Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four selected patients with progressive systemic sclerosis showed deposition of immunoglobulins and complement in diseased renal arteries and arterioles. Although three patients had hypertension, in two of these malignant, the third patient did not have hypertension over a four year period. These findings suggest that immune complexes may be involved in the pathogenesis of some cases of progressive systemic sclerosis, the primary target being the vascular system.
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PMID:Immunohistochemical findings in the renal vascular lesions of progressive systemic sclerosis. 12 3

The renal lesions of a 5-year-old girl with progressive systemic sclerosis are described. The nephropathy was clinically characterised by moderate proteinuria, microscopic hematuria and transient hypertension. Light microscopy showed membranoproliferative glomerulonephritis of segmental character. On electron microscopy intramesangial, subendothelial and extramembranous glomerular deposits were observed. By immunofluorescence miscrosocpy deposit of IgG, Clq, C4, C3, C5, C8 and C9 in a predominantly subendothelial location were found in all glomeruli. Vascular lesions were of minor degree. Histological and immunohistological findings are compatible with an immune complex disease.
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PMID:Membranoproliferative glomerulonephritis in systemic sclerosis of childhood. 15 Jun 97

A 49 year old black female patient with progressive systemic sclerosis (scleroderma), multiple organ system involvement, uremia and malignant hypertension, was treated with maintenance hemodialysis and bilateral nephrectomy. Bilateral nephrectomy controlled refractory hypertension and appeared to alter the natural course of systemic sclerosis.
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PMID:Scleroderma kidney disease: a therapeutic approach with nephrectomy and hemodialysis. 60 57

Patients with progressive systemic sclerosis (PSS, scleroderma) exhibit a variety of immunologic abnormalities. To verify whether the renal vascular lesions of such patients might be mediated by an immunologic mechanism, kidney tissues of 16 patients with PSS were investigated by means of fluorescence, light, and electron microscopy; elution of tissue-bound antibody; and fixation of heterologous (guinea pig) complement. Controls consisted of 12 nonsclerodermatous patients with similar levels of hypertension with no evidence of associated immunologic abnormalities. Diffuse vascular deposits of immunoglobulins (predominantly IgM) and/or complement (predominantly Clq) were found in all 16 patients with PSS. These deposits were bound to the intima of intralobular and arcuate arteries which, by light microscopy, often exhibited typical fibromucinous alterations. Elution of antibody and heterologous complement fixation studies suggested that such reactants may represent the interaction of complement-fixing antibody and antigen. Electron microscopies studies demonstrated abundant fibrillar and ground substance material in the arterial intima but features of deposited (circulating) immune complexes were not found. By contrast, in the hypertensive (control) group, deposits of immunoglobulin (s) and/or complement were rare and, when present, were mostly confined to the arterioles. As judged by the results of elution and heterologous complement fixation, these arteriolar deposits appeared to represent trapped rather than specifically bound serum proteins. The possible signficance of these findings are discussed in relation to immunologic mechanisms which might be implicated in the pathogenesis of the renal vascular disease of PSS.
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PMID:Immunopathology of the renal vascular lesion of progressive systemic sclerosis (scleroderma). 64 27

A young woman with progressive systemic sclerosis (PSS) and renal failure who received a renal transplant from her mother suffered accelerated loss of allograft function in the absence of hyperacute rejection or severe hypertension. A biopsy specimen and pathologic examination of the transplanted organ showed a fluorescent antibody pattern and vascular changes that were indistinguishable from those in the patient's native kidneys. This clinical sequence is a departure from the relative success of renal transplantation in the few previously reported cases of PSS where it has been used as therapy for renal failure.
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PMID:Apparent recurrence of progressive systemic sclerosis in a renal allograft. 78 67

In order to evaluate the usefulness of echocardiography in detection and characterization of pulmonary arterial hypertension (PAH) in scleroderma patients, we performed M-mode, two-dimensional, and Doppler echocardiography in 71 patients with progressive systemic sclerosis (PSS) and related syndromes: mixed connective tissue disease (MCTD) and overlap syndromes. We estimated systolic pressure gradients across the tricuspid valve from the peak velocity of tricuspid regurgitation (TR) by color-flow guided continuous wave Doppler. TR velocities of analyzable quality for gradient estimation were obtained in 28 patients (39%), of whom 12 showed PAH (peak TR velocity > or = 2.5 m/sec). In comparison, analyzable TR was recorded in 19 (35%) of 55 patients with left-sided cardiac disease. None of the 12 with Doppler-estimated PAH showed left ventricular dilatation or decreased fractional shortening by M-mode and two-dimensional measurements. Nonsimultaneous cardiac catheterization confirmed PAH in 8 of 9 with Doppler-estimated PAH and in 3 of 12 without analyzable TR who had hemodynamic study. Doppler-estimated right ventricular systolic pressures (RVSP) correlated well with catheterization-measured pulmonary arterial systolic pressures (PASP) (< 0.01). Our results indicate that Doppler echocardiography is useful in detecting subclinical PAH and estimating PASP in patients with collagen vascular disease. The results of pulmonary function studies suggest that PAH in MCTD is mainly caused by pulmonary vasculopathy.
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PMID:Echocardiographic evaluation of pulmonary arterial hypertension in patients with progressive systemic sclerosis and related syndromes. 143 24

The paper reports the case of a 58-year-old woman suffering from mucosal and cutaneous lichen planus (LP) with esophageal and oral involvement, who had complained of dysphagia for approximately the past two years. The diagnosis of esophageal LP was made using esophagoscopy with mucosal biopsy. The differential diagnosis between LP and progressive systemic sclerosis is discussed; a cold-test, plethysmography of the limbs, an anti-ENA Sci-70 antibody assay, X-rays of soft tissues and esophagomanometry were performed for this purpose, and all proved normal. The presence of conditions implicated in the etiopathogenesis of LP (psychic disorders and the administration of psychoactive drugs, anti-hypertensives and FANS) were observed, together with an already known association with arterial hypertension. Altered hepatic function (with an increase in cholestasis enzymes) was also noted which is probably attributable to the prolonged use of psychoactive drugs (hepatic biopsy showed severe steatosis).
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PMID:[Mucocutaneous lichen planus with esophageal involvement. A clinical case]. 154 25

An abnormality of blood vessels was noted in a biopsy of a renal transplant. This took the form of apparent development of a new artery inside and concentric with the old, with elastic laminae and a muscular media, separated from the old internal elastic lamina by poorly cellular tissue. In a systematic study of material from another 119 renal transplants, 13 nephrectomy specimens for chronic pyelonephritis and hydronephrosis, 28 renal biopsies showing interstitial nephritis, and 18 renal biopsies showing small vessel vasculopathy of accelerated hypertensive type, similar arterial changes were seen in another 10 renal transplants that showed chronic vascular rejection, 1 case of chronic interstitial nephritis, and 3 cases of vasculopathy, 2 with accelerated hypertension and 1 with systemic sclerosis. One renal transplant also showed apparent development of new muscular veins inside old veins. Immunohistological study for smooth muscle actin confirmed that the apparently new arterial and venous structures contained smooth muscle cells. The arterial abnormality may be called arterialisation of intrarenal arteries. This change appears to be not rare, is distinctive, and has scarcely been previously recognised or reported as a response of intrarenal blood vessels to damage.
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PMID:Arteries and veins formed within renal vessels: a previously neglected observation. 156 60

A prospective study was performed to determine urinary albumin excretion in a group of 28 patients with systemic sclerosis. At the initial screen one patient had proteinuria and three had microalbuminuria. One year later these abnormalities persisted and in two of of the patients serum creatinine had significantly increased. In addition, a further three patients had developed microalbuminuria. In a control group of 10 patients with primary Raynaud's disease none had microalbuminuria. In a second control group of 16 patients with unrelated skin diseases one patient had microalbuminuria and one proteinuria, but both these patients had a history of hypertension. It is concluded that microalbuminuria is more common in patients with systemic sclerosis than in patients of equivalent age with other dermatological conditions but no vascular disease.
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PMID:Microalbuminuria in systemic sclerosis. 157 87

Patients with mixed connective tissue disease (MCTD) exhibit clinical features of systemic lupus erythematosus (SLE), progressive systemic sclerosis or scleroderma (PSS), and polymyositis-dermatomyositis (PM-DM). In their sera is an unusually high titer of a circulating antinuclear antibody with specificity for a nuclear ribonucleoprotein antigen. Pleuropulmonary manifestations are common in MCTD and the incidence varies from 20% to 85%. The pleuropulmonary complications include pleural effusion, interstitial pulmonary processes, pulmonary arterial hypertension (PAH), pulmonary vasculitis, pulmonary thromboembolic phenomena, aspiration pneumonia, and hypoventilatory failure. Pulmonary vascular pathology with progressive PAH and cor pulmonale is the most serious complication of MCTD. The pleuropulmonary manifestations in MCTD are similar to the respiratory problems well recorded in SLE, PSS, and PM-DM. Even though the pleuropulmonary complications are common in MCTD, they may remain clinically inapparent until fatal complications ensue.
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PMID:Lungs in mixed connective tissue disease. 157 26


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