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

In 4 patients with clinical signs of dermatomyositis, confirmed by electromyography and muscle biopsy, a form of muscle fatigue was detected which was expressed clinically by predominantly proximal motor deficit, with phonation and deglutition disturbances, slightly influenced by prostigmine. In all patients, stimulation of the ulnar nerve at 3--10 Hz induced a decrement of muscle-evoked potentials in abductor digiti minimi and at 15--50 Hz an increment at the end of the trains (1.2 sec in duration) of repetitive stimulation (preceded in two cases by a decrement in the response to the fifth stimulus in the train). Stimulation at 30 Hz for 10 sec resulted in a transient facilitation, followed (at 3 Hz stimulation) by postactivation exhaustion which disappeared after 5--15 min. The post-tetanic facilitation, the incremental response and the myasthenic symptoms reverted to normal under treatment with corticosteroids, an immunosuppressor agent and guanidine hydrochloride. A mixed, pre- and postsynaptic mechanism is presumed to underlie the muscle fatigue in our patients. Electron microscopy of muscle biopsies disclosed zones of necrosis and, in incipient stages, large agglomerations of glycogen that had disorganized the structure of myofibrils. The end-plates in the biopsies were larger than normal and the cholinesterase reaction was hyperactive. Serum immunoelectrophoretic and electrophoretic data--increase of IgG and IgM, decrease of IgA and hypergammaglobulinaemia -- point to a possible autoimmune mechanism of the neuromuscular disorders in our patients.
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PMID:Myasthenia in patients with dermatomyositis: clinical, electrophysiological and ultrastructural studies. 21 37

Molecular hybridization using an enterovirus group specific probe detected virus RNA in muscle biopsy samples from 25 of 96 cases of inflammatory muscle disease and similarly from 41 of 158 cases of postviral fatigue syndrome (PFS). Enterovirus RNA was detected in only two of 152 samples of control muscle. The inflammatory myopathy group comprised patients with polymyositis (PM), juvenile dermatomyositis (JDM) or adult dermatomyositis (DM), and all showed the presence of an inflammatory infiltrate and fiber necrosis on histological examination of a muscle biopsy sample. In contrast, muscle samples from the PFS group were histologically normal except for non-specific changes such as occasional single fiber atrophy. By analogy with enteroviral myocarditis, which can progress to a post-inflammatory disease with persistence of virus in myocardium and disposes to the rapid development of dilated cardiomyopathy, we propose that PFS syndrome may be a sequela of a previous inflammatory viral myopathy.
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PMID:Persistence of enterovirus RNA in muscle biopsy samples suggests that some cases of chronic fatigue syndrome result from a previous, inflammatory viral myopathy. 840 78

Sight threatening ocular complications are rare in adult patients with dermatomyositis. We encountered a 52-year-old female with dermatomyositis who had severe visual disturbance and rapidly progressive intersitial pneumonia. She was admitted to our hospital because of skin erythema, general fatigue, mild fever, and severe bilateral visual disturbance. Rentinal hemorrhages, cotton wool spots, and macular edema were observed in her fundus at the first ophthalmic examination. A diagnosis of dermatomyositis was made because of the myogenic pattern of her electromyogram, elevation of serum creatine kinase, and skin lesions. Oral prednisolone treatment was started and the retinopathy was improved, but was complicated by acute interstitial pneumonia. The interstitial pneumonia was not respond to steroid pulse therapy with methylprednisolone, and the patient died of respiratory failure on the 47th day after the onset of visual symptoms. In adult dermatomyositis patients, the complication of severe retinopathy should be considered as a risk factor for rapid progress of interstitial pneumonia.
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PMID:[A case of dermatomyositis with severe retinopathy in a patient who died of acute interstitial pneumonia]. 917 Aug 52

A 47-year-old woman visited a clinic with dyspnea which had continued for two months and was followed by general fatigue and fever. Antibiotics were not effective. Edematous erythema occurred on her face, elbows, knees and feet, and she entered our hospital. A skin biopsy revealed interface dermatitis with severe edema and mucinosis in dermis. Diffuse bilateral infiltration was observed in the chest X-ray, and laboratory findings showed increased LDH, GPT, GOT and CPK. No antinuclear factor was detected. Her respiratory condition rapidly worsened, and she died eight days after hospitalization in spite of corticosteroid pulse therapy. The autopsy revealed that the main cause of death was diffuse alveolar damage (DAD). Interstitial pneumonia related to dermatomyositis is not histologically uniform; the response to the therapy depends on its histological type. The patients with dermatomyositis who have poor prognosis are clinically characterized by acute onset with general symptoms and less pronounced muscle weakness; they generally show DAD in their lungs. We need to establish a simple method for distinguishing histological types of interstitial pneumonia and adequate therapy for each one.
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PMID:An autopsy case of dermatomyositis with rapidly progressive diffuse alveolar damage. 951 7

The authors report three cases of dermatomyositis, which ha been erroneously diagnosed as "chronic fatigue syndrome" due to the presence of elevated titers of serum anti-Epstein Barr antibodies.
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PMID:Three cases of dermatomyositis erroneously diagnosed as "chronic fatigue syndrome". 971 54

A 44-year-old woman visited our hospital in August 1997 with complaints of general fatigue and low-grade fever. Initial examination disclosed slightly elevated muscle enzymes and inflammatory reactions. A diagnosis of dermatomyositis was made on the basis of findings of skin symptoms consistent with Gottron's signs and a slight decrease in muscular strength. Reticular shadows were observed in both lower lung fields on chest X-ray films, and examination of transbronchial lung biopsy specimens revealed interstitial pneumonitis. After 1 course of steroid pulse therapy, the patient was placed on a regimen of prednisolone (PSL) at 60 mg/day and cyclosporin (CsA) at 200 mg/day. Although her skin symptoms and interstitial pneumonitis temporarily subsided, aggravated interstitial pneumonitis and elevated blood LDH levels were observed as the steroid dose (PSL) was being tapered. After a second course of steroid pulse therapy, the PSL dose was increased to 50 mg/day and CsA was replaced by methotrexate (MTX), at 7.5 mg/week. This brought the patient's interstitial pneumonitis under control. Although our patient had dermatomyositis complicated by interstitial pneumonitis which belongs to the group of poor prognosis, she responded favorably to combination chemotherapy with low-dose MTX.
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PMID:[Successful combination chemotherapy with low-dose methotrexate and steroids for dermatomyositis complicated by interstitial pneumonitis]. 1049 4

A 46-year-old female was admitted to our hospital due to general fatigue, systemic edema and dyspnea with history of systemic sclerosis (SSc). The patient was diagnosed as mixed connective tissue disease (MCTD) based on Raynaud phenomenon, a high anti-RNP antibody level and clinical symptoms and laboratory findings suggesting SSc, dermatomyositis (DM) and systemic lupus erythematosus (SLE). After the admission, both alveolar hemorrhage and a rapidly progressive glomerulonephritis (RPGN) also developed and laboratory findings showed a positive remark of myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) and anti-glomerular basement membrane (GBM) antibody. She was therefore re-diagnosed as microscopic polyarteritis nodosa (microscopic PAN) combined with MCTD and treatment with high dose prednisolone and steroid pulse therapy dramatically improved general conditions and lung symptoms, but maintenance dialysis was persistent because of irreversible renal failure. However, 3 months after the admission, she died of acute exacerbation of interstitial pneumonitis that was unresponsive to steroid pulse therapy. Autopsy revealed interstitial pneumonitis with alveolar hemorrhage and crescentic glomerulonephritis (CrGN), in which immunofluorescent microscopy showed no deposition in agreement with pauciimmune type. The histological findings supported the diagnosis; primary microscopic PAN combined with MCTD, which is a quite rare case, to our knowledge. Furthermore, co-existence of MPO ANCA and anti-GBM antibody, clinical and histological findings of the case also lead us to reconsider the relevance of these antibodies to pathogenesis and/or categories of microscopic PAN and Goodpasture's syndrome.
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PMID:[A case of mixed connective tissue disease with microscopic polyarteritis nodosa associated with perinuclear-antineutrophil cytoplasmic antibody and anti-glomerular basement membrane]. 1061 88

Magnetic resonance imaging (MRI) and P-31 magnetic resonance spectroscopy (P-31 MRS) provide unique, quantitative data that cannot be obtained from routine laboratory tests. MRI is the method of choice for imaging of muscle abnormalities. It is also a very sensitive technique for localizing nonhomogeneous inflammation in inflammatory myopathies such as dermatomyositis, juvenile dermatomyositis, amyopathic dermatomyositis, polymyositis, and inclusion body myositis. During treatment of inflammatory myopathies, the extent and severity of inflammation may decrease at varying rates, but weakness and fatigue remain serious clinical problems. The metabolic abnormalities detected with P-31 MRS are more persistent and can be used for objective patient evaluation after the disappearance of inflammation and normalization of serum levels of muscle enzymes. With P-31 MRS, biochemical defects are quantitated, including low levels of ATP and phosphocreatine (PCr) and elevated concentrations of ADP and inorganic phosphate (Pi), which may all be related to weakness and fatigue. Thus, MRI and P-31 MRS are useful in assessing the status of patients with inflammatory myopathies during treatment with prednisone and immunosuppressive drugs.
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PMID:Utility of magnetic resonance imaging in the evaluation of patients with inflammatory myopathies. 1147 53

Amyopathic dermatomyositis is a variant of dermatomyositis that is characterized by the typical skin rash but without the muscle abnormalities. It has been proposed that the amyopathic and myopathic forms of dermatomyositis exist on a continuum, a concept that is supported by family and genetic studies and the observation that a small proportion of amyopathic patients transform to a frankly myopathic state. The amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that are usually normal or show only minimal abnormalities. Despite the lack of weakness, many patients complain of debilitating fatigue. More sensitive measures of muscle function, such as P-31 magnetic resonance spectroscopy, suggest that muscle metabolism is abnormal in amyopathic patients. The amyopathic form is more commonly seen in adults than in children, although juvenile cases are reported. Some early series suggested no association with underlying malignancies, but recent reports indicate that malignancies occur. Determining whether a patient has amyopathic rather than myopathic disease may have prognostic implications.
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PMID:Amyopathic dermatomyositis. 1147 54

Dermatomyositis and polymyositis are the two major idiopathic inflammatory myopathies. The Bohan and Peter's criteria are still useful despite the probably different pathogenesis of the two myopathies. Cutaneous manifestations of dermatomyositis include heliotrope rash and Gottron's papules. The heliotrope rash, with or without edema, in a distribution involving periorbital skin is very suggestive of the diagnosis. Papules may be found overlying the "kneedle" of the hand or the elbows, knees, feet. Periungueal erythema with telangiectasis were characteristic but not pathognomonic. Scalp involvement is common. Skin lesions of dermatomyositis may precede the development of the myopathy and may persist after the control of the myositis. Some patients have an amyopathic dermatomyositis with normal muscle-enzyme, magnetic resonance scan and muscle biopsy. Muscle disease affects the proximal muscles, is generally symmetrical and symptoms are fatigue, weakness and sometimes myalgia. Proximal dysphagia reflects an involvement of striated muscle of the pharynx or proximal esophagus. Camptocormia reflects a severe involvement of paravertebral muscle. Other systemic features may be seen: pulmonary involvement (mostly interstitial pneumonitis and hypoventilation), arthralgias or arthritis, cardiac involvement, vasculatis and calcinosis particularly in children or adolescents with dermatomyositis. Malignant disease is associated with idiopathic inflammatory myopathies with a frequency of approximatively 10 to 15% in dermatomyositis and 5 to 10% in polymyositis and is strongly correlated with age, more than 50% of the patient over 65 years old were found to have a cancer. In the absence of malignant disease, the mainstay therapy for dermatomyositis and polymyositis is systemic corticosteroids (mostly 1mg/kg). In the lake of response or high dose dependance, intravenous immunoglobulins or immunosuppressive drugs like methotrexate or azathioprine may be discuss. Cyclophosphamide show some effectiveness in interstitial pneumonitis. Cyclosporin might be effective in children, less in adults. The efficacy of tacrolimus, mycophenolate mofetil, leflunomide and anti-TNF therapy need some prospective studies to determine if there are of value in idiopathic inflammatory myositis.
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PMID:[Dermatomyositis and polymyositis: clinical aspects and treatment]. 1196 87


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