Cases reported "Scleroderma, Systemic"

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1/546. Systemic sclerosis revealing T-cell lymphoma.

    We describe a case of systemic sclerosis (SSc) occurring together with malignant lymphoma. A 43-year-old man, who had noticed sclerodactyly 1 month before consultation, was admitted for progressive skin sclerosis on his forearms and chest. SSc was diagnosed. Immediately after admission, skin sclerosis rapidly extended to the neck and trunk, and subcutaneous tumors developed on the neck, chest and back. skin sclerosis was prominent at the sites where subcutaneous tumors were present. The tumors were diagnosed as non-Hodgkin's lymphoma of T-cell phenotype derived from soft tissue. Following 4 cycles of chemotherapy, he had complete remission and the skin sclerosis remarkably improved. It is possible that cytokines produced by T-cell lymphoma cells were responsible for the development of skin sclerosis in this case.
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2/546. Evidence of cell-mediated cardiac myocyte injury involved in the heart failure of a patient with progressive systemic sclerosis.

    A 54-year-old woman with progressive systemic sclerosis (PSS) was admitted to hospital because of dyspnea and chest pain. Echocardiogram revealed diffuse hypokinesis of the left ventricle (ejection fraction 24%). methylprednisolone, heparin, and diuretics were administered, without benefit. anemia, thrombocytopenia, and renal dysfunction rapidly progressed, and she died of heart failure on the 14th hospital day. Immunohistochemical study of the myocardial tissue showed mild to moderate cell infiltration, mainly consisting of natural killer (NK) cells, macrophages, cytotoxic T lymphocytes (CTLs), and T helper cells. perforin, a cytolytic factor, was expressed in the infiltrating CTLs and NK cells, indicating that these cells were activated killer cells. Furthermore, human leukocyte antigen classes I and II, intercellular adhesion molecule-1, as well as costimulatory molecules B7-1, B7-2, and CD40, all of which are known not to be expressed in cardiac myocytes under normal conditions, were moderately to strongly expressed in cardiac myocytes. There was no detectable level of enterovirus genomes in the polymerase chain reaction products from the myocardial tissue of this patient. These findings strongly suggest that the infiltrating killer cells recognized cardiac myocytes as target cells and directly damaged them by releasing perforin. Enhanced expression of these antigens may have played an important role in the activation and cytotoxicity of the infiltrating killer cells. Absence of enterovirus genomes in the myocardial tissue may suggest that this autoimmune process is primarily induced by PSS.
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3/546. Acute hepatitis after riluzole administration.

    riluzole is a new drug representing the first active treatment for amyotrophic lateral sclerosis. We report the cases of two patients who developed acute hepatitis after taking riluzole at the recommended dose (100 mg daily) for 7 and 4 weeks, respectively. In both cases, liver histology showed hepatocellular damage with inflammatory infiltration and microvesicular steatosis without fibrosis. liver enzymes returned to normal 4 and 8 weeks, respectively, after riluzole withdrawal. In one case, the readministration of riluzole was followed by the relapse of hepatitis. These two observations strongly suggest that riluzole can induce acute hepatitis with associated hepatocellular damage and microvesicular steatosis. They also suggest that liver enzymes should be monitored during treatment with riluzole.
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4/546. MPO-ANCA-Positive crescentic glomerulonephritis: a distinct entity of scleroderma renal disease?

    Scleroderma renal crisis is characterized by intimal thickening of the afferent glomerular arterioles resulting in hypertension and fibrinoid necrosis of the capillary tuff. We report a 67-year-old man with long-standing systemic sclerosis who developed normotensive progressive renal failure, proteinuria, and a nephritic urinary sediment with serum myeloperoxidase-antineutrophil cytoplasmatic antibodies (MPO-ANCA). Renal biopsy showed pauci-immune crescentic glomerulonephritis but none of the typical vascular changes of scleroderma renal crisis. Because comparable cases have recently been reported from japan, normotensive MPO-ANCA-positive crescentic glomerulonephritis may form an entity of progressive renal failure in scleroderma.
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5/546. Marked and sustained improvement two years after autologous stem cell transplantation in a girl with systemic sclerosis.

    Autologous transplantation of hematopoietic stem cells has recently been proposed as a possible treatment for autoimmune diseases that are associated with a very severe prognosis. A 12-year-old girl who, since 4 years of age, had systemic sclerosis with progressive pulmonary involvement underwent autologous peripheral blood-derived stem cell transplantation (aPBSCT) using CD34 selection, cyclophosphamide, and the infusion of the monoclonal antibody CAMPATH-1G. Following transplantation, in the absence of any treatment other than symptomatic therapy, the patient's exertional dyspnea and alveolitis disappeared and she experienced a marked improvement in skin score, height velocity, and general well-being that has persisted 2 years after the transplantation procedure. Autologous PBSCT associated with the infusion of the monoclonal antibody CAMPATH-1G appears to be a useful therapy for otherwise intractable forms of progressive systemic sclerosis.
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6/546. diagnosis and physiopathology of laryngeal deposits in autoimmune disease.

    We report the clinical features and pathology of a previously unreported form of vocal fold disease seen in 4 patients, 3 of whom were diagnosed as autoimmune disease. The characteristic features of these lesions were found as bilateral transverse lesions in the mid portion of the vocal folds. The patients had dysphonia and diplophonia. Stroboscopic examinations showed 180-degree phase shifts between the anterior and posterior portion of the vocal folds. Case 1 had systemic lupus erythematosus, case 2 had Hashimoto's thyroiditis, and case 3 had progressive systemic sclerosis. Prior to the onset of hoarseness, autoimmune antibody titers were increased. These cases need differential diagnosis from vocal fold nodules and cysts. Two cases were recurrent after endoscopic surgery, 1 recurring 3 times. Glucocorticoid was effective in preventing the recurrence in the early phase. recurrence may have occurred because the surgical strategy was the same as that used for vocal fold nodules or cysts.
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7/546. Watermelon-stomach as a cause of chronic iron deficiency anemia in a patient with systemic sclerosis.

    Watermelon-stomach is a rare cause of gastrointestinal bleeding. There has been an increasing number of reports on the association of this lesion with diseases of the scleroderma group, causing chronic, sometimes severe gastrointestinal blood loss. The present report presents the case of a 75-year-old female with limited cutaneous systemic sclerosis and watermelon-stomach, which was the cause of her long-standing sideropenic anemia.
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8/546. Atypical esophageal diverticula associated with progressive systemic sclerosis.

    Five cases of unusual esophageal diverticula associated with progressive systemic sclerosis (scleroderma) or collagen vascular disease are presented. These wide-mouthed saccular diverticula were infrequently seen in a group of PSS patients with the typical motility disturbance of esophageal involvement and are reminiscent of the diverticula of the colon involved by PSS.
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9/546. Thrombotic thrombocytopenic purpura as an initial presentation of limited systemic sclerosis.

    Thrombotic thrombocytopenic purpura (TTP) is a rare complication of scleroderma (systemic sclerosis, SSc). In the 5 reports documenting the association of TTP and SSc, the TTP syndrome developed on a background of well established SSc. We describe a 51-year-old woman with a 5 month history of an evolving connective tissue disease syndrome who presented initially with TTP, followed 4 months later by limited cutaneous SSc and Raynaud's phenomenon.
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10/546. Takayasu's arteritis overlapping with systemic sclerosis.

    We describe an unusual case of overlap between Takayasu's arteritis (TA) and systemic sclerosis (SSc). TA has been found in association with several diseases, but not with SSc. To our knowledge this is the first case report of TA associated with SSc in the literature. It is possible that the expression of the two diseases in our patient was influenced by the presence of genetic factors predisposing to both TA and SSc.
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