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

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

Sporadic inclusion body myositis (sIBM) is considered to be the most common acquired muscle disease associated with aging. It is a disabling disorder still without effective treatment. sIBM causes weakness and atrophy of the distal and proximal muscles. Involvement of quadriceps and deep finger flexors are clues to early diagnosis. Dysphagia in the course of the disease is common. Muscle biopsy shows chronic myopathic features, lymphocytic infiltration invading non-necrotic fibbers, rimmed vacuoles and accumulation of amyloid-related proteins. It remains uncertain whether sIBM is primarily an immune-mediated inflammatory myopathy or a degenerative myopathy with an associated inflammatory component. This review describes the epidemiology and clinical features of the disease as well as the current genetic and pathogenic concepts and therapeutic approaches. Despite recent clues, in many respects sIBM remains an unsolved mystery.
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PMID:Sporadic inclusion body myositis: an unsolved mystery. 1947 72

Dysphagia is an important yet inconsistently recognized symptom of inclusion body myositis (IBM). It can be disabling and potentially life-threatening. We studied the prevalence and symptom-sign correlation of dysphagia. Fifty-seven IBM patients were interviewed using a standard questionnaire for dysphagia and 43 of these underwent swallowing videofluoroscopy (VFS). Symptoms of dysphagia were present in 37 of 57 patients (65%). Nevertheless, only 17 of these patients (46%) had previously and spontaneously complained about swallowing to their physicians. Both symptoms of impaired propulsion (IP) (59%) and aspiration-related symptoms (52%) were frequently mentioned. Swallowing abnormalities on VFS were present in 34 of 43 patients (79%) with IP of the bolus in 77% of this group. The reported feeling of IP was confirmed by VFS in 92% of these patients. Dysphagia in IBM is common but underreported by the vast majority of patients if not specifically asked for. In practice, two questions reliably predict the presence of IP on VFS: 'Does food get stuck in your throat' and 'Do you have to swallow repeatedly in order to get rid of food'. These questions are an appropriate means in selecting IBM patients for further investigation through VFS and eventual treatment.
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PMID:Detecting dysphagia in inclusion body myositis. 1960 45

THE INFLAMMATORY MYOPATHIES INCLUDE THREE DISTINCT ENTITIES: polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM). A T-cell-mediated cytotoxic process in PM and IBM and a complement-mediated microangiopathy in DM are the hallmarks of the underlying autoimmune processes. The most consistent therapeutic problem remains the distinction of PM from the difficult-to-treat mimics such as s-IBM, necrotizing myopathies and inflammatory dystrophies. This review provides a step-by-step approach to the treatment of inflammatory myopathies, highlights the common pitfalls and mistakes in therapy, and identifies the emerging new therapies. In uncontrolled studies, PM and DM respond to prednisone to some degree and for some period of time, while a combination with one immu-nosuppressive drug (azathioprine, cyclosporine, mycophenolate, methotrexate) offers additional benefit or steroid-sparing effect. In contrast, IBM is resistant to most of these therapies, most of the time. Controlled studies have shown that IVIg is effective and safe for the treatment of DM, where is used as a second, and at times first, line therapy. IVIg seems to be also effective in the majority of patients with PM based on uncontrolled series, but it offers transient help to a small number of patients with IBM especially those with dysphagia. Bona fide patients with PM and DM who become resistant to the aforementioned therapies, may respond to rituximab, tacrolimus or rarely to an tumor necrosis factor alpha inhibitor. For IBM patients, experience with alemtuzumab, a T-cell-depleting monoclonal antibody, is encouraging.
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PMID:Therapeutic advances and future prospects in immune-mediated inflammatory myopathies. 2118 May 74

This study compared self-reported incidence and prevalence of dysphagia in inflammatory myopathy diseases with instrumental data from videofluoroscopy in a cohort of 18 patients with inflammatory myopathies (inclusion body myositis, polymyositis, and dermatomyositis). We found a high self-report of incidence of dysphagia and demonstrated that symptoms described by the patients follow a characteristic pattern. We conclude that there is a high incidence of dysphagia in all three of the inflammatory myopathies. Questions about swallowing should routinely be included in inflammatory myopathy patient examinations in order to appropriately refer patients for further investigation of their swallowing function and avoid the complications associated with dysphagia.
Dysphagia 2012 Mar
PMID:Dysphagia in inflammatory myopathy: self-report, incidence, and prevalence. 2144 90

Inclusion body myositis (IBM) is the most common acquired myopathy in people older than 50 years. IBM typically presents with distal upper extremity weakness accompanied by proximal lower extremity muscle weakness. Associated clinical findings include asymmetric weakness, foot drop, and dysphagia. The pathogenesis of IBM is not clear. In this article the authors briefly discuss postulated pathogenic mechanisms. Although no proven pharmacotherapy exists, some promising candidates are discussed.
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PMID:Inclusion body myositis: diagnosis, pathogenesis, and treatment options. 2144 18

The nature of the swallowing impairment in patients with sporadic inclusion body myositis (s-IBM) has not been well characterized. In this study, we examined ten consecutive s-IBM patients using videofluoroscopy (VF) and computed pharyngoesophageal manometry (CPM). The patients were divided into two groups: patients with complaint and without complaint of dysphagia. VF results indicated pharyngeal muscle propulsion (PP) at the hypopharyngeal and upper esophagus sphincter (UES) in all s-IBM patients. Patients without complaint of dysphagia showed a mild degree of PP, whereas a severe form of PP was observed in patients with complaint of dysphagia. CPM revealed that negative pressure during UES opening was not observed in the s-IBM patients with complaint of dysphagia. Incomplete opening and PP at the UES were observed in all s-IBM patients. These results indicate that the dysphagic processes occur subclinically in s-IBM patients who may not report swallowing impairments.
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PMID:A dysphagia study in patients with sporadic inclusion body myositis (s-IBM). 2199 33

Sporadic inclusion body myositis (sIBM) is an intractable and progressive skeletal muscle disease of unknown cause that has no curative treatment. Its prevalence varies among countries and ethnic groups. The clinical course is slow and chronic worsening. Diagnosis of sIBM is usually made 5 years after onset. Muscle weakness and atrophy in the quadriceps, wrist flexor, and finger flexors are the typical neurological findings of sIBM. Dysphagia and asymmetric weakness are often found as well. Serum creatine kinase is usually below 2,000 IU/L. Muscle biopsy typically reveals endomysial inflammation, invasion of mononuclear cells into non-necrotic fibers, and rimmed vacuoles, suggesting that inflammation and degeneration are coexist in the pathomechanism. The etiology of sIBM is still unknown; however, genetic factors, aging, lifestyle, and environmental factors may be involved. Recent studies have implicated amyloid beta accumulation, defects of proteolysis, and immune system abnormalities in the pathomechanism of sIBM. sIBM is generally refractory to current therapy, such as steroids or immunosuppressants. Recently, alemtuzumab, which targets T cells, has resulted in improvement in quantitative muscle strength testing. New strategies to induce proteolysis and autophagy, accelerate muscle regeneration, inhibit myostatin, and modulate inflammatory cells are promising. Elucidation of the pathomechanism of sIBM is the key to developing effective therapies.
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PMID:[Inclusion body myositis]. 2206 73


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