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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Inclusion body myositis is characterized by an insidious onset, progressive indolent course, and is generally felt to be refractory to standard therapy for myositis. We reviewed the charts of 32 patients with muscle biopsy findings suggestive of inclusion body myositis. The average time from symptom onset to diagnosis was 37 months, but initially 40% were incorrectly diagnosed. Twenty-eight patients (88%) were classified as definite or probable inclusion body myositis and were treated with various combinations of prednisone and immunosuppressive agents. Sixty-eight percent of those treated experienced a decrement in function and muscle strength. Three patients exhibited longterm improvement while 12 patients experienced delayed progression, defined by short term improvement in strength or a stable functional class, All of these patients received therapy, 5 in the form of methotrexate and prednisone. All untreated patients deteriorated clinically. In summary, (1) inclusion body myositis is a clinically distinct entity which is frequently misdiagnosed initially. (2) While clinical improvement with therapy is rare, our observations support recent reports that therapy may be associated with a slower rate of clinical progression. (3) Optimal therapy remains uncertain, but the use of low dose methotrexate and prednisone may warrant further study.
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PMID:Inclusion body myositis: analysis of 32 cases. 133 40

Myositis describes a heterogeneous group of disorders whose main pathologic feature is chronic inflammation of the affected muscles. The association of myositis with other autoimmune diseases, the response to corticosteroid and immunosuppressive therapy, the frequent occurrence of autoantibodies, and the presence of chronic inflammatory cells in the affected muscles of patients with myositis indicate that the myositis syndromes are autoimmune diseases. This review summarizes recent observations on the role of humoral and cellular mechanisms in myositis. During the past year, the most notable contributions included studies on the relationship among autoantibodies and various clinical and epidemiologic features of patients with myositis; further evidence for T-cell involvement in the pathogenesis of myositis; demonstration of amyloid proteins in muscle fibers of patients with inclusion body myositis; and a controlled trial of plasma exchange and leukapheresis in myositis.
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PMID:Immune aspects of myositis. 133 83

Pathological diagnosis of dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM) should be possible in almost all cases when an appropriately involved muscle is biopsied. DM shows characteristic patterns of muscle fiber damage and capillary damage. Lymphocytes and macrophages are seen in PM and IBM partially invading non-necrotic fibers. IBM is also characterized by rimmed vacuoles with membranous whorls, characteristic masses of filaments in cytoplasm and sometimes in nuclei, and grouped atrophic fibers. Muscle fiber damage in PM is more variable. Inflammatory myopathy can be associated with HTLV-1 and HIV infection. In the latter a strong resemblance to PM is reported. Separate, still less well characterized forms of inflammatory myopathy occur in young children.
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PMID:The pathological diagnosis of specific inflammatory myopathies. 134 42

During the course of a systematic study of T cell lines derived from muscle of patients with various inflammatory myopathies, we identified a new form of polymyositis that is mediated by gamma-delta T cells. In the affected patient's muscle CD3+CD4-CD8- gamma-delta T cells surrounded and invaded nonnecrotic muscle fibers in the same way as CD3+CD8+ alpha-beta T cells surround and invade nonnecrotic muscle fibers in inclusion body myositis and other forms of polymyositis. Gamma-delta T cells were extremely rare or absent in muscles and muscle-derived T cell lines in other patients with polymyositis, inclusion-body myositis, dermatomyositis or granulomatous myopathy. This new form of polymyositis has provided us with a unique opportunity to study cytotoxic gamma-delta T cells and their muscle-fiber targets in situ. All muscle fibers expressed HLA-class I antigen and the 65-kD heat-shock protein. The autoaggressive behavior of the gamma-delta T cells is consistent with the hypothesis that in some inflammatory myopathies autoinvasive T cells recognize muscle fiber associated antigen(s). Further studies are needed to define the type of gamma-delta T cell receptor used and the antigen(s) recognized by gamma-delta T cells in this rare type of autoimmune muscle disease.
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PMID:The role of gamma-delta T lymphocytes in inflammatory muscle disease. 153 37

The IIM are a heterogeneous group of systemic rheumatic diseases which share the common features of chronic muscle weakness and mononuclear cell infiltrates in muscle. A number of classification schemes have been proposed for them, but none takes into consideration the marked immunologic, clinical, and genetic heterogeneity of the various clinical groups. We compared the usefulness of myositis-specific autoantibodies (anti-aminoacyl-tRNA synthetases, anti-SRP, anti-Mi-2 and anti-MAS) to the standard clinical categories (polymyositis, dermatomyositis, overlap myositis, cancer-associated myositis, and inclusion body myositis) in predicting clinical signs and symptoms, HLA types, and prognosis in 212 adult IIM patients. Although patients with inclusion body myositis (n = 26) differed in having significantly more asymmetric and distal weakness, falling, and atrophy than other patients, there were few other significant differences among the other clinical groups. In contrast, autoantibody status defined distinct sets of patients and each patient had only 1 myositis-specific autoantibody. Patients with anti-amino-acyl-tRNA synthetase autoantibodies (n = 47), compared to those without these antibodies, had significantly more frequent arthritis, fever, interstitial lung disease, and "mechanic's hands"; HLA-DRw52; higher mean prednisone dose at survey, higher proportion of patients receiving cytotoxic drugs, and higher death rates. Those with anti-signal recognition particle antibodies (n = 7) had increased palpitations; myalgias; DR5, DRw52; severe, refractory disease; and higher death rates. Patients with anti-Mi-2 antibodies (n = 10) had increased "V-sign" and "shawl-sign" rashes, and cuticular overgrowth; DR7 and DRw53; and a good response to therapy. The 2 patients with anti-MAS antibodies were the only ones with alcoholic rhabdomyolysis preceding myositis; both had insulin-dependent diabetes mellitus, and both had HLA-B60, -C3, -DR4, and -DRw53. These findings suggest that myositis-specific autoantibody status is a more useful guide than clinical group in assessing patients with myositis, and that specific associations of immunogenetics, immune responses, and clinical manifestations occur in IIM. Thus the myositis-specific autoantibodies aid in interpreting the diverse symptoms and signs of myositis patients and in predicting their clinical course and prognosis. We propose, therefore, that an adjunct classification of the IIM, based on the myositis-specific autoantibody status, be incorporated into future studies of their epidemiology, etiology, and therapy.
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PMID:A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. 165 47

Myofascial pain and dysfunction is the primary diagnosis in a large proportion of facial pain complaints. Myofascial disease can present in the form of trigger points, fibromyositis, myositis, muscle spasm, and muscle weakness. The purpose of this study is to research for quantifiable physiological differences between groups of normal subjects and myofascial pain dysfunction (MPD) patients. The first specific aim was to determine the opening of the jaw at which maximal isometric tension can be produced by the jaw closing muscles, with the hypothesis that this opening of maximal tension would be less for a group of MPD patients than for a group of normal subjects being tested. The second aim was to test the hypothesis that the maximal isometric bite forces for the two groups would differ. Patients with mandibular dysfunction are reported to have a lower maximal bite than normal subjects. Bite force was measured with the T-Scan system. An 80 micron horseshoe-shaped sensor connected to a dedicated IBM XT computer recorded the data. A self-contained printer produced the hard copy for later analysis. Vertical dimension or jaw opening was increased in 0.5-mm increments using double flat plane appliances standardized by the Relator. A MANOVA was used for statistical analysis of the data. MPD patients had significantly higher bite forces at 8.0, 8.5, 9, and 9.5 mm. Normal subjects had higher force values at 5.0 mm. There was no significant difference in mean maximal bite force between groups.
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PMID:Length-tension relations of the masticatory elevator muscles in normal subjects and pain dysfunction patients. 207 94

Inclusion body myositis is a rare and slowly progressive myositis associated with cytoplasmic inclusions and fibrillar nuclear material. These histopathologic findings are of unknown significance. The clinical presentation of IBM has marked similarities to that of chronic polymyositis with proximal greater than distal weakness and muscle wasting more pronounced in the lower than upper extremities. In contrast to polymyositis, however, relatively few individuals report neck flexor weakness or dysphagia. Corticosteroid treatment is usually ineffective. The clinical, histopathologic and electrophysiologic findings in a patient with IBM are presented. Of particular interest in this report is the detailed motor unit recruitment frequency data. A number of previous IBM reports fail to mention specific electrophysiologic data or present evidence suggestive of a possible combined neuropathic and myopathic disease. Recruitment intervals of 150 ms or greater in combination with decreased motor unit duration and amplitudes in the involved muscles imply a myopathic pathophysiology. These findings are discussed in relation to electrophysiologic data from previously reported cases.
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PMID:Inclusion body myositis. An electrophysiologic study. 215 41

We studied the immunologic correlates of disease activity and differences among subgroups of patients with idiopathic inflammatory myopathy by analysing phenotypic and activation marker expression on peripheral blood mononuclear cells (PBMC). Compared with controls, myositis patients with clinically active disease (n = 51) had significantly lower proportions of CD8+ cells and higher proportions of PBMC that expressed DR, CD3- DR, CD14- DR, interleukin-2 receptors, and the late T cell activation markers CD26 and TLiSA1. TLiSA1 expression, a marker for cytotoxic differentiation, correlated significantly with both clinical activity indices and serum levels of muscle-associated enzymes. In serial studies of seven patients, the proportion of PBMC expressing MHC class II antigen and late T cell activation markers decreased as myositis disease activity decreased, independent of type of therapy. Among the clinical subgroups, polymyositis (n = 21) and inclusion body myositis (n = 11) were virtually indistinguishable; dermatomyositis patients (n = 19) showed decreased proportions of CD3+DR+ and TLiSA1+ cells, and increased proportions of CD20+ and CD20+DR+ cells compared with the other two groups. Patients with autoantibodies to histidyl-tRNA synthetase (Jo-1 antigen, n = 11) had significantly lower proportions of CD3+ and CD4+ cells, lower CD4/CD8 ratios, and higher proportions of CD+ cells expressing CD20, compared with patients without anti-Jo-1 antibodies. These findings support the concept that activated lymphocytes, especially cells undergoing anamnestic responses and cytotoxic differentiation, are important in the pathogenesis of idiopathic myositis. Moreover, taken together with other studies, these data suggest that groups of patients segregated by clinical or autoantibody status have different mechanisms of systemic immune activation and immunopathology.
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PMID:Lymphocyte activation markers in idiopathic myositis: changes with disease activity and differences among clinical and autoantibody subgroups. 216 21

The authors report two male patients with inclusion body myositis (IBM) and review the features of this condition in the literature. This is an uncommon type of idiopathic myositis which involves males more often than females; it usually develops in elderly patients; its course is very slow; it is usually associated with distal weakness; neurophysiological studies show a mixed "myogenic" and "neurogenic" pattern; and it is usually unresponsive to corticosteroids. The diagnosis is basically made on the basis of the histological features, mainly consisting of vacuoles surrounded by a basophilic haze in histochemical stains of frozen tissue and, particularly, by the presence of characteristic microfilaments in ultrastructural studies.
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PMID:[Myositis with inclusion bodies: a little-known variety of idiopathic inflammatory myopathy]. 255 35

Idiopathic inflammatory myopathy, a category encompassing polymyositis, dermatomyositis, and a number of other disorders, is very uncommon, but has been the focus of intense study in the Arthritis and Rheumatism Branch of the National Institute of Arthritis and Musculoskeletal and Skin Diseases for the past several years. We describe the clinical picture, stressing the need for biopsy to ensure correct diagnosis. It is especially important to recognize the treatment-resistant variant, inclusion body myositis. The extraskeletal manifestations, particularly the cardiopulmonary, oropharyngeal, gastrointestinal, and endocrine involvement, are described. The cardiopulmonary involvement, especially interstitial lung disease, arrhythmias, and cardiac failure, may dominate the clinical picture. The known causes are varied, and include drugs, toxins, and some infectious agents, however, in most cases a cause cannot yet be identified. Circumstantial evidence suggests that picornaviruses may initiate some cases in humans, and a very similar disease in mice caused by a picornavirus is actively under study. Studies of autoantibodies and cellular immune function support a central role for disordered immunity in the pathogenesis. The myositis-specific autoantibodies, especially those directed at certain enzymes important in protein synthesis (the aminoacyl-transfer RNA synthetases), are found in a clinically distinct subset of patients. Although most patients respond initially to corticosteroids, cytotoxic drugs are sometimes added when steroid toxicity or refractoriness develops. We describe several newer therapies under study for such cases and outline future directions in research.
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PMID:Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders. 266 48


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