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Query: UMLS:C0020538 (hypertension)
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The renal angiographic findings in our two patients with scleroderma and recent onset of hypertension included minor changes in the distal interlobar and arcuate arteries and a nephrogram displaying diffuse, spotty lucencies. Although the spotted nephrogram is occasionally seen in cases of severe nephrosclerosis, we believe that, in the absence of major arterial changes in the arcuate and distal interlobar arteries, it is virtually diagnostic of renal scleroderma.
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PMID:The spotted nephrogram of renal scleroderma. 17 39

Following effective long-term antihypertensive therapy, hemodialysis could be discontinued in 4 patients with malignant hypertension in view of improved renal function. Diagnoses included nephroangiosclerosis (2 cases), scleroderma and chronic glomerulonephritis. All patients had symptoms of hypertension or renal disease for at least one year prior to initiation of hemodialysis treatment. At the outset, blood pressure averaged 249 +/- 43/150 +/- 22 mm Hg (mean +/- SD) and all patients had grade IV hypertensive retinopathy. After 1 to 20 months strict blood pressure control, renal function had improved to such a degree that hemodialysis could be discontinued. One year later, blood pressure averaged 138 +/- 20/89 +/- 6 mm Hg and serum creatinine 3,2 +/- 1,2 mg/100 ml. These data suggest that in hypertensive patients with chronic renal failure, strict control of blood pressure is of the utmost importance whatever the severity and nature of the underlying renal disease.
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PMID:[Discontinuation of chronic hemodialysis due to improved kidney function caused by the control of arterial hypertension]. 39 20

The coexistence of organ-specific and nonorgan-specific autoimmune diseases is an interesting phenomenon. A 52-year-old woman was admitted with fever, general discomfort, polyarthritis, and Raynaud's phenomenon. Physical examination revealed a goiter of stony consistency, hardening, paleness, and atrophy of the skin on the face and upper limbs, and blood hypertension (180/110 mmHg). The biological data included leukopenia, moderate anemia, and a very high sedimentation rate. The latex test was positive (+++); LE cells positive (+); hypergammaglobulinemia (3.5 g); antinuclear antibodies, 1/1280 with an immunofluorescence granular pattern; antithyroid antibodies, 1/160. There was pulmonary, renal, and gastrointestinal involvement compatible with scleroderma, which was confirmed by skin biopsy. A thyroidectomy revealed the existence of a papillary carcinoma with thyroiditis. Responde to treatment with immunosuppressive agents, hypotensive drugs, and thyroid substitution therapy was initially good. The patient was readmitted 8 months later with general discomfort and a severe hyperproteinemia (10 g/100 ml), including 65 percent gammaglobulin and requiring various sessions of plasmapheresis. The patient was discharged, but died suddenly 4 months later. The association of lupus and scleroderma in this patient is discussed and the possibility of its being a mixed connective tissue disease is discarded. The association of this condition with Hashimoto's thyroiditis, and the latter with papillary carcinoma of the thyroid are analyzed. The peculiar features of this case are pointed out. The authors postulate that the cause of the sudden death was a vascular cerebral complication induced by the extreme hyperproteinemia.
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PMID:[Scleroderma with traces of disseminated lupus erythematosus associated with Hashimoto's thyroiditis and papillary carcinoma of the thyroid gland (author's transl)]. 58 90

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

Two cases of scleroderma are presented in which malignant hypertension developed abruptly, accompanied by rapidly progressive renal failure. The malignant hypertension was associated with high plasma renin levels and like other forms of hyperrenninemic hypertension and uremia, was refractory to both antihypertensive medication and extracellular fluid volume control with hemodialysis. Blood pressures became controllable only after bilateral nephrectomy was performed, and in each case resulted in a reversal of a rapidly progressive downhill course. Though both patients had multisystem involvement at the onset of renal failure, the non-renal manifestations of scleroderma have not progressed rapidly. Consequently, when patients with scleroderma develop malignant hypertension and uremia, aggressive therapy with dialysis and nephrectomy may significantly prolong survival.
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PMID:Malignant hypertension and uremia in scleroderma: efficacy of nephrectomy and hemodialysis. 88 14

The pathologic lesions of the kidney in scleroderma in many respects resemble those of malignant hypertension, perhaps even in the absence of comparable blood pressure elevation. Because the malignant vascular changes have been related to hyperreninemia, we measured plasma renin activity in 23 patients with scleroderma with or without hypertension and/or renal failure. We found that high renin levels in most cases shortly preceded or coincided with a phase of sudden deterioration of the disease, characterized by a rapidly progressive renal failure. The outcome of this phase was invariably fatal, except for two patients in whom bilateral nephrectomy successfully arrested the rapid downhill course. These findings suggest that an unexplained increase in circulating renin levels in an otherwise stable patient with scleroderma may be taken as a possible marker of imminent deterioration requiring close monitoring and immediate therapeutic intervention.
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PMID:Is elevated plasma renin activity of prognostic importance in progressive systemic sclerosis? 92 42

1. The anti-hypertensive effect of converting enzyme inhibition was evaluated in twenty-three hypertensive patients (seven renovascular, four essential, four malignant, one scleroderma, three chronic renal failure, four primary or idiopathic aldosteronism). 2. In sixteen patients a single injection (1--4 mg/kg) of the inhibitor produced an immediate anti-hypertensive effect, which lasted up to 16 h. In six patients the anti-hypertensive effect of the inhibitor was significantly improved after sodium depletion. 3. Plasma renin activities increased and plasma aldosterone concentrations decreased consistently except in idiopathic aldosteronism. 4. Converting enzyme inhibition provides a direct way of defining the degree of renin-dependency of the hypertension. Accordingly, it can be used diagnostically and for planning appropriate therapy. Therapeutically, it could be advantageous in hypertensive emergencies because of its safety, specificity and capacity to reduce aldosterone secretion.
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PMID:The use of angiotensin-converting enzyme inhibitor in the diagnosis and treatment of hypertension. 107 92

Small cutaneous vessels, obtained by ear lobe biopsies, were studied in 14 patients with various chronic nephritides and in 10 normal controls. The capillaries in the group of patients with nephritis were found to undergo two main changes: thickening of the adventitia reticularis and perivascular cellular infiltration in an inverse ratio. This infiltration was seen to be made up of mononuclear cells and an increased number of mast cells in various stages of degranulation. Changes in the basement membrane as seen by electron microscopy are not constant. All of the above changes were absent in the controls and are similar to what has been described in previous studies in both experimental and spontaneous pathologic conditions, such as experimental hypertension, diabetes mellitus, scleroderma, rheumatoid arthritis, etc. Small vessel involvement in chronic nephritides could be part of a process of diffuse microvascular damage that includes the kidneys or it may be related to hypertension or to the biochemical changes which follow uremic and pre-uremic states.
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PMID:Peripheral small vessel involvement in chronic nephritides. 118 6

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


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