11/622. 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.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
12/622. scleromyxedema.scleromyxedema is a sclerotic variant of papular mucinosis, in which lichenoid papules and scleroderma-like features are present. We describe a patient with scleromyxedema with IgG type lambda chain paraprotein, a systemic sclerosis-like illness, and myositis. The patient's serum contained Scl 70 antibodies, characteristic of scleroderma. electromyography showed signs of acute myositis and the creatine phosphokinase (CPK) level was elevated. Multiply passaged fibroblasts from the patient's skin lesions showed altered growth response in vitro. The patient was treated with cyclosporin (4 mg/kg/day) with improvement.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
13/622. Spinal anaesthesia for caesarean section in a patient with systemic sclerosis.We describe the management of a diabetic primigravid woman with systemic sclerosis and thrombocytopaenia who required Caesarean section for pre-eclampsia. This was performed successfully under spinal anaesthesia.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
14/622. Acute effects of nebulised epoprostenol in pulmonary hypertension due to systemic sclerosis.Pulmonary hypertension often has a lethal outcome in systemic sclerosis and the treatment is challenging. epoprostenol is a potent pulmonary vasodilator and its efficacy has been demonstrated when delivered by the intravenous and aerosolized routes. We report the haemodynamic and functional benefits of epoprostenol administered by inhalation to a spontaneously breathing patient with partially reversible pulmonary hypertension due to systemic sclerosis. Aerosolized epoprostenol, equivalent to the maximum tolerated intravenous dose (31.2 micrograms), produced a 58% fall in pulmonary vascular resistance, increased the cardiac output by 42% and improved functional performance by one MET (3.5 ml kg-1 min-1 of oxygen uptake) without any significant side-effects. Selective distribution of epoprostenol by the inhaled route may offer a new strategy for treatment of pulmonary hypertension.- - - - - - - - - - ranking = 1.2000003271402keywords = sclerosis, ms (Clic here for more details about this article) |
15/622. Imaging of fibrosis of the oral mucosa by 20 MHz sonography.OBJECTIVES: To compare the ultrasonographic (US) appearances of the oral mucosa in health with patients with systemic sclerosis (SSc). methods: An innovative 20 MHz US scanner was used to examine the lips, cheeks and oral vestibule of ten healthy persons and ten patients with SSc. The clinical, US and histopathological features of one patient with a fibro-epithelial polyp of the buccal mucosa are reported in detail. RESULTS: Two patients with SSc displayed increased echogenicity due to fibrotic deposits. A similar echo pattern was seen in the case of the histopathologically verified fibro-epithelial polyp of the buccal mucosa. CONCLUSION: 20 MHz sonography may be suitable as a non-invasive tool for evaluation of fibrosis of the oral mucosa.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
16/622. Global improvement of systemic scleroderma under long-term administration of octreotide.octreotide has proven to be effective for the treatment of intestinal dysmotility in patients with scleroderma in short-term administration. We report a global improvement of scleroderma manifestations under long-term administration of octreotide. A 53-year-old black woman was diagnosed with a four-year history of progressive and severe systemic scleroderma, with diffuse skin sclerosis, myositic involvement, impaired carbon monoxide transfer factor (57% of the predicted normal value and severe digestive involvement with pseudo-obstruction and bacterial overgrowth into the intestinal lumen). After one month of octreotide (75 mug/d), oral feeding was restarted and weight gain of 6.5 kg was achieved. After 8 months of treatment, normal weight was obtained and skin induration was spectacularly reduced and pigmentation returned to a normal state. dyspnea disappeared and physical activity was quite normal. octreotide effects on intestinal transit are unclear and may be secondary to immunomodulation or neurotransmission effects. Extradigestive effects of octreotide in scleroderma have not been studied. This report suggests that long-term administration of octreotide may be beneficial in the treatment of patients with systemic scleroderma. Long-term trials are required to confirm these preliminary results.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
17/622. Polyneuropathy as initial manifestation of systemic sclerosis (scleroderma).We report the first case of a young female patient who developed a sensory-motor polyneuropathy, without any skin or internal involvement characteristic of SSc, but with a serological positivity of antitopoisomerase I antibodies. After 4 years she developed a rapid skin tightening with lung involvement, in a full blown picture of the diffuse subset of SSc. The case suggests that the peripheral nervous system deserves more attention, in particular in the earliest phase of SSc.- - - - - - - - - - ranking = 0.8keywords = sclerosis (Clic here for more details about this article) |
18/622. scleromyxedema is a scleroderma-like disorder and not a coexistance of scleroderma with papular mucinosis.We present four cases of scleromyxedema with scleroderma-like cutaneous changes mimicking systemic sclerosis and stress the importance of their differentiation from true scleroderma. scleromyxedema should be recognized as an entity since it differs from scleroderma in the pathogenesis, histopathology of cutaneous lesions, type of visceral involvement (if present), frequent association with paraproteinemia, the course and prognosis.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
19/622. Immunohistological study endothelin-1 and endothelin-A and B receptors in two patients with scleroderma renal crisis.Scleroderma renal crisis (SRC) developed in two patients with systemic sclerosis (SSc) and they died from respiratory failure. Findings on autopsy revealed congestion and oedema in both lungs and intimal thickening of the small renal arteries in both patients. Immunohistological investigations showed positive staining of anti-human endothelin (ET)-1 in the media of the small renal arteries and ET-B receptor in the medial smooth muscle of the small renal arteries. This observation suggests an important pathophysiological role of ET-1 in the development of SRC in some patients with SSc.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
20/622. De novo systemic sclerosis after radiotherapy: a report of 3 cases.We describe 3 patients in whom the onset of systemic scleroderma occurred shortly after ionizing irradiation for nasopharyngeal or breast carcinoma. This relationship has been described rarely as has the exacerbation of a preexisting scleroderma after irradiation. This gives indications for direction of studies.- - - - - - - - - - ranking = 0.8keywords = sclerosis (Clic here for more details about this article) |
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