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

Idiopathic inflammatory myositis is characterized by progressive weakness of the proximal muscles. There is a higher risk of malignancy than in the normal population. The aim of this study was to evaluate the frequency of malignancy among 251 myositis patients. We also compared clinical and immunological characteristics of cancer-associated myositis with primary myositis. There were no malignancies among polymyositis, overlap, or juvenile myositis patients. Twenty-two of ninety dermatomyositis patients also had a malignant disease. Patients with cancer-associated dermatomyositis were significantly older than primary myositis patients and had more severe cutaneous and muscle symptoms. Dysphagia and diaphragmatic involvement were more frequent among cancer-associated patients, while extramuscular features were less frequent. After successful treatment of the malignancy, we were able to manage myositis symptoms. One-year survival rate was significantly better in primary dermatomyositis patients. The subset of cancer-associated myositis differs from primary myositis in many aspects of its clinical and immunological features. Prognosis and life expectancy in cancer-associated myositis patients is determined by the underlying malignant disease. Therefore, age- and sex-specific examinations for detection of an underlying malignancy are important in the management of patients with dermatomyositis.
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PMID:Cancer-associated myositis: clinical features and prognostic signs. 1612 45

The aim of this study was to evaluate outcome in children with juvenile dermatomyositis (JDMS) at a tertiary care center in North India and have a long term follow-up. Medical records of children with JDMS managed at a tertiary care hospital were reviewed during a 13-year period to determine (1) interval between onset of symptoms and diagnosis, (2) treatment modalities used and (3) long term functional outcome. Thirty three patients diagnosed with JDMS met the inclusion criteria. Mean age at diagnosis was 8.7 +/- 3.3 years. Mean duration of disease prior to treatment was 1.18 years (range 1 month-5 years). The total follow-up period was 136.7 patient-years. Immunosuppressive therapy was given in 31/33 and a distinct monocyclic course was seen in 72.7% cases. Lipodystrophy was seen in 10/33 (30.3%), calcinosis in 7/33 (27.3%), cutaneous ulcers in 6/33 (18.2%), dysphagia in 5/33 (15.2%), and contractures in 4/33 (12.1%) cases. A steady and sustained response was seen in patients who had received "adequate" doses of steroids at the time of initiation of treatment. Methotrexate, hydroxychloroquine, azathioprine and intravenous immunoglobulin were used in patients with poor response to corticotherapy. There were two deaths in our series. Stepwise, aggressive treatment directed at achieving rapid and complete control of muscle inflammation is highly successful in minimizing the long-range sequelae of JDMS. Our patients seem to have a different clinical profile on follow-up as compared to series published from the West.
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PMID:Twelve years experience of juvenile dermatomyositis in North India. 1616 42

Subcutaneous generalized edema associated with dermatomyositis (DM)/polymyositis (PM) is extremely rare. Herein we report a case of severe subcutaneous generalized edema complicating DM. A 78-year-old woman was hospitalized in our department because of massive edema in the four limbs. Elevated muscle enzymes, heliotrope rash, results of electromyography, and muscle biopsy confirmed the diagnosis of DM. The absence of other diseases that could cause the symptoms indicated that massive edema was correlated with the pathophysiology of DM. Although myopathy and edema responded well to oral prednisolone, dysphagia persisted. We conclude that subcutaneous generalized edema can occur during the course of DM/PM, and subcutaneous vasculopathy may be involved in the pathogenesis of DM/PM.
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PMID:Severe subcutaneous generalized edema in a patient with dermatomyositis. 1743 77

Generalized subcutaneous edema is an uncommon manifestation of inflammatory myopathy. We report a 48-year-old female patient who presented with severe generalized edema, an erythematous skin rash, dysphagia and proximal muscle weakness. She was diagnosed with dermatomyositis from the clinical signs, increased muscle enzymes, electromyographic findings and a muscle biopsy. Magnetic resonance imaging revealed increased signal intensity in the muscular and subcutaneous layers. The conditions causing generalized edema were excluded. It was concluded that the generalized edema was secondary to dermatomyositis. Aggressive treatments with high-dose glucocorticoids and immunosuppressive agents were used to control the severe subcutaneous edema.
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PMID:Acute dermatomyositis associated with generalized subcutaneous edema. 1819 26

The incidence of lung neoplasms is increasing in Brazil and in the world, probably as a result of the increase in smoking. Due to the greater number of cases, atypical presentations appear. We report the case of a 66-year-old hypertensive male smoker who presented progressive proximal muscular weakness and, in two months, evolved to dysphagia, dysphonia, and V-shaped skin lesions on the chest. A chest X-ray showed a spiculated pulmonary nodule in the right upper lobe. The biochemical analysis revealed elevated creatine kinase levels. After complementary tests and biopsies, the patient underwent right upper lobectomy. Histopathology showed a moderately differentiated adenocarcinoma. The overall analysis of the case and a review of the literature allow us to suggest that the clinical profile of the patient was a result of an overlap of two paraneoplastic syndromes (dermatomyositis and Lambert-Eaton myasthenic syndrome) secondary to lung adenocarcinoma.
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PMID:Lung adenocarcinoma, dermatomyositis, and Lambert-Eaton myasthenic syndrome: a rare combination. 1854 31

A 46 year old woman presented with a one month history of rash and mylagia. The history, clinical findings and blood tests all supported a diagnosis of dermatomyositis. The patient later developed dysphagia and was successfully treated with intravenous immunoglobulin. Investigations and treatment of dysphagia in the context of dermatomyositis are discussed.
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PMID:Dysphagia secondary to dermatomyositis treated successfully with intravenous immunoglobulin: a case report. 1865 69

A number of autoimmune disorders have been identified in which IVIg treatment may be beneficial. Evidence for the use of IVIg in inflammatory myopathies comes from controlled trials in dermatomyositis (DM) and sporadic-inclusion body myositis (s-IBM). In DM, muscle strength was increased and neuromuscular scores and skin rashes improved. Results for s-IBM have not been as encouraging as those observed for DM. Subsequently, IVIg should be recommended as a second-line therapy in DM and used for life-threatening dysphagia in s-IBM. Using an animal model of experimental autoimmune myasthenia gravis (MG), studies also indicate that IVIg can significantly improve clinical symptoms and affect pathogenic idiotypic antibodies. In human MG, studies indicate that IVIg exhibited equal efficacy compared to plasmapheresis. IVIg can therefore be recommended for use in an MG crisis or in lieu of plasmapheresis. The role of IVIg in the chronic management of MG has not been studied. IVIg has also been investigated in autoimmune CNS disorders. In a controlled study in patients with stiff person syndrome IVIg was effective, with improvements in the distribution of stiffness index and heightened sensitivity scores. For neurodegenerative diseases such as Alzheimer's disease, post-polio syndrome, pain, fibrosis, and autoimmune sleep disorders, some early promising results for the use of IVIg are emerging, but remain to be fully investigated. In conclusion, IVIg appears to be an effective treatment for a number of autoimmune disorders, however, optimal dosing and pharmacogenetic studies are necessary.
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PMID:IVIg in other autoimmune neurological disorders: current status and future prospects. 1868 21

Polymyositis (PM), dermatomyositis (DM) and sporadic inclusion body myositis (s-IBM) are severe inflammatory muscle disorders of unknown cause, which may present life-threatening complications. Prognosis and response to medications may be predicted not only from the clinical and pathologic diagnostic group into which a patient belongs, but also from the patient's myositis-specific antibody status, extraskeletal muscle involvement, and the interval between onset of muscle weakness, and the start of the treatment. Corticosteroids remain the mainstay of treatment in PM and DM. In patients refractory or intolerant to corticosteroids, another therapy, often an immunosuppressive agent, or intravenous immunoglobulin (IVIg), is added. IVIg seems the treatment of choice in severe myositis with dysphagia. New molecules, anti-TNF and monoclonal antibodies anti-CD20 justifies randomised trial and long term follow up.
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PMID:[Current therapy for polymyositis and dermatomyositis]. 1892 83

The three major immune-mediated inflammatory myopathies, dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM), each have their own distinctive clinical features, underlying pathogenetic mechanisms and patterns of muscle gene expression. In DM a complement-dependent humoral process thought to be initiated by antibodies to endothelial cells results in a microangiopathy with secondary ischemic changes in muscles. On the other hand, in PM and IBM there is a T-cell response with invasion of muscle fibers by CD8+ lymphocytes and perforin-mediated cytotoxic necrosis. In IBM degenerative changes are also a feature and comprise autophagia with rimmed vacuole formation and inclusions containing beta-amyloid and other proteins whose accumulation may be linked to impaired proteasomal function. The relationship between the inflammatory and degenerative component remains unclear, as does the basis for the selective vulnerability of certain muscles and the resistance to conventional forms of immunotherapy in most cases of IBM. Patients with DM or PM usually respond to treatment with glucocorticoids and immunosuppressive agents but their use remains largely empirical. Intravenous immunoglobulin therapy can be used to achieve disease control in patients with severe weakness or dysphagia, or in patients with immunodeficiency, but its use is limited by expense. Emerging therapies for resistant cases include TNFalpha inhibitors (etanercept, infliximab) and monoclonal antibodies (rituximab, alemtuzumab). However, experience with these therapies is still limited and there is a need for randomized trials to test their efficacy and establish guidelines for their use in clinical practice.
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PMID:Inflammatory muscle diseases. 1897 52

An overlap syndrome of dermatomyositis and scleroderma is reported. The case corresponded to a 27-year-old female with a clinical picture of 14 months evolution, characterized by proximal muscles weakness, erythematous rash in wrists, knees, ankles, Gottron sign, heliotrope periorbital rash and dysphagia. A muscle biopsy was compatible with dermatomyositis; meanwhile the skin biopsy was compatible with scleroderma. Muscle enzymes were increased. Interestingly, the antinuclear antibody determination in HEp-2 cells was positive with a remarkable titer of 81,920 exhibiting a nucleolar pattern. Anti-Jo1 antibody was negative, but anti-PM/Scl-100 positive. The patient received methylprednisolone and cyclophosphamide pulses, with gradual improvement. Present report constitutes a case of overlap dermatomyositis-scleroderma syndrome, with anti-PM/Scl autoantibodies (anti-exosome). The remarkable of this case was the exceptional high antinucleolar antibody titer.
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PMID:A dermatomyositis and scleroderma overlap syndrome with a remarkable high titer of anti-exosome antibodies. 1913 55


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