Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A young woman with mixed connective tissue disease (MCTD) had an isolated trigeminal sensory neuropathy as an early manifestation of the disease. Raynaud phenomenon occurred almost synchronously with the onset of trigeminal neuropathy and was followed by myositis, diffuse hand swelling, synovitis, and increased ribonucleoprotein antibody. Mixed connective tissue disease has overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by high-titer antibody to ribonucleoprotein.
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PMID:Isolated trigeminal sensory neuropathy: early manifestation of mixed connective tissue disease. 21 41

Five children with Sharp syndrome are described presenting a non-erosive polyarthritis, hand and finger swelling, Raynaud phenomenon, myositis, dermatomyositis or SLE-like rash. Characteristic laboratory findings are, apart from elevated sedimentation rate, anemia and leucopenia, high titer IgM rheumatoid factors and antinuclear antibodies (ANA). The latter show speckled pattern, contain IgG, bind complement components and are directed against ribonuclease-sensitive nuclear antigens. All patients have antibodies against the so-called extractable nuclear antigens (Anti-ENA) and antibodies against ribonucleoproteins (Anti-RNP). Since children with Sharp syndrome rarely show renal or cerebral involvement, the prognosis seems to be fairly good.
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PMID:[Juvenile Sharp syndrome (mixed connective tissue disease)]. 30 64

Six patients with eosinophilic fasciitis are presented. This syndrome is characterized by indurative swellings of arms and legs, with rapidly progressing difficulties in extending elbows, wrists, and fingers, and often limited motion of shoulders and ankle joints. Pain when contracting muscles, and weakness of proximal muscles and hand grip are common features. The frequent occurrence of localized skin lesions has presented differential diagnostic difficulties to systemic sclerosis and to polymyositis. Visceral involvement and Raynaud's phenomena, however, are absent or mild. Blood eosinophilia, hypergammaglobulinemia, and unspecific signs of inflammation are found. Biopsy of muscle fascia gives characteristic histopathological findings of cell infiltrations (mostly mononuclear cells, frequently eosinophils) and vascular proliferation, in the middle layer of a thickened fascia. Skin changes are prevalent, but not conclusive for the diagnosis, and myositis in some patients might be difficult to distinguish from polymyositis. The importance of the clinical recognition of eosinophilic fasciitis and the inclusion of fascia in diagnostic muscle biopsies, is underlined.
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PMID:Eosinophilic fasciitis. Review and report of six cases. 53 17

Sera with an antinuclear immunofluorescence titre of 1/000 were taken consecutively from the diagnostic routine flow and examined for agglutinating antibodies against desoxyribonucleic acid (DNA) and extractable nuclear antigens (ENA). Passive haemagglutination tests with antigen-coated tanned erythrocytes were used and the specificity of the reactions was corroborated by testing against enzyme-treated cells. After the exclusion of the DNA-reacting 15%, three major groups and one minor could be distinguished on a serological basis. The largest group (41%) contained cases with a speckled immunofluorescence pattern and a RNase-trypsin sensitive agglutination reaction with ENA coated cells (sRNP). Nearly all cases of mixed connective tissue disease and scleroderma fell into this group which also contained 44% of the SLE cases. Symptomatically the group was characterized by remarkably high incidences of Raynaud's syndrome and myositis. The major group next in size comprised cases with a homogeneous immunofluorescence pattern but no reaction against DNA or ENA. Half of the cases within this group had the diagnosis SLE; they also constituted 42% of all SLE cases. The only other diagnosis of significant frequency within the group was unspecified collagenosis (23%). The symptomatology of the group was rather uncharacteristic, with the exception of the low incidence of Raynaud's syndrome. The third major group comprised cases with a speckled immunofluorescence pattern but no agglutination reaction against ENA or DNA. This group had a very high incidence of rheumatoid factor and also the highest incidence of visceral lesions among the groups. Yet the group contained only a small proportion (14%) of the SLE cases and the rheumatoid arthritis cases were about equally shared between this and the first group. The most common diagnosis in the group was unspecified collagenosis (40%). A fourth, small but homogeneous group contained cases with a speckled immunofluorescence pattern and a reaction with Sm antigen, i.e. an enzyme-resistant agglutination reaction with ENA. Six cases in this group had the diagnosis SLE. No diagnosis was available in two cases.
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PMID:Symptomatology and diagnosis in connective tissue disease. Antibodies to extractable ribonucleoprotein in 123 patients reacting with cell nuclei in the immunofluorescence test. 79 May 56

A new and distinct rheumatic disease with features of systemic lupus erythematosus (SLE), progressive systemic sclerosis and polymyositis is described. Typical symptons are Raynaud's syndrome, swollen hands and fingers, polyarthritis or polyarthralgia and myositis. Other symptoms are less common and include skin lesions, decreased pulmonary diffusing capacity, diminished esophageal motility, lymphadenopathy, and polyserositis. The diagnosis of mixed connective tissue disease (MCTD) can be established by demonstration of a high titer of antinuclear antibodies and antibodies against extractable nuclear antigen (anti-ENA). Both antibodies are directed against ribonuclease-sensitive antigen substrate, which permits differentiation of patients with MCTD from those with other rheumatic diseases. A relatively favourable prognosis and a good response to corticoid medication are further characteristics of this disease. Three personally observed patients with MCTD are described.
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PMID:[The Sharp syndrome ("mixed connective tissue disease")]. 108 43

Three patients with mixed connective tissue disease (MCTD) had clinical features that included a high incidence of Raynaud phenomenon, arthritis, myositis, and swollen hands. The diagnostic laboratory test result was the presence of high titers of antibody to extractable nuclear antigen. These antibody titers are notably reduced or abolished in patients with MCTD when the tanned red blood cells that are used in the test are pretreated with ribonuclease. Speckled antinuclear antibodies were present in all patients. Patients with MCTD have a low incidence of renal disease, are responsive to treatment with prednisone, and have a good prognosis.
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PMID:Mixed connective tissue disease. 108 51

Anti-Jo 1 antibodies define a subgroup of patients with polymyositis, which are characterized by the presence of extra-muscular involvement as pulmonary fibrosis, although Raynaud's phenomena, arthritis, tenosynovitis, pleuritis and pericarditis may also be present. Given the fact that extra-muscular signs may precede the clinical diagnosis of polymyositis, as in the two cases that we present here, these antibodies may help us to diagnose the disease before the onset of myositis.
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PMID:[Comments with respect to 2 patients with anti-Jo 1 antibodies]. 139 62

The clinical and laboratory features of 32 patients with anti-PM-Scl were studied. Patients with this rare autoantibody suffered from a homogenous overlap connective tissue disease defined by Raynaud phenomenon (32/32), features of scleroderma (31/32), arthritis (31/32, erosive in 9/32), myositis (28/32), lung restriction (25/32), calcinosis (15/32), and sicca (11/32). Significant renal and neurologic involvement was uncommon. All patients examined (22/22) had HLA-DR3, and 50% of these patients were homozygous. Our patients responded favorably to moderate immunosuppression and, with therapy, the disease generally has a good prognosis; over 50% of our series (17/32) remained well on minimal or no immunosuppression after a median follow-up of 8 years.
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PMID:The clinical and immunogenetic features of patients with autoantibodies to the nucleolar antigen PM-Scl. 143 28

We describe a 55-year-old woman with an 8-year history of mixed connective tissue disease (MCTD). Her condition was characterized by severe Raynaud's, swollen fingers, digital ulceration and gangrene, esophagitis, polyarthropathy, myositis and restrictive lung function. She consistently had antibodies to U1-RNP. Rheumatoid factor was present in low titer. She developed atlantoaxial subluxation, a feature seen commonly in rheumatoid arthritis, reported in spondyloarthropathy and a small number of patients with systemic lupus erythematosus but not described in MCTD.
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PMID:Atlantoaxial subluxation in a patient with mixed connective tissue disease. 183 16

51 patients with rheumatoid arthritis and high rheumatoid factors (mean titres 928) underwent examination for the demonstration of an extraarticular organ manifestation within the scope of the cooperation between the Department of Medicine of the Karl-Marx-University Leipzig and the Institute for Rheumatology of the Academy of Medical Sciences of the USSR in Moscow. The frequency of nodous rheumatism (about 60%) is comparable with the frequency of polyneuropathy. In 20% of the patients a systemic muscle atrophy, a hepatomegaly as well as a Raynaud-syndrome were stated. By means of skin biopsy in 28% perivascular infiltrates were found. Altogether in 6 patients (12%) a participation of the lungs and the pleura, respectively, could be proved. Only rarely a clinically manifest heart disease caused by the rheu-we we found an pericardial effusions in 3 cases. In systemic manifestation, such as myositis, participation of the eyes or vasculitis of the digital arteries with necrosis, were only sporadically to be established. Among 22 patients we found an pericardial effusions in 3 cases. In systemic manifestation in most cases increased parameters of activity were found. From the practical point of view apart from increased titers of the rheumatoid titres and circulating immune complexes (C1q-BT) increased concentrations of the C-reactive protein are prognostically significant. The presence of high rheumatoid factor titres alone as well as the isolated presence of rheumatic nodes must not always be connected with an unfavourable prognosis. When severe extraarticular manifestations are present a possibly early, intensive occasionally extracorporeal treatment is indicated.
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PMID:[Diagnostic and prognostic significance of so-called rheumatoid vasculitis--2]. 371 9


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