, for a second opinion, with fever, skin lesions and general muscle pain. He has been treated in another hospital with antibiotics on suspicion of erysipelas. A week later skin lesions developed on the metacarpophalangeal and proximal carpophalangeal joints of the hands and nose. His mobility was impaired due to muscle pain and muscle weakness. He also showed proximal muscle atrophy and most importantly a typical heliotrope rash in the eyes. Based on these clinical observations, the most likely diagnosis was dermatomyositis. The diagnosis was confirmed by the presence of increased serum creatine kinase levels and abnormalities in skin and muscle biopsy. Prednisone (70 mg/kg) was initiated, but after 19 days the patient developed a Pneumocystis jiroveci pneumonia. He died of respiratory failure a few days later.
 ...
PMID:Red fist and muscle weakness with a rare complication. 2455 76

Panniculitis is a rare cutaneous manifestation of dermatomyositis (DM). The appearance of panniculitis during treatment with methotrexate (MTX) is exceptional and has only been described in 3 cases. We report a case of a 50-year-old woman suffering from DM since 1997 who was treated with corticosteroids showing favorable clinical and biological evolution. When a relapse occurred 2 years later, she was treated with higher-dose of corticosteroids in combination with a 7,5 mg weekly dose of methotrexate. The evolution was rapidly favorable. Eighteen months later, the patient had multiple subcutaneous nodules on limbs and buttocks. Anatomopathological examination showed panniculitis. There was no evidence supporting progression in DM. Prednisone dose was increased to 0.5 mg/kg/day, always in combination with MTX, without any clear signs of improvement. MTX treatment was stopped and the cutaneous lesions completely disappeared in 2 months without any relapse. This objective response lasted for 42 months. Our observation is particular given the occurrence of panniculitis in a patient undergoing treatment for dermatomyositis with methotrexate and illustrates the difficulties in the diagnosis. This entity must be known despite its exceptional nature since cutting off MTX treatment generally induces the disappearance of subcutaneous nodules.
...
PMID:[Panniculitis in patient undergoing treatment for dermatomyositis with methotrexate]. 3258 6

Treatment of dermatomyositis (DM) is often achieved with a stepwise algorithm. However, the literature lacks quality evidence to support the use of this therapeutic strategy. The result of a stepwise therapeutic strategy in the management of skin-only DM is presented to better understand the clinical outcomes and allow for future studies. A cohort of 102 patients with DM, 41 of whom had skin-only disease, were seen between July 2009 and April 2013 at a referral-based connective tissue disease clinic. The Cutaneous Dermatomyositis Disease Area and Severity Index was used to prospectively assess disease severity and the outcomes in 41 adult patients with skin-only DM were analyzed. Of the 41 patients with skin-only DM, 23 patients (56.1%) received antimalarial medications alone and 18 patients (43.9%) received second- or third-line agents. Ten patients (24.4%) remained at the first level of the treatment algorithm and received only hydroxychloroquine. Prednisone was included in the treatment regimen for 11 patients with skin-only disease (26.8%). The results show that management of cutaneous DM often requires second-line agents because antimalarial medications alone are insufficient to treat most patients with skin-only disease.
 ...
PMID:The systemic management of cutaneous dermatomyositis: Results of a stepwise strategy. 2923 12

Polymyalgia rheumatica (PMR) is a chronic systemic inflammatory disease that is common in individuals older than 70 years. Classic symptoms of PMR include pain in the neck, pelvic girdle, and shoulders. Morning stiffness that lasts at least 30 minutes is typical. Glucocorticoids are the mainstay of PMR management, and prednisone 12.5 to 25 mg/day or equivalent is recommended. Giant cell arteritis is a comorbidity of PMR. Dermatomyositis is a rare, idiopathic inflammatory myopathy characterized by erythematous skin lesions and inflammation of skeletal muscles. Dermatomyositis manifests as proximal muscle weakness and fatigue that occurs when patients rise from a seated position, walk, climb stairs, or lift objects. It is a systemic condition and also may affect joints, the esophagus, and lungs. Prednisone is started at a dose of 60 mg/day and then tapered slowly, based on response, to prevent recurrence. Dermatomyositis may be associated with malignancy.
 ...
PMID:Autoimmune Conditions: Polymyalgia Rheumatica and Dermatomyositis. 3264 Jan 51


1