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1/196. Vanishing pulmonary hypertension in mixed connective tissue disease.

    A 29-year-old woman with mixed connective tissue disease presented with signs of progressive pulmonary hypertension. After admission to the hospital her condition worsened rapidly and she developed a cardiac arrest resistant to cardiopulmonary resuscitation. Therefore, emergency extracorporeal assist was performed. No pulmonary embolism was found. Right heart catheterisation showed severe pulmonary hypertension, which was treated with nitric oxide ventilation. She was weaned from the extracorporeal assist with high doses of inotropic agents. Because of suspicion of exacerbation of her underlying disease, which led to pulmonary hypertension, immunosuppressive treatment was started with high doses of corticosteroids and plasma exchange. This resulted in slow recovery over the next four weeks. Control echocardiography showed complete normalisation of cardiac function without signs of pulmonary hypertension. Two months after admission she was discharged from the hospital in good condition. ( info)

2/196. myocarditis of mixed connective tissue disease: favourable outcome after intravenous pulsed cyclophosphamide.

    A 30-year-old woman with mixed connective tissue disease was admitted with Wernicke's aphasia and progressive dyspnoea with chest pain. Multiple brain infarcts on a computed tomographic scan were compatible with a thromboembolic aetiology. echocardiography showed marked hypokinesia of the posterior wall, biventricular dilatation and a decreased left-ventricle ejection fraction (40%). A diagnosis of myocarditis was made on myocardial biopsies disclosing interstitial lymphocytic infiltrates and myocardial fibre necrosis. A treatment with steroids and monthly pulsed cyclophosphamide was introduced. The heart function rapidly improved as assessed by a left-ventricle ejection fraction of 55% and remained stable 17 months thereafter. ( info)

3/196. "Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis.

    We describe 3 patients with dermatomyositis who presented with flagellate erythema. This cutaneous eruption is characterized by erythematous linear lesions on the trunk and proximal extremities. Histologic examination of this eruption in one of our cases revealed an interface dermatitis. review of the literature and records of 183 patients with connective tissue diseases from our institution has shown that this peculiar eruption has been reported only in dermatomyositis. Because of the location of this eruption, we encourage the use of the term "centripetal flagellate erythema" to distinguish this entity from other linear eruptions seen in patients with connective tissue diseases. ( info)

4/196. A morbid course in a girl with mixed connective tissue disease.

    We describe an 18-year-old girl with a 13-year history of mixed connective tissue disease whose clinical course was unique: she ultimately developed end-stage kidney and bowel disease that led to a protracted morbid clinical course. We report this case to alert pediatric nephrologists to the importance of early recognition of possible intestinal disease in these patients. Unfortunately, no therapy is currently known to reverse the pathological process in the bowel, but multiorgan transplantation might be an option if the numerous medical complications of end-stage bowel disease can be successfully controlled. ( info)

5/196. Severe mixed connective tissue disease in a woman with pure gonadal dysgenesis: estrogens do not influence disease expression.

    mixed connective tissue disease (MCTD) is more prevalent in women during the child bearing years, suggesting that estrogens may play a role in disease expression. We describe a woman who developed MCTD despite pure gonadal dysgenesis, i.e., a disease associated with permanently very low plasma levels of estrogens. The onset of MCTD and subsequent life threatening disease course over 15 years occurred while she declined exogenous hormonal replacement therapy. Concurrent presence of estrogens is not necessary for onset, persistence, or exacerbation of severe MCTD. ( info)

6/196. methotrexate-induced papular eruption in patients with rheumatic diseases: a distinctive adverse cutaneous reaction produced by methotrexate in patients with collagen vascular diseases.

    BACKGROUND: In the past few years, low doses of methotrexate have been used for treatment of patients with rheumatoid arthritis and other collagen vascular diseases, mainly as an immunosuppressive and corticosteroid-sparing drug. Several cutaneous adverse reactions have been described in association with methotrexate therapy. OBJECTIVE: We describe the clinical and the histopathologic features of distinctive cutaneous lesions that appeared in 4 patients with acute bouts of collagen vascular diseases who were receiving methotrexate therapy. methods: We clinically and histopathologically evaluated cutaneous lesions caused by methotrexate therapy in 4 patients, 2 with systemic lupus erythematosus, 1 with rheumatoid arthritis, and 1 with Sharp syndrome. RESULTS: Clinically, lesions consisted of erythematous indurated papules most commonly located on proximal areas of the extremities. Histopathologic examination of these papules showed an inflammatory infiltrate mainly composed of histiocytes interstitially arranged between collagen bundles of the dermis, intermingled with few neutrophils. In some foci of deeper reticular dermis, small rosettes composed of clusters of histiocytes surrounding a thick central collagen bundle were seen. Cutaneous lesions showed a direct chronologic relationship with methotrexate therapy, and they disappeared when the drug was tapered or withdrawn and corticosteroids were increased. CONCLUSION: patients receiving low doses of methotrexate for acute bouts of collagen vascular diseases may experience characteristic cutaneous lesions with distinctive clinical and histopathologic findings shortly after methotrexate administration. We discuss the differential diagnosis with other dermatoses showing similar histopathologic findings that have been described in patients with collagen vascular diseases. ( info)

7/196. A case of Graves' disease associated with autoimmune hepatitis and mixed connective tissue disease.

    The patient was a woman of forty-eight. liver dysfunction was pointed out at the age of forty-five. She was admitted to hospital because of her hyperthyroidism. Her palmar skin was wet and her fingers were swollen like sausages. She had a diffuse and elastic hard goiter with a rough surface. The serum levels of free T3 (9.6 pg/mL) and free T4 (3.76 ng/dL) were high and that of TSH (0.11 microU/mL) was low. The activity of TSH-binding inhibitory immunoglobulin (TBII) was 89%. The uptake rate of 123I to the thyroid was 55.1% and the uptake pattern was nearly diffuse. The goiter was proved to contain several nodules by ultrasonography, but aspiration cytology showed no malignant cells. She was diagnosed to have Graves' disease with adenomatous goiter. She also had high ALT (34 IU/L) and gamma-globulin (1.97 g/dL). She had positive antinuclear antibody (speckled type), positive anti-ribosomal nuclear protein antibody, and positive LE cell phenomenon. The liver biopsy revealed mononuclear cell infiltration with fibrosis in the portal area. These data indicated that she also had autoimmune hepatitis (AIH) and mixed connective tissue disease (MCTD). The analysis of human leukocyte antigen (HLA) showed positive A11 which had been reported to relate to Graves' disease, and positive DR4 which had been reported to relate to AIH and MCTD. These results suggested that HLA would determine susceptibility to three distinct autoimmune diseases in this case. ( info)

8/196. Severe gastrointestinal bleeding in a uremic patient treated with estrogen-progesterone therapy.

    Gastrointestinal bleeding is a frequent complication in hemodialysis patients; angiodysplasia is a potential cause, with a higher incidence in uremic patients. We describe a case of severe anemia (Hemoglobin up to 3.5 g/dl) secondary to diffuse angiodysplastic lesions in a hemodialysis patient with mixed connective tissue disease. The case is characterised both by the severity of the clinical picture (extension and entity of angiodysplastic lesions, frequency of bleeding episodes) and by the patient's religious faith which made her reject blood transfusions. We underline the efficacy of estrogen-progesterone therapy in view of the modest results obtained with other therapeutic strategies on bleeding. ( info)

9/196. Low-dose cyclosporin for multiple colonic ulcers associated with mixed connective tissue disease.

    This report describes a patient with multiple colonic ulcers and mixed connective tissue disease. The histological findings of the colonic lesions showed vasculitis with T-cell infiltration, and the peripheral T cells were frequently in the activated phase of the cell cycle. In this patient, low-dose cyclosporin treatment (2.5 mg/kg/day) inhibited the T-cell activation in the peripheral lymphocytes and was very effective in the gastrointestinal disorder, which might be related to T-cell activation. This case suggests the possibility that even low-dose cyclosporin can exert a great influence on peripheral T cells and directly inhibit T-cell activation, thereby improving symptoms related to T-cell activation. ( info)

10/196. Klinefelter's syndrome accompanied by mixed connective tissue disease and diabetes mellitus.

    We report a rare case of Klinefelter's syndrome (KS) with mixed connective tissue disease (MCTD), diabetes mellitus (DM) and several endocrine disorders. A 57-year-old man presented with polyarthritis and tapering fingers with Raynaud's phenomenon on admission. In addition to a karyotype of 47, XXY, a marked restrictive change in respiratory functional test, a myogenic pattern in electromyogram, the positive tests for anti-RNP antibody indicated that this was a case of KS complicated with MCTD. The patients also presented DM with insulin resistance, hyperprolactinemia, slight primary hypothyroidism and hypoadrenocorticism. The mechanism for these coincidences remains to be elucidated. ( info)
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