1/41. A case of normotensive scleroderma renal crisis after high-dose methylprednisolone treatment.A 68-year-old male was admitted for interstitial pneumonia associated with scleroderma. High-dose methylprednisolone was administered for treatment of the pneumonitis. Two weeks later, anemia, thrombocytopenia and progressive increase in BUN, creatinine and LDH were observed. Although the blood pressure remained normotensive, renal biopsy showed thrombosis of the polar arterioles and glomerular capillaries. The affected interlobular artery included concentric intimal thickening and thrombosis in the lumen. Our findings suggested that the antecedent use of high-dose corticosteroids is involved in precipitating normotensive renal crisis. Corticosteroids should be used in low doses and with great caution in scleroderma patients.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/41. A case of basal cell carcinoma of the skin in a patient with systemic sclerosis.A 46-year-old man complained of pain and stiffness in both hands. Most of his fingertips were shortened, and Raynaud's phenomenon was evident. The skin on his face and hands was hard and taut. An ulcerating mass measuring 3.2 x 2.5 cm was found on his left infraorbital area. biopsy revealed basal cell carcinoma. Antinuclear antibody and anti-topoisomerase I antibody were strongly positive. A high-resolution computed tomography (CT) scan of the lungs revealed interstitial fibrosis. Esophageal manometry showed low lower esophageal sphincter pressure and gastroesophageal reflux. Under the diagnosis of basal cell carcinoma accompanying systemic sclerosis (SSc), surgical excision of the mass was performed, and immunosuppressant and vasodilator therapies were conducted.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/41. Pseudohypertension in a patient with diffuse scleroderma.Pseudohypertension is the artifactual elevation of blood pressure that occurs secondary to noncompressible blood vessels. It has been described in patients with uremia, diabetes mellitus, and severe atherosclerosis. If unrecognized, the condition may lead to inappropriate and potentially harmful therapy. We report a case of pseudohypertension in a 65-year-old man with diffuse scleroderma. His blood pressure as assessed by conventional sphygmomanometry was at least 240/135 to 145 mm Hg. Intra-arterial blood pressure was found to be 107/52 mm Hg. The severe rise in blood pressure as measured by sphygmomanometry led to the concern of scleroderma renal crisis and potentially harmful therapy. Intra-arterial pressure monitoring confirmed the presence of pseudohypertension, however. This is the first reported case of pseudohypertension in a patient with diffuse scleroderma.- - - - - - - - - - ranking = 5keywords = pressure (Clic here for more details about this article) |
4/41. Pulmonary-renal syndrome in systemic sclerosis.BACKGROUND AND OBJECTIVE: Renal failure, pulmonary hypertension, and interstitial lung disease are major causes of morbidity and mortality in systemic sclerosis (SSc). However, the concomitant occurrence of pulmonary hemorrhage associated with acute renal failure in SSc has been rarely described. The present study is the first analysis of pulmonary-renal syndrome in SSc. PATIENT AND methods: We present a 44-year-old woman with SSc who died of a fulminant course of acute renal failure associated with diffuse alveolar hemorrhage. We termed this uncommon and fatal complication of SSc scleroderma-pulmonary-renal syndrome (SPRS). A search of the English-written literature yielded reports of 10 additional similar cases. These patients, together with our present case, form the basis of the present analysis. RESULTS: The average age of the patients with SPRS was 46 years. The majority of the patients (80%) were women, and most had diffuse SSc. SPRS occurred an average of 6.4 years after disease onset and was associated with prior fibrosing alveolitis and/or D-penicillamine treatment. Interestingly, normotensive renal failure seems to characterize the scleroderma patients, because 9 of 11 (82%) had normal blood pressure. SPRS bears a poor prognosis: all of the 11 patients (100%) died within 12 months of admission. However, only 60% of the 5 patients for whom we have treatment data received corticosteroids. CONCLUSIONS: Pulmonary-renal syndrome is a rare but fatal complication of SSc. Because the treatment data are scarce and the prognosis is poor, aggressive treatment with pulse corticosteroids, cyclophosphamide, and possibly plasmapheresis is suggested.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/41. The watermelon rectum.This is the first reported case of rectal vascular ectasia in association with gastric antral vascular ectasia (watermelon stomach). The patient, a 39-year-old woman with systemic sclerosis, reported chronic gastrointestinal blood loss and iron deficiency anemia. She had aperistalsis of the distal esophagus, decreased lower esophageal sphincter pressure leading to severe gastroesophageal reflux, and dysmotility of the small intestine. diagnosis was made by endoscopic appearance of visible linear watermelon-like vascular stripes in the gastric antrum and rectum. histology confirmed the vascular nature of this disorder, showing dilated and thrombosed capillaries in the lamina propria. Gastric biopsy showed associated fibromuscular hyperplasia in the lamina propria. However, the colonic biopsy showed only focal hyalinization in the lamina propria. The patient was successfully treated using endoscopic bipolar electrocautery. Watermelon stomach has previously been described in association with systemic sclerosis. Based on the histopathologic similarity between rectal vascular ectasia, gastric antral vascular ectasia, and systemic sclerosis, we suggest that the rectal vascular lesion may represent a component of the gastrointestinal manifestations of systemic sclerosis.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/41. pulmonary veno-occlusive disease and the CREST variant of scleroderma.pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension. The authors describe a 48-year-old female with the calcinosis-Raynaud's phenomenon-Esophageal dysmotility-Sclerodactyly-Telangiectasia (CREST) variant of scleroderma who developed acute pulmonary hypertension with pulmonary infiltrates and a normal pulmonary capillary wedge pressure. At post mortem examination typical changes of pulmonary veno-occlusive disease were found. Similarities between this and other cases in the literature suggest a possible association between the CREST variant of scleroderma and pulmonary veno-occlusive disease.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
7/41. A case of pulmonary veno-occlusive disease associated with systemic sclerosis.The case of a patient with pulmonary veno-occlusive disease associated with systemic sclerosis is reported. The patient presented with progressive dyspnoea. echocardiography and cardiac catheterization study demonstrated right-sided heart failure. The CXR suggested pulmonary hypertension and interstitial pulmonary oedema. We suspected pulmonary veno-occlusive disease based on radiological and haemodynamic findings. Treatment with prednisolone resulted in a reduction in pulmonary arterial pressure and CXR findings improved 2 months later, but no further effect was observed. The patient died 7 months later and at autopsy the lungs showed prominent thickening of the interlobular septa and small branches of pulmonary veins showed intimal thickening.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/41. Treatment of secondary pulmonary hypertension with bosentan and its pharmacokinetic monitoring in ESRD.Pulmonary hypertension (PH) is a rare disease with a very poor prognosis. Certain pharmacologic approaches, which reduce pulmonary arterial pressure (PAP) and thereby prevent end-stage cardiopulmonary failure, have been used during recent years. endothelin-1 has been found to be involved in the pathogenesis of PH. The dual endothelin-receptor antagonist, bosentan, was recently approved for the treatment of pulmonary arterial hypertension. The drug is mainly cleared by hepatic elimination. Severe renal dysfunction does not affect the single-dose pharmacokinetics of bosentan to a clinically relevant extent. Whether renal replacement therapy, however, interferes with the pharmacokinetics of bosentan is unknown. The authors report on the use of bosentan (125 mg twice daily) and its pharmacokinetic monitoring in a 19-year-old woman with PH and end-stage renal disease secondary to scleroderma. Treatment was well tolerated without drug-specific adverse effects. After 12 months of treatment, pulmonary arterial pressure had normalized (48 mm Hg before start of treatment, 27 mm Hg at last follow-up). On the basis of analyzing samples from Genius-hemodialysis by a liquid chromatography assay with tandem mass spectrometry detection, the authors determined the bosentan dialysis clearance to be as low as 3.5 mL/min. Bosentan for the treatment of secondary PH seems to be safe as well as effective in end-stage renal disease patients and no adjustment of the bosentan dosing regimen appears necessary.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
9/41. A case of scleroderma spectrum disorder with anticentriole antibody and pulmonary hypertension.We describe the case of a patient with anticentriole antibody-positive scleroderma spectrum disorder (SSD) who developed pulmonary hypertension. A 54-year-old woman had noticed Raynaud's phenomenon and digital ulcers during the winter for the past 10 years. Although sclerodactyly was not present, digital ulcers, swelling of her hands, and phalangeal contracture were observed. An indirect immunofluorescence test revealed anticentriole antibody. Other SSc-specific antoantibodies were negative. An echocardiogram demonstrated that the estimated right ventricular systolic pressure was increased to 51 mmHg. She was diagnosed as SSD with pulmonary hypertension. This is the first case of SSD with anticentriole antibody to develop pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
10/41. Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports.We describe two systemic sclerosis (SSc) patients with isolated pulmonary arterial hypertension (PAH) who were given treatment with 50 mg oral sildenafil per day. We evaluated the efficacy of oral sildenafil for isolated PAH in SSc patients by direct assessment with cardiac catheterization before and 6 months after the initiation of sildenafil. Right-heart catheterization demonstrated decreased mean pulmonary artery pressure, decreased pulmonary vascular resistance, and increased cardiac output after treatment with sildenafil. brain natriuretic peptide levels were gradually decreased. The 6-min walking distance was greatly extended. Moreover, the physical conditions of both patients were much improved. We recognized no adverse events. We propose that oral sildenafil may be beneficial as a selective pulmonary vasodilator and as long-term treatment in SSc patients with isolated PAH.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
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