Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Plasma concentrations of the recently isolated potent vasoconstrictory peptide endothelin were measured in 382 patients. The investigations were performed by means of a sensitive radioimmunoassay specific for Endothelin-1, 2. The results from 110 healthy volunteers displayed a normal range of 44.67 +/- 3.51 pg/ml. Significantly raised levels were found in 33 patients with chronic end-stage renal failure both before and after hemodialysis. In contrast, 35 patients with compensated renal insufficiency did not differ from the normals. Sixty-five patients after kidney transplantation revealed significantly elevated levels, as did 27 patients with acute myocardial infarction, 8 after coronary bypass surgery, and 5 with liver cirrhosis. The mean values of 27 patients with untreated hypertension, 22 with secondary hypertension, of various causes and 16 with coronary artery disease were comparable to the normal population. The values were significantly decreased in 9 pregnant women with hypertension and proteinuria. A marked decline was found in 5 patients with systemic lupus erythematodes, while 20 patients with rheumatoid arthritis demonstrated only a slight decrease. The pathophysiological role of endothelin as a local or circulating hormone in regulating systemic blood pressure or release of other hormones remains to be determined.
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PMID:[Plasma endothelin in normal probands and patients with nephrologic-rheumatologic and cardiovascular diseases]. 221 2

A 23-year-old African American woman with a past medical history of systemic lupus erythematous (SLE), secondary hypertension, and end stage renal disease (ESRD) on hemodialysis for eight years was stable until she developed symptomatic severe mitral regurgitation with preserved ejection fraction. She underwent a bioprosthetic mitral valve replacement (MVR) at outside hospital. However, within a year of her surgery, she presented to our hospital with NYHA class IV symptoms. She was treated for heart failure but in view of her persistent symptoms and low EF was considered for heart and kidney transplant. This was a challenge in view of her history of lupus. We presumed that her stenosis of bioprosthetic valve was secondary to lupus and renal disease. We hypothesized that her low ejection fraction was secondary to mitral stenosis and potentially reversible. We performed a dobutamine stress echocardiogram, which revealed an improved ejection fraction to more than 50% and confirmed preserved inotropic contractile reserve of her myocardium. Based on this finding, she underwent a metallic mitral valve and tricuspid valve replacement. Following surgery, her symptoms completely resolved. This case highlights the pathophysiology of lupus causing stenosis of prosthetic valves and low ejection cardiomyopathy.
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PMID:Severe Bioprosthetic Mitral Valve Stenosis and Heart Failure in a Young Woman with Systemic Lupus Erythematosus. 2761 Feb 49

A 42-year-old woman was referred from a primary care centre for severe hypertension, stage 3A chronic kidney disease and proteinuria. This was associated with a significant obstetric history of pre-eclampsia during her previous two pregnancies. Secondary hypertension was suspected and autoimmune workup was positive for anticardiolipin IgG and lupus anticoagulant. A renal biopsy showed evidence of chronic thrombotic microangiopathy, with electron microscopy features suggestive of fibrillar glomerulonephritis. The diagnosis of antiphospholipid syndrome with antiphospholipid-associated nephropathy was made. She was started on anticoagulation with warfarin, and her hypertension was controlled with lisinopril and amlodipine with subsequent improvement in proteinuria. She remains on regular follow-up to monitor for possible development of malignancy or connective tissue disease.
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PMID:Chronic thrombotic microangiopathy secondary to antiphospholipid syndrome, presenting with severe hypertension and chronic renal impairment. 3152 21