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

Two cases of proliferative myositis, four cases of proliferative fasciitis and one mixed form of proliferative myositis and fasciitis have been analyzed in terms of cell differentiation and DNA content. Light microscopically, the lesions were characterized by a mixture of proliferating spindle-shaped cells and uni-, bi- or occasionally multinucleated ganglion cell-like cells. The spindle cells showed ultrastructural features and immunohistochemical properties, including an immunoreactivity for smooth muscle-specific actin, indicative of a myofibroblastic differentiation. The ganglion cell-like cells displayed some resemblance to active osteoblasts ultrastructurally and differed immunohistochemically from the spindle cells by being non-immunoreactive for smooth muscle-specific actin. None of the two cell types showed immunoreactivity for desmin, myoglobin or factor VIII RAG. It is suggested that the two cell types represent different lines of cell differentiation. The cytologic features in smears, as seen in two cases of proliferative fasciitis and one case of proliferative myositis, are considered to be characteristic of these lesions and to permit the diagnosis to be made by fine-needle aspiration. In two of the cases, the lesion was diagnosed only cytologically and thereafter disappeared spontaneously within a month. Cytometric DNA measurements, using two different image analysis systems on Feulgen-stained sections and smears, revealed a "diploid" spindle-shaped cell population with a variable proportion of cells with scattered DNA values. The ganglion cell-like cells differed from the spindle cells by having a broad DNA peak in the diploid region and additional peaks in the tetraploid region, as well as a higher proportion of cells with scattered DNA values compared with those of the spindle-shaped cells. The results of the quantitative DNA analysis are well in keeping with the benign and proliferative nature of these lesions. However, with the technique used here, quantitative DNA analysis, does not distinguish these pseudosarcomatous fibrous lesions from diploid and tetraploid soft tissue sarcomas.
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PMID:Proliferative myositis and fasciitis. A light and electron microscopic, cytologic, DNA-cytometric and immunohistochemical study. 158 81

We studied four cases of proliferative myositis by the avidin-biotin-peroxidase complex technique, using a panel of 12 antibodies, and by electron microscopy. The aim was to clarify the nature of their constituent cells, specifically the giant ganglion-like cells and spindle cells, and to discuss the implications for histogenesis. In all cases, both cell types showed positive cytoplasmic staining with antibodies to vimentin, actin (C4), and alpha-smooth muscle actin-1, but in only one was there positive staining with desmin. No staining was obtained with factor XIIIa, muramidase, alpha-1-antitrypsin, myoglobin, S-100 protein, CAM 5.2, factor VIII-related antigen, or neuron-specific enolase. By electron microscopy, both types of cells were seen to contain numerous thin filaments, dense bodies, coated and pinocytotic vesicles, active and dilated rough endoplasmic reticulum, few microvilli, and incomplete desmosomal junctions. Our findings imply a myofibroblastic nature for the giant ganglion-like cells and spindle cells. Our observations also support the hypothesis that they are derived from a pericytic cell.
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PMID:Proliferative myositis. An immunohistochemical and ultrastructural study. 205 61

During the last several years, there has been increasing interest in the use of intravenous immune globulin for immunosuppression. Although the mechanism(s) of action remains to be delineated, immune globulin therapy has been shown to be effective in some antibody-mediated disorders. In Rh disease, antibody-induced cytopenias, myasthenia gravis, and the clotting disorder associated with anti-factor VIII antibody, intravenous immune globulin has had therapeutic benefit. It was of interest that intravenous immune globulin may partially ameliorate the tendency toward dilation of the coronary vessels in Kawasaki disease. If this disorder represents a vasculitis of the small feeding vessels of the coronary arteries, could this agent influence other forms of collagen vascular disease? Pilot studies in dermatomyositis and polymyositis and systemic juvenile rheumatoid arthritis indicate benefit from intravenous immune globulin therapy. In these studies we used a high-dose protocol consisting of 1 gm/kg/day of immune globulin for 2 days every 4 weeks. In patients with either myositis or systemic juvenile rheumatoid arthritis, beneficial effects were seen. In the former group of patients, increased proximal muscle strength and reduction in creatine kinase levels were observed. In the latter group of patients a marked reduction in systemic features was observed. The amount of corticosteroids required was reduced in both groups of patients. These studies indicate the potential for intravenous immune globulin in collagen vascular disorders and the need for carefully controlled trials of this form of therapy.
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PMID:The use of intravenous immune globulin in collagen vascular disorders: a potentially new modality of therapy. 279 2

Myositis in childhood is characterized by elevated serum levels of muscle-derived enzymes, proximal symmetrical muscle weakness, abnormal EMG findings, and a muscle biopsy, which frequently documents an inflammatory process. In the pediatric age group, JDMS, which has characteristic cutaneous involvement in addition to myositis, is much more common than PM and is more common among female patients. With the use of steroids, mortality has been reduced from 33 per cent to 7 per cent. The development of calcifications can be the most debilitating consequence of JDMS. It is our premise that JDMS is a distinct disease entity and that the increase in HLA-B8 and DR3 in JDMS suggests that genetic background may predispose to disease development. There are conflicting data concerning immunologic abnormalities in JDMS, but there appears to be impairment of natural killing and evidence of complement activation. Results of tests for ANA frequently are positive in JDMS, but Jo-1 antibody, found in some adults with PM, has not been found in JDMS. Most newly diagnosed JDMS patients have antibodies to coxsackie B that may be related to the pathogenesis of this disease. Specific pathologic findings of endothelial cells containing reticulotubular inclusions are associated with small vessel occlusion, subsequent obliteration, and increased factor VIII levels in clinically active disease. In addition to physical therapy, steroids are used most frequently, but other immunosuppressive agents and plasmapheresis have been tried in severely ill children. Rigorous evaluation of the efficacy of these modalities is needed.
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PMID:Juvenile dermatomyositis. 353 4

Iliopsoas haematoma is a well-recognized complication of haemophilia, and is considered as potentially life threatening and significantly associated with morbidity. There are only rare reports on the incidence or outcomes of iliopsoas bleeding since the widespread usage of modern therapies for haemophilia. In this study, we present the experience of Ege University Haemophilia Centre with iliopsoas bleeding and its early and late complications. We reviewed 146 haemophiliacs (106 haemophilia A, 40 haemophilia B). Fourteen iliopsoas bleeding episodes were identified in eight haemophiliacs. Three patients (37%) had one episode, four (50%) had two episodes and one (13%) had three episodes. Two patients had a high titre inhibitor against factor VIII and accounted for three bleeding episodes (21%). We did not observe any episodes in six patients receiving prophylaxis. Iliopsoas haematomas were confirmed by ultrasonography in all patients. In physical examination, the most common symptoms were thigh, hip and groin pain, hip flexion contracture, abdominal tenderness and paraesthesia in the distribution of the femoral nerve. The mean duration of therapy with clotting factor concentrate was 7.8 +/- 1.6 days. The mean duration of hospitalization was 4.8 +/- 2.0 days. All patients started to receive a physical therapy program 6.0 +/- 2.4 days after the initiation of haemostatic therapy which lasted 20.0 +/- 6.0 days. Ultrasonographic findings related to iliopsoas haematoma disappeared in all patients within 3 months from the initial episodes. Only in one patient with mild haemophilia A, heterotopic bone formation (myositis ossificans) developed as a long-term complication. In conclusion, pain around the hip joint, femoral neuropathy and hip flexion contracture in a patient with haemophilia should alert the physician to the possibility of an iliopsoas haematoma. Early and effective factor replacement therapy is essential in the prevention of the complications.
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PMID:Iliopsoas haemorrhage in patients with haemophilia: results from one centre. 1612 89

Inherited bleeding disorders (IBDs) are caused by quantitative and qualitative alterations of either platelets or plasma proteins involved in coagulation and fibrinolysis. Hemophilias are the most frequent IBDs; however, accumulated data from various studies reported that von Willebrand disease (VWD) is the most common cause of IBD, with an increased incidence of platelet function defects, mostly due to the increased rate of consanguinity in some communities. VWD is an inherited disorder of homeostasis due to quantitative or qualitative defect of von Willebrand factor. Data on its epidemiology and impact in developing countries are limited. The objective of this study was to assess the local prevalence of some IBD and establish the clinical and historical variables that are predictive for those bleeding disorders in pediatrics. The study involved 43 children with various bleeding manifestations and 15 age- and sex-matched controls, recruited from the Pediatrics Hematology Clinic at the National Research Centre, Sausan Mubarek children's hospital in Cairo, Egypt and the King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. Hematological profile included platelet counts and function, prothrombin time, partial thromboplastin time, factor VIII antigen and its activity, factor IX antigen and its activity, von Willebrand factor and its activity assayed with multimeric analysis. A total of 12 (27.9%) children had VWD, 11 (25.5%) had hemophilia A, three (7%) had hemophilia B, seven (16.3%) had platelet dysfunction and 10 (23.3%) had bleeding with undiagnosed cause. Two of the VWD cases had type I, three had type II, four had type III and one case appeared to have type IIM and another to have IIB VWD. Bruising and epistaxis were the main symptoms in all children with VWD The majority of platelet dysfunction disorders were diagnosed as Glanzmann's thrombasthenia. VWD and Glanzmann's thrombasthenia should be considered not uncommon causes of IBDs in children in Egypt and Kingdom of Saudi Arabia. Routine hematological screening should be mandatory in children with positive family history of bruising and bleeding as a predictor for IBD.
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PMID:The spectrum of inherited bleeding disorders in pediatrics. 1900 43