Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 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.
 ...
PMID:[Plasma endothelin in normal probands and patients with nephrologic-rheumatologic and cardiovascular diseases]. 221 2

Preeclampsia, eclampsia and HELLP syndrome are severe complications of pregnancy associated with a high morbidity and mortality for both mother and foetus. The issue of prognosis with regards to risk of relapse is therefore of upmost importance for both the patients and doctors managing their subsequent pregnancies. The overall risk of relapse is estimated to be less than 10%. The risk is especially increased in case of early onset, before the 28(th) week of amenorrhea. In such patients, an underlying renal disease, essential hypertension or secondary hypertension might be identified. A renal consultation must ensure the complete resolution of the hypertension and proteinuria, otherwise investigations need to be carried out looking for an underlying renal or vascular disease affecting the renal parenchyma or outflow. Screening for a hereditary thrombophilia is performed in the immediate post-partum period following and early onset PE, especially if associated with an IUGR, fetal death, or in an early and severe relapse. A renal biopsy is only rarely considered: it is either performed within the first post-partum days whenever a systemic disease flare-up is suspected (i.e. SLE), or late if the proteinuria failed to settle beyond 6 months post-part. Severe PE is a marker of a vascular disease of short duration, but must be considered a risk factor for later atherosclerosis with cardiovascular complications, justifying long term cardiovascular, renal and metabolic follow-up.
 ...
PMID:[Late prognosis after preeclampsia]. 2048 49

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.
...
PMID:Severe Bioprosthetic Mitral Valve Stenosis and Heart Failure in a Young Woman with Systemic Lupus Erythematosus. 2761 Feb 49

Aortic aneurysms are not commonly reported among patients with systemic lupus erythematosus (SLE). We report a case of a 47-year-old Filipino female diagnosed with SLE 17 years ago maintained on prolonged oral steroids, azathioprine, and hydroxychloroquine. She also had lupus nephritis, secondary hypertension, and dyslipidemia. She initially presented with a week-long watery nonbloody diarrhea with associated diffuse crampy abdominal pain and generalized weakness. She was admitted for a week at a provincial hospital and was given an unrecalled antibiotic with resolution of symptoms. Upon discharge, however, she experienced two weeks of severe right lower quadrant pain radiating to the back and left lower quadrant, with no history of diarrhea, vomiting, dysuria, and fever. Complete blood count showed slight leukocytosis and elevated C-reactive protein. Abdominal imaging revealed a saccular infrarenal aneurysm with dissection. An atherosclerotic mechanism was primarily considered, but a vasculitic process was likewise considered due to elevated acute phase reactants. The initial plan was Endovascular Aneurysm Repair (EVAR) but due to financial limitations, an exploratory laparotomy with infrarenal endoaneurysmorrhaphy was eventually performed. Intraoperative findings were a saccular infrarenal aneurysm with dissection up to the proximal right common iliac artery and an abscess compartment within the false lumen in the anterior aortic wall. Abscess culture yielded high growth of Salmonella group B. Micrographs of the aortic wall biopsy showed fibrin deposition necrosis and calcification with peripheral viable cellular infiltrates consisting of neutrophils and foamy macrophages. Inadvertently placing an endovascular graft in an infected aortic aneurysm would have led to graft infection and catastrophic morbidity. We highlight the significance of having a high index of suspicion for infectious causes of aortitis among immunocompromised patients presenting with aneurysm prior to pursuing an endovascular versus an open approach for repair.
 ...
PMID:Infectious Aortitis with Abdominal Aortic Aneurysm in a 47-Year-Old Female with Systemic Lupus Erythematosus. 3108 27

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.
 ...
PMID:Chronic thrombotic microangiopathy secondary to antiphospholipid syndrome, presenting with severe hypertension and chronic renal impairment. 3152 21