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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This article reviews the clinical features and emphasises the treatment of polymyositis and dermatomyositis. Pharmacological intervention with corticosteroids and immunosuppressive agents is discussed. In addition, strategies regarding the initiation and subsequent tapering of these drugs are provided, and an algorithmic approach to the management of myositis is provided. Therapeutic modalities for patients with refractory disease are considered, and the potential adverse effects of such treatment are discussed. Since patients with myositis often have disease-related complaints other than muscle weakness, a practical treatment approach for these problems is also outlined.
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PMID:Current management of polymyositis and dermatomyositis. 265 93

A 49-year-old woman was admitted in February 1987, with a six-month history of joint pain, maculopapular and erythematous rash, proximal muscle weakness and a two-month history of skin ulceration and dyspnea on exertion. Physical examination showed Gottron's papules on her fingers and a faint heliotrope rash. Biopsy of erythematous skin lesions on the shoulder and the back of the hand revealed perivascular inflammatory cell infiltration and tiny ulcerative lesions of the cutaneous tissue. Biopsy of the right quadriceps muscle showed type II fiber atrophy and slight perivascular lymphocytic infiltrate, whereas serum CPK level was within normal range. Chest X-ray film showed granular infiltrates in both lower lung fields. Based on the current findings the case was diagnosed as dermatomyositis associated with interstitial pneumonia. The administration of prednisolone, 30 mg/day resulted in the improvement of the skin lesions and muscle weakness, while the intensity of lung infiltrates was little affected. Three months after steroid therapy, the patient was readmitted because of increasing dyspnea on exertion and multiple skin ulcers. Chest X-ray revealed a small amount of gas in the mediastinum, in addition to slight deterioration of interstitial lung disease. In spite of various treatments for pneumomediastinum, including bed rest, administration of analgesics and oxygen inhalation, it developed rapidly, complicated severe subcutaneous emphysema and right-sided pneumothorax. Although high-dose prednisolone therapy and mediastinal drainage were performed, the pneumomediastinum was not resolved and she died from respiratory failure. At autopsy, predominant histological features of the lungs were acute interstitial pneumonia with hyaline membrane and edematous granulation formation in the alveoli.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of dermatomyositis which rapidly developed to respiratory failure in the presence of pneumomediastinum]. 268 9

A retrospective review of 50 patients with dermatomyositis was performed to determine the temporal relationship between onset of muscle weakness and skin involvement. We found that cutaneous changes sometimes preceded muscle weakness more than a year before the onset of muscle weakness. These findings suggest that the characteristic dermatomyositis eruption without muscle weakness should not preclude a diagnosis of dermatomyositis, and these cases should be carefully followed.
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PMID:Cutaneous changes of dermatomyositis precede muscle weakness. 271 9

Dermatomyositis in childhood is an uncommon disease, affecting muscle and skin. The disease usually has an insidious onset; the proximal muscle groups are classically more affected than the distal group. If left untreated, the disease will either spontaneously arrest or will progress until the child is completely bedridden, with death secondary to hypoventilation and aspiration. For a definitive diagnosis 3 or 4 of the following criteria (plus rash) are required: 1) symmetrical limb girdle weakness; 2) muscle biopsy evidence of myositis and muscle necrosis; 3) elevation of muscle enzymes; 4) electromyographic changes of myositis. The main pathologic feature of juvenile dermatomyositis is vasculitis affecting small arteries and veins of muscle, skin and gastrointestinal tract. Whether muscle from patients with polymyositis contains a specific auto antigen or is contaminated with an immunogenic infectious agent such as a virus (coxsackie virus, for instance) remains unclear. Childhood dermatomyositis is almost uniformly responsive to steroid treatment; there is a good chance of remission with minimal risk of secondary complications with an initial low dosage of prednisone (1 mg/kg/day). The use of additional drugs such as azathioprine, methotrexate or cyclophosphamide is reserved for patients who are either not completely responsive to steroids or difficult to wean off steroids. Cyclosporine A has been proposed to achieve a reduction in steroid dosage.
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PMID:[Dermatomyositis in children]. 271 39

We have reviewed the results obtained in 21 dermatomyositis patients who were treated with plasma exchanges (PE) in 8 french centres between 1980 and 1986. Patients and methods. Seven of the 21 patients studied were male and 14 were female; 16 were children under 15 years of age. The disease was initially acute in 17 cases, subacute in 3 cases and chronic in 1 case. Plasma exchanges were performed as first-line therapy in 13 patients and after failure of the usual treatments in 18 patients. The decision to use PE therapy was prompted by an increase in muscle weakness in 14 of these 18 patients and by a lack of improvement despite treatment in the remaining 4 patients. PE therapy was started 17 months on average after the beginning of treatment. At the time 10 patients were under systemic corticosteroid therapy (prednisone or prednisolone greater than 1 mg/kg/day in 7 cases); 7 patients were receiving immunosuppressants jointly with corticosteroids (prednisone or prednisolone greater than 1 mg/kg/day in 5 cases); and 1 patient had systemic corticosteroids (3 mg/kg/day), methotrexate and antilymphocyte serum. In 3 patients, 2 of whom had severe muscle weakness. PE's were performed from the start. Our 21 patients underwent a total of 234 plasma exchanges. Each patient had a mean series of 11 +/- 6 PE's spread over 11 +/- 3 weeks. In addition to PE therapy, 12 patients received corticosteroids (greater than 1 mg/kd/day in 7 cases) and 8 had corticosteroids (greater than 1 mg/kd/day in 5 cases) together with immunosuppressants. One patient who was put on TE therapy from the start received no other treatment. It must be noted that in 12 patients the introduction of PE was accompanied by another therapeutic change.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Plasma exchange in dermatomyositis. A retrospective study of 21 cases]. 274 99

A retrospective study, paying particular attention to the clinical and evolutive aspects of the disease, was performed on 44 subjects affected by polymyositis/dermatomyositis (PM/DM) and hospitalized at the Institute of Medical Clinics of the 1st School of Medicine of Naples University. On the basis of the different clinical pictures, the cases were classified into the following groups: primary PM (4); primary DM (19); DM/PM associated with malignancy (6); childhood DM/PM (3); PM/DM associated with connective tissue disorders (12). Diagnosis was established in terms of the following criteria: a) symmetrical and mostly proximal bilateral muscle weakness (100%); b) elevation of serum enzymes (86.3%); c) electromyographic findings of myopathy sometimes with fibrillation potentials, increased insertional irritability and pseudo-myotonic discharges (93.1%); d) muscle biopsy changes compatible with a clinical form of polymyositis (83.3% out of 30 cases); e) dermatological manifestations including particularly pink or lilac edema-erythema over the periorbital areas, wine-red maculae, Gottron's sign, "poikiloderma vasculare atrophicans", telangiectasias and skin vasculitis (86.3%). An involvement of the extraneural apparatus and organs was present in 40 patients; the most damaged was the osteoarticular apparatus, followed by esophagus, lung, heart and kidney; such pathology was rarely present in the childhood form. A follow-up of the disease has been performed in 36 cases and the therapy consisted fundamentally of high dose corticosteroids (mostly prednisone), associated, in a minority of cases, with methotrexate. A clinical improvement was observed in most cases and a remission of the disease in part of the latter. However, a worsening of the illness was noticed only in the patients suffering from PM/DM associated with malignancy, and mortality rate was 11.1% in all.
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PMID:[Clinico-developmental aspects in 44 cases of polymyositis/dermatomyositis]. 278 12

Recent research findings implicate picornaviruses in the etiology of human polymyositis/dermatomyositis and suggest that genetic factors play a role in susceptibility to these diseases. We compared 2 variants of encephalomyocarditis (EMC) virus for their ability to induce polymyositis in adult mice, and evaluated what role the genetic background of the host plays in the degree of myositis induced. While BALB/c mice developed minimal myositis when infected with a diabetogenic variant (EMC-D), the same strain inoculated with a newly isolated myopathic variant (EMC-221A) developed viral dose-dependent elevations in muscle-associated enzymes, bilateral limb muscle weakness, and the histopathologic changes of severe polymyositis. Mice with different genetic backgrounds showed significantly different susceptibilities to EMC-221A. These data suggest that the severity of polymyositis induced by EMC virus is influenced by both the viral and host genomes.
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PMID:Viral and host genetic factors influence encephalomyocarditis virus-induced polymyositis in adult mice. 303 71

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.
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PMID:Inflammatory myopathies. 306

Authors report clinical and laboratory findings, treatment and evolution of six girls and three boys aged between 20 months and 13 years, diagnosed of juvenile polymyositis-dermatomyositis in the last seven years. Presenting symptoms were asthenia and proximal muscle weakness; in 3 cases characteristic skin lesions were associated. All were treated initially with prednisone p.o. (1-2 mg/kg/day) response being favourable in seven. Two patients with chronic evolution were treated with methotrexate and IV bolus of methylprednisolone.
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PMID:[Polymyositis and dermatomyositis in childhood]. 306 18

The anaesthetic management of two patients with severe muscle weakness--one suffering from acute dermatomyositis, the other from acute polymyositis--is described. Both patients presented for surgery for malignancy. Anaesthesia was induced with etomidate in one, thiopentone in the other. Alfentanil was used for analgesia and atracurium for muscle paralysis in both. Neuromuscular blockade was monitored using a peripheral nerve stimulator and no problems were experienced. Recovery of neuromuscular transmission and ventilatory function after operation were normal.
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PMID:Anaesthesia and acute dermatomyositis/polymyositis. 313 34


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