1/17. role of lipids in the progression of renal disease in systemic lupus erythematosus patients.Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease marked by immune-complex mediated lesions in small blood vessels of various organs, especially the kidneys, although other factors may also be implicated in the pathogenesis of the disease. This article focuses on the role of lipids in the progression of glomerular, vascular and tubulo-interstitial lesions in two patients with lupus nephritis associated with pronounced hyper- and dyslipidemia. The pathogenesis of progressive glomerulosclerosis in both patients appears to be multifactorial. In addition to immune complex mediated lupus glomerulonephritis, progressively active in the first patient, severe nephrotic-range persistent proteinuria, arterial hypertension associated with hyperfiltration and hyperperfusion injuries and, to a minor extent, hyper- and dyslipidemia were observed. Immunological and non-immunological factors were shown to contribute to the development of tubulo-interstitial lesions. In both patients, in addition to local immune deposits, prominent tubulo-interstitial lipid deposits were probably causally related to both hyperlipidemia and the increased permeability of the glomerular filtration barrier. Tubular lesions were highlighted by intracytoplasmic lipid droplets as well as small cleft-like spaces found to be impacted in the tubular lumina. They were seen to penetrate tubular epithelial cells and eventually lodge in the interstitium, surrounded by mononuclear cell infiltrates and foam cells. In both patients, hypertensive angiopathy and extraglomerular vascular immune deposits were demonstrated. In addition, in the second patient, arteriolar and small arterial hyaline was found at the age of 28 years to be full of lipids and calcium precipitates, suggesting a peripheral atherosclerosis-like process which never occurs as a natural age-related condition. In conclusion, all parts of the nephron may be involved in the pathogenetic process causally related or influenced by hyper- or dyslipidemia. Associated either with endothelial cell injury and consequent insudation of lipids in the vascular walls, glomerular filtration barrier injury with hyperfiltration, or tubulo-interstitial lipid deposition, the mechanism of tissue damage by lipids in all parts of the nephron shares similarities with the pathogenesis of systemic atherosclerosis.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/17. An unusual case of systemic lupus erythematosus, lupus nephritis, and transient monoclonal gammopathy.A 23-year-old female patient suffering from active systemic lupus erythematosus (SLE) was treated with azathioprine (2 mg/kg per day) and prednisone. lupus nephritis class III with increasing proteinuria developed 28 months after disease onset. Treatment was switched to monthly pulse cyclophosphamide administered intravenously for 6 months (total dose 6.3 g), followed by oral azathioprine and low-dose prednisone to maintain partial remission. Eight months later, the patient developed an acute exacerbation of SLE with fever, proteinuria of 9.1 g/day, pancytopenia, and cerebral involvement with cephalgias and a grand mal seizure. She responded well to high-dose corticosteroids (500 mg prednisolone pulses over 3 days, i.v.) and was azathioprine switched from to methotrexate (12.5-15 mg per week). Under this treatment, lupus activity gradually decreased and the patient felt well again. Five years after the initial diagnosis of SLE, a rapidly increasing immunoglobulin g-kappa type (IgG-kappa) monoclonal gammopathy developed, reaching a maximal serum paraprotein concentration of 73.5 g/l. bone marrow biopsy revealed 15% of moderately abnormal, highly differentiated plasma cells arranged in small clusters and expressing IgG-kappa. No bony lesions were detectable on skeletal radiographs. Pulses of dexamethasone (40 mg) were administered and led to a transient decrease of paraproteinemia to a minimum of 31.9 g/l, followed by an increase to 62 g/l. At that point, high-dose chemotherapy supported by autologous stem cell transplantation was considered. Due to an intermittent pneumococcal septicemia, methotrexate was discontinued and dexamethasone was replaced by 5-10 mg cloprednol. At this point, totally unexpectedly, the paraprotein decreased spontaneously without any further cytostatic treatment and was no longer detectable 1 year later. Concomitantly, plasma cell counts in bone marrow biopsies fell to below 5%. As SLE remained inactive, the patient became pregnant and gave birth to a healthy child. During late pregnancy, SLE activity flared up with rising proteinuria and blood pressure. Therefore, after delivery, cyclophosphamide (100 mg/day, orally) was readministered for 4 months, resulting in an improvement of kidney function with stable proteinuria of 1-2 g/l to date. paraproteins are no longer detectable. In conclusion, this case report documents the rare event of transient paraproteinemia in a patient with SLE. A self-limiting regulatory defect in the control of a terminally differentiated B-cell clone may be the origin of this phenomenon.- - - - - - - - - - ranking = 1.1073100248312keywords = blood pressure, pressure (Clic here for more details about this article) |
3/17. Clinicopathological conference: an 11-year-old boy with recurrent infections, hypertension, skin rash, and nephritic syndrome.In this clinicopathological conference, an 11-year old boy who presented with recurrent pyogenic infections, hypertension, malar rash, various skin lesions and nephritic syndrome since five years of age is discussed. He was hospitalized for clinical investigation with skin and renal biopsies at 10 years of age. Using clinicopathological data obtained from his last admission, a clinical diagnosis was reached, and the disorders of the complement system causing patients to show these signs or symptoms are emphasized. At the end of the discussion, a clinicopathological correlation is given for making the diagnosis.- - - - - - - - - - ranking = 5keywords = hypertension (Clic here for more details about this article) |
4/17. Remission achieved in chronic nephropathy by a multidrug approach targeted at urinary protein excretion.Regardless of the pattern of renal involvement, increased urinary protein excretion rate is the best independent predictor of progression of chronic nephropathies and short-term reduction in proteinuria has been reported to be renoprotective in the long term. Despite such evidence, however, the therapeutic target in renoprotection is almost exclusively on blood pressure control. We report the clinical course of a patient with chronic nephropathy after the institution of a multidrug treatment titrated against urinary protein excretion to achieve renoprotection. The present findings indicate that adjusting renoprotective therapy according to the decline in protein excretion in a multidrug strategy may stabilize or even reverse renal disease progression. This approach should be formally explored in prospective studies.- - - - - - - - - - ranking = 1.1073100248312keywords = blood pressure, pressure (Clic here for more details about this article) |
5/17. thyroiditis induced by interferon in dialysis.We describe a dialysis patient who acquired acute hepatitis c infection. Her primary renal disease was systemic lupus erythromatosis. She was having goitre but clinically euthyroid and her thyroid function test was normal. To avoid long term complications of hepatitis c we elected to treat her with Interferon 3 million units subcutaneously 3 times a week. During treatment she developed some transient side effects initially which subsided but later she felt pressure symptoms around her neck. When we checked her TSH and thyroid antibodies these were elevated. Though this could be related to HCV, rarely, but we think the thyroid change is mostly related to Interferon. Some possible explanation of the effect of Interferon on thyroid have been reviewed and we think patients getting such drugs should be under close monitoring to avoid permanent thyroid dysfunction.- - - - - - - - - - ranking = 0.063693290491478keywords = pressure (Clic here for more details about this article) |
6/17. Simultaneous malignant hypertension and cardiac tamponade.Malignant hypertension and cardiac tamponade are uncommon but potentially life-threatening medical emergencies. Both conditions may be associated with collagen vascular diseases, such as systemic lupus erythematosus. We report a case of acute cardiac tamponade associated with malignant hypertension secondary to lupus nephritis. Immediately after pericardiocentesis, blood pressure declined substantially. Although malignant hypertension is seen with modest frequency in patients with systemic lupus erythematosus, true cardiac tamponade is a less common complication of lupus serositis. Acute, simultaneous presentation of both life-threatening entities and the hemodynamic course have not been described.- - - - - - - - - - ranking = 8.1073100248312keywords = blood pressure, hypertension, pressure (Clic here for more details about this article) |
7/17. Lupus vasculopathy combined with acute renal failure in lupus nephritis.Several risk factors have been associated with the prognosis of lupus nephritis. However, few studies have focused on renal vascular lesions (such as thrombi due to immune complexes) as a prognostic factor in this disease. Here we present a case of systemic lupus erythematosus (SLE) in a 12-year-old girl who exhibited acute renal failure and severe hypertension on admission. Renal pathology findings included diffuse proliferative glomerulonephritis (class IVb) and lupus vasculopathy (LV) with immune complex deposition within glomerular capillaries and the preglomerular arteriolar lumen. Her clinical condition deteriorated rapidly, even after cyclophosphamide and methylprednisolone pulse therapy. It improved after 5 days of plasmapheresis and remained stable for up to 6 months under regular treatment. We suggest that renal biopsy performed early in SLE patients with renal involvement should be studied carefully for the presence of vascular lesions. Additionally, plasmapheresis can be considered in patients with LV refractory to other modalities of therapy.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
8/17. Interpretation of cardiac pathophysiology from pressure waveform analysis: pericardial compressive hemodynamics, Part II.Constrictive physiology characteristically alters atrial and ventricular waveforms. Normal pressure and flow responses to inspiration are blocked or reversed. The impairment of early diastolic filling is the common feature of restrictive myocardial, as well as diseased pericardial, processes. Low pressure tamponade can limit cardiac output, but may be difficult to detect by clinical signs alone. Examination of the pressure waveforms may provide clues to the diagnosis of constrictive physiology in these patients.- - - - - - - - - - ranking = 0.44585303344034keywords = pressure (Clic here for more details about this article) |
9/17. Lupus vasculopathy combined with renal infarction: unusual manifestation of lupus nephritis.A 30-year-old woman with a 10-year history of systemic lupus erythematosus was admitted to our hospital because of the onset of hypertension and renal dysfunction. Renal arteriogram revealed multiple renal infarctions, and cut-off or tapering-stenosis in the interlobular arteries. Renal biopsy showed concentric intimal thickening with narrowed lumen in some arterioles and deposition of IgG/IgM/complement 3 in the wall of arteriole without any active lesions or immune complex deposition in glomeruli. The present case indicates that this type of renal vascular lesion in lupus nephritis, lupus vasculopathy, may cause renal infarction and the loss of renal function without active glomerular lesions.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
10/17. stevens-johnson syndrome induced by mizoribine in a patient with systemic lupus erythematosus.A 32-year-old Japanese woman, who had a treatment history of systemic lupus erythematosus (SLE) with lupus nephritis world health organization class IV for 11 months, visited our hospital due to fever, facial erythema, and erosion of the oral cavity on November 10, 2003. Her mucosal erosion and facial skin erythema progressed over the following week, and stevens-johnson syndrome was diagnosed due to pathological findings of the skin. Among the administrated drugs, only mizoribine, started 6 months earlier, produced a positive reaction in the drug lymphocyte stimulation test. Increased prednisolone and high dose intravenous gamma-globulin were given successfully. cyclosporine at 50 mg was administered to control the SLE, followed by an increase to 100 mg on January 7, 2004. She suffered from abdominal pain, blindness, and convulsion on January 9. The magnetic resonance image of her brain prompted a diagnosis of reversible posterior leukoencephalopathy syndrome. After withdrawal of cyclosporine and control of hypertension, symptoms disappeared rapidly. cyclophosphamide pulse therapy was successfully administrated to control lupus nephritis. This is the first report describing the relationship between stevens-johnson syndrome and mizoribine. Although the use of mizoribine is thought to be safe, careful observation is necessary.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
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