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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM, Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10,000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM.
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PMID:Characteristics of juvenile dermatomyositis in Japan. 914 Dec 68

In order to know the prognosis of dermatomyositis, 119 patients with dermatomyositis were investigated from June to September 1995 by Shanghai Medical Cooperation Center on Dermatomyositis. Ten prognostic factors were studied with Lifereg Procedure multiple factor analysis. The results showed that 2 and 5-year survival rates were 75.3% and 53.2% respectively. The most common cause of death was lung infection and then was malignant tumor. Unfavorable prognostic factors were old age, malignant tumor, interstitial lung disease, dysphagia, cardiac involvement, fever, failure of remission and steroid therapy alone. The survival curve showed that mortality rate was high in the first three years, less between three and seven years and none after seven years. The results showed that dermatomyositis is a disease with poor prognosis. Patients with dermatomyositis can be adversely affected by many factors and should be treated regularly with steroid and immunosuppresive drugs as early as possible.
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PMID:[Prognostic factors of dermatomyositis: analysis of 119 cases]. 981 59

Clinical data of 34 patients with DM, who have been treated during the years 1971 and 1998 were evaluated. 79% of the patients (27 patients) were female, 21% of them (7 patients) were male. 59% of the patients (20 pts) were between the ages of 41 and 50 years. The characteristic heliotrop rash were observed in 26 patients, Gottron's papules in 20 patients, poikiloderma in 2 patients, calcification, ulcers, Raynaud syndrome in 1 patient. 3 of the 34 patients presented with strongly itching erythematopapulosus symptoms, most prominently on the scalp. Cardiac involvement were present in 10 patients (29%), lung involvement in 8 patients (23%), gastrointestinal complaints in 11 patients, dysphagia, dysphonia in 4 patients, joint pain in 5 patients. Overlap syndrome--scleroderma-dermatomyositis, SLE-dermatomyositis--was present in 2 patients. 9 of the 34 patients (26%) suffered from malignant tumours: gastric, breast, lung, epipharynx carcinoma, malignant melanoma. 13 of the 34 patients have been treated with corticosteroids together with immunosuppressor agents, in most cases azathioprin was administered. Cyclosporin was given in 7 cases, chloroquine in 2 cases.
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PMID:[Dermatomyositis: clinical study of 34 patients]. 1069 80

A pilot study was conducted to assess the efficacy of early treatment of severe juvenile dermatomyositis (JDMS) patients with intravenous methylprednisolone (IVMP) and methotrexate (MTX). Twelve children diagnosed with severe JDMS were treated with IVMP and MTX. Six patients were treated early (within 6 weeks of the diagnosis) while in the other six patients, MTX was started 5-72 months after the diagnosis was made. The clinical responses of the patients to treatment, including alterations in muscle strength, muscle enzyme levels and corticosteroid dosage as well as the development of side-effects, were recorded. The indications for starting the treatment were defined and documented. The primary measures of response were resolution of the clinical indications for treatment, decreased activity of the disease manifestations and tapering of the corticosteroids to the minimal dose or discontinuation without clinical or biochemical flare. The main indications for starting IVMP and MTX were dysphagia and severe cutaneous vasculitis. All the patients received MTX orally for at least 8 months, as well as IVMP (30 mg/kg/dose), but none of the patients was on additional second-line treatments. The six patients who were treated early with MTX showed a significant clinical improvement. In five out of the six, the corticosteroid dosage was eventually reduced to <5 mg/day. None of them developed calcinosis. In contrast, two of the six patients who were treated late with MTX developed calcinosis. This study suggests that MTX and IVMP are a useful combination in the early treatment of severe JDMS. Given the fact that our sample was small, further studies in a controlled trial are necessary to confirm these findings.
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PMID:Efficacy of early treatment of severe juvenile dermatomyositis with intravenous methylprednisolone and methotrexate. 1079 26

Acute inflammatory myopathy with severe subcutaneous edema is extremely rare and has been reported in only a handful of cases. We describe two similar patients presenting with this disorder and generalized rash. Unlike the five previously reported cases, the clinical and histologic features of our two patients are more suggestive of dermatomyositis than polymyositis. Nevertheless, scrutinizing all seven reported patients, a number of specific characteristics could be defined. All patients were adult males. Dysphagia was present in four. In six patients, acute inflammatory myopathy was idiopathic while malignancy was present in one. Two patients died despite intensive therapy, three improved on corticosteroid treatment, and two recovered spontaneously. In all patients, limb involvement with marked subcutaneous edema was present, clinically mimicking deep vein thrombosis in both our patients. The presence of severe subcutaneous edema may be a hallmark of a distinctive variant of acute inflammatory myopathy. More cases are needed to discern subtypes of this general entity and to establish guidelines for treatment and prognosis.
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PMID:Acute inflammatory myopathy with severe subcutaneous edema, a new variant? Report of two cases and review of the literature. 1141 62

Hypertrophy of the cricopharyngeal muscle is a serious clinical condition that can cause severe dysphagic symptoms, including prolonged deglutition and postdeglutitive aspiration. Although the therapeutical concepts are well established, the pathogenic mechanism of cricopharyngeal hypertrophy remains unclear. We present a patient with a ten-year history of progressive dysphagia. The neurological and MRI findings were normal. However, videocineradiography showed severe hypertrophy of the cricopharyngeal muscle. This condition was first treated by injections of botulinum toxin, which did not alleviate the symptoms. Next, myotomy and muscle biopsy were performed. Histological evaluation disclosed lymphoplasmacellular florid myositis, single-fiber atrophy, and muscle fiber necrosis with phagocytosis. There were no signs of inclusion body myositis or oculopharyngeal muscular dystrophy. Our finding of severe cricopharyngeal muscle hypertrophy associated with myositis has been published previously (n = 34). The study presented here shows cricopharyngeal dysphagia associated with various systemic diseases, including motor neuron disease, general granulomatous disease, dermatomyositis, or inclusion body myositis. Isolated changes of the cricopharyngeal muscle were described in 65% of the cases.
Dysphagia 2001
PMID:Cricopharyngeal muscle hypertrophy associated with florid myositis. 1172 Mar 99

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

We retrospectively studied 132 patients with dermatomyositis; 84 had idiopathic, 30 paraneoplastic, 5 juvenile and 13 amyopathic forms of the disease. The commonest features were macular erythema, heliotropic erythema and Gottron's papules. Flagellate erythema occurred in 5% of patients with idiopathic dermatomyositis and correlated with the disease activity. Necrotic lesions were also found in this group of patients but did not always signal malignancy. The prevalence of malignancy was high (23%). Raynaud's phenomenon occurred in 10.6% of patients, also in those with malignancy. Dysphagia, interstitial lung disease and arthralgias affected 20%, 8% and 40% of patients, respectively. Anti-Jo-1 antibodies were found in 5% of patients with idiopathic dermatomyositis and low titre ANA in 1/3 of patients. ANA did not correlate with the disease activity. We confirmed the data from the literature, but no cutaneous sign, constitutional symptom or circulating antibody was found marking a particular subtype of the disease.
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PMID:Dermatomyositis in 132 patients with different clinical subtypes: cutaneous signs, constitutional symptoms and circulating antibodies. 1201 99

The nature of the oropharyngeal dysphagia in polymyositis/dermatomyositis (PM/DM) has been investigated by EMG methods. Nineteen patients with PM/DM were studied. The oropharyngeal phase of swallowing was evaluated by the electrophysiological methods measuring the laryngeal relocation time, pharyngeal transit time and the triggering of the pharyngeal phase of swallowing reflex. The EMG of cricopharyngeal muscle of the upper esophageal sphincter was also recorded in 10 patients. The patients have been compared with a group of 22 healthy controls matched with age and gender. Dysphagia limit was also measured for all patients and control subjects. Fourteen out of 19 patients could not swallow 20 ml or less amount of water at one go and divided the bolus into two or more pieces (piecemeal deglutition) in comparison to normal subjects. In PM/DM patients, the triggering of the swallowing reflex for the voluntarily initiated swallow was normal while the pharyngeal phase of swallowing was significantly prolonged. The cricopharyngeal sphincter muscle EMG demonstrated severe abnormalities in halves of the patients investigated. These findings demonstrated the weakness of the striated oropharyngeal muscles. Cricopharyngeal sphincter muscle was affected less frequently and showed either hyperreflexic or hyporeflexic states during swallowing. It is concluded that the pharyngeal stage of oropharyngeal swallowing is mainly involved in patients with PM/DM.
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PMID:Oropharyngeal dysphagia in polymyositis/dermatomyositis. 1556 50

A 78 year-old woman was admitted to our hospital because of subacutely progressive dysarthria, dysphagia, proximally dominant muscle weakness and erythema in the neck and back. She was diagnosed as having rheumatoid arthritis (RA) at the age of 60 and treated with bucillamine (BUC) for 8 years. Laboratory tests included a rheumatoid factor of 1,472U/ml. Serum creatine kinase level was slightly elevated. The activated T cells in the peripheral blood were markedly increased. Needle EMG demonstrated myogenic changes. The magnetic resonance image of the left upper arm showed diffuse muscle atrophy and inflammatory changes in the triceps muscle. The muscle biopsy revealed perivascular inflammatory cell infiltraton and type II fiber atrophy. A biopsy from the skin showed mild perivascular inflammatory cell infiltraton. According to the results of these findings, she was thought to have dermatomyositis due to BUC. After withdrawal of BUC followed by the administration of prednisolone 1mg/kg, her symptoms improved and activated T cells in the peripheral blood were decreased. In Japan, BUC is widely accepted as an effective drug in the treatment of RA, even though it is known to induce some autoimmune diseases. However, the mechanism of the development of autoimmune disease is unclear. We considered that the long-term use of bucillamine could trigger an autoimmune response such as an increase in activated T cells and the development of dermatomyositis-like clinical features in our patient. In conclusion, when RA patients treated with BUC show a clinical picture compatible with dermatomyositis, its causative relationship has to be considered.
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PMID:[Bucillamine-induced dermatomyositis-like clinical features in a patient with rheumatoid arthritis]. 1571 1


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