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)

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
 ...
PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

A prospective study was performed in our center on 60% (n = 36) of patients with systemic lupus erythematosus (SLE) to determine the prevalence and severity of pulmonary hypertension. Twenty-six healthy subjects of similar age and sex served as controls. Pulmonary artery systolic pressure was calculated from the sum of the peak tricuspid insufficiency Doppler pressure gradient and an estimate of right atrial pressure based on inferior vena cava size and its degree of inspiratory collapse. Five patients with SLE (14%) had pulmonary hypertension, defined as pulmonary artery systolic pressure greater than 30 mm Hg. Cardiac indices determined by planimetry of biplane apical 2-dimensional echocardiographic images were low or normal in the patients with pulmonary hypertension implying increased pulmonary vascular resistance as the etiology for elevated pulmonary artery pressure. The mean pulmonary artery systolic pressure in patients with SLE was 25 +/- 10 mm Hg vs 20 +/- 2 in controls (p = 0.002). No control had a pulmonary artery systolic pressure greater than 23 mm Hg. Patients with pulmonary hypertension had a shorter duration of SLE and steroid therapy and a higher prevalence of cytotoxic treatment and Raynaud's phenomenon in comparison to those with normal pulmonary artery pressures. The prevalence of systemic hypertension, interstitial lung disease, pleurisy, pericarditis, cutaneous manifestations, arthritis, renal disease, central nervous system involvement, and hematologic abnormalities was similar in patients with SLE with normal and elevated pulmonary artery pressure. Our study suggests that pulmonary hypertension in SLE is common but usually mild.
 ...
PMID:Pulmonary hypertension in systemic lupus erythematosus. 233 68

Cardiologic and laboratory parameters were studied in 21 patients with systemic lupus erythematosus (SLE) with cardiopulmonary symptoms (CPS), 20 SLE patients without CPS and 45 age- and sex-matched healthy controls. The most frequent cardiac abnormalities in patients with CPS included pericardial effusion (24%), ventricular enlargement (20%), mitral regurgitation (19%) and tricuspid regurgitation (14%). No structural abnormalities were observed in SLE patients without CPS. Mean calculated and derived echocardiographic values in both groups of SLE patients differed significantly from those observed in normal controls (p < 0.004). Patients with CPS had significantly lower mean values of ejection fraction (p < 0.05) and fractional shortening (p < 0.03). However, the frequencies of functional abnormalities in patients with CPS did not differ significantly from those observed in patients without CPS. There were no remarkable laboratory findings in SLE patients with CPS compared to those without. The finding that some SLE patients may have functional cardiac abnormalities in the absence of CPS is an important one. It raises the question as to whether asymptomatic cardiac involvement in SLE is a separate entity or whether it heralds symptomatic cardiopulmonary involvement.
 ...
PMID:Asymptomatic cardiac involvement in systemic lupus erythematosus. 779 6

The purpose of this study was to determine the prevalence and progression of pulmonary hypertension over a 5-year follow-up period in 28 patients with systemic lupus erythematosus (SLE) who were originally enrolled in an echocardiographic study of pulmonary hypertension in 1985 and 1986. Twenty healthy volunteers without cardiac or pulmonary disease participated as normal controls. Each patient and control underwent a complete Doppler echocardiographic study. Doppler echocardiographic recordings of tricuspid insufficiency, with saline contrast enhancement when necessary, were used to calculate pulmonary artery systolic pressure according to the modified Bernoulli equation. Doppler echocardiographic measurement of cardiac output was performed at rest for each subject, and pulmonary resistance was calculated by dividing the pulmonary artery systolic pressure by the cardiac output. These results were compared to results of the original studies to detect serial changes in pulmonary pressure and pulmonary resistance; results were also compared to the group of normal controls. The prevalence of pulmonary hypertension increased from 14% at the first study to 43% at follow-up. A significant increase in mean systolic pulmonary artery pressure was detected in the SLE patients during the follow-up period: 23.4 vs 27.5 mm Hg (p < 0.005). In addition, a significantly higher pulmonary artery pressure was detected in the SLE patients compared with the normal controls (p < 0.005).(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Five-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus. 787 81

The aim of this study was to assess the relationship between the incidence and severity of valvular regurgitation and the presence of high levels of anticardiolipin antibodies in a group of patients with systemic lupus erythematosus. Fifty patients aged 35.5 +/- 13.4 years and 84 healthy age and sex matched controls were studied with two dimensional echocardiography with color flow imaging. IgG and IgM anticardiolipin antibodies were measured in all patients within a week of the echocardiographic study. Patients had a similar incidence of aortic, tricuspid and pulmonic regurgitation than normals. However there was a greater incidence of mitral regurgitation among patients (56 vs 21% p < 0.001). The seven patients with moderate or severe mitral regurgitation had Libman Sacks vegetations of the valve. Twenty five of 28 patients with mitral regurgitation had increased anticardiolipin antibodies; moreover, these levels were significantly higher among patients with mitral regurgitation and thickened mitral valves than those with normal valves. Patients with increased anticardiolipin antibodies had a higher incidence of Libman Sacks vegetations. No association between the presence of these antibodies and the severity of aortic, pulmonic or tricuspid regurgitation was observed. It is concluded that the incidence of mitral valve regurgitation is increased in systemic lupus erythematosus and related to raised anticardiolipin antibodies.
 ...
PMID:[Systemic lupus erythematosus: valvular regurgitation and its relation to anticardiolipin antibodies]. 830 11

The aim of this study was to determine the prevalence of cardiac valve disease in systemic lupus erythematosus or in patients with primary antiphospholipid syndrome and to assess the role of the antiphospholipid antibodies as risk factor for endocardial lesions. We studied 39 consecutive patients with systemic lupus erythematosus (mean age 34 +/- 12 years, 38 female and one male), 20 women with primary antiphospholipid syndrome (mean age 32 +/- 4 years) and 20 normal subjects (mean age 35 +/- 8 years, 15 female and five male). All patients with primary antiphospholipid syndrome had increased levels of serum anticardiolipin antibodies and recurrent fetal abortions; some of them also had arterial and/or venous thrombosis and/or thrombocytopenia. M-mode, two-dimensional and Doppler echocardiography were performed in all patients. IgG anticardiolipin antibodies were measured by an enzyme-linked immunosorbent assay. Valvular lesions were observed in 15 patients (38%) with systemic lupus erythematosus. These abnormalities included: mitral valve thickening or vegetation, mitral valve prolapse and aortic valve vegetation; mitral, aortic and tricuspid regurgitation; mitral stenosis. None of the patients with primary antiphospholipid syndrome and of the normal subjects was found to have valvular abnormalities. In systemic lupus erythematosus, high levels of anticardiolipin antibodies were detected in 73% of the patients with valvular lesions and in 67% of the patients without valvular lesions (P > 0.05). We conclude that valvular involvement is frequent in patients with systemic lupus erythematosus but it is apparently unrelated to antiphospholipid autoimmunization.
 ...
PMID:Cardiac valve involvement in systemic lupus erythematosus and primary antiphospholipid syndrome: lack of correlation with antiphospholipid antibodies. 852 6

Congenital heart block (CHB) can result in intrauterine cardiac failure leading to fetal or neonatal loss. To establish perinatal hemodynamic factors which might predict adverse outcome, six fetuses with CHB diagnosed between 20 and 30 gestational weeks were examined by echocardiography at 2-week intervals. Neonatal morbidity and outcome in infancy are detailed. The fetuses showed a significant decrease in ventricular rate (VR) with advancing gestation (60 +/- 7 vs 51 +/- 4 beats/min, p = 0.03). Cardiac decompensation defined as hydrops or pericardial effusion was associated with VR of lower than 55 beats/min in two fetuses. Three mothers had a therapeutic trial with a sympathomimetic and digoxin. Salbutamol increased VR 10% in one of three fetuses treated. Digoxin decreased pericardial effusion in one hydropic fetus with autoimmune myocarditis. In this fetus, poor left ventricular fractional shortening (LVFS) was accompanied with high umbilical artery resistance index (RI). High amniotic fluid erythropoietin indicated severe hypoxia preceding death. Pacemaker was indicated in all the newborns. At the age of 2 weeks all the surviving infants had tricuspid regurgitation and a shunt through foramen ovale due to asynchronized atrioventricular contraction. During the 12-month follow-up two of five surviving infants had no symptoms. One had symptomatic neonatal lupus. Two infants had patent ductus arteriosus, one with dilated cardiomyopathy. In conclusion, poor fetal outcome was associated with low VR, low LVFS, and high RI. Despite early pacing, morbidity was high in infancy due to cardiomyopathy and associated heart defects. Regular echocardiographic monitoring during pregnancy and after delivery is required in order to optimize care and timing of any interventions.
 ...
PMID:Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment, and outcome in six cases. 1152 12

Although patients undergoing cardiac surgery often present with diverse comorbidities, those with coagulation derangements are especially challenging. The present report describes the management of a patient who presented with a Factor V Leiden mutation, lupus anticoagulant, and acquired activated protein C resistance. A 42-year-old female presented with acute shortness of breath and chest pain. She was otherwise healthy 1 month prior to admission when she presented with dysfunctional uterine bleeding, resulting in the transfusion of three units of packed red blood cells. Coagulation evaluation revealed that the patient had lupus anticoagulant, factor V Leiden mutation and an activated protein C resistance. The patient presented with an acute myocardial infarction and was found to have 90% stenosis of her left main coronary artery, moderate mitral and tricuspid regurgitation, and a left ventricular ejection fraction of 25%. An emergent off-pump coronary artery bypass procedure with placement of a vein graft to the left anterior descending artery was completed. Intraoperative thrombophilia was encountered as evidenced by both an elevated thromboelastograph coagulation index (+3.6) and an acquired antithrombin-III deficiency. Postoperatively, the patient was placed on low molecular weight heparin, but developed heparin-induced thrombocytopenia and was switched to a direct thrombin inhibitor, argatroban. The following case report describes the coagulation management of this patient from the time of admission to discharge 43 days later, and the unique challenges this combination of hemostatic defects present to the clinicians.
 ...
PMID:Coagulation management of a patient with factor V Leiden mutation, lupus anticoagulant, and activated protein C resistance: a case report. 1591 49

The most common valves involved in systemic lupus erythematosus are the mitral and aortic valves. Although isolated tricuspid valve involvement is quite rare, the authors report such a case. A 42-year-old woman presented with exertional dyspnea and was found to have a cardiac murmur. Echocardiography showed a stenotic tricuspid valve with vegetations on all 3 cusps. No other valvular vegetation could be detected. Concomitant tricuspid regurgitation was noted too. Blood culture results were negative. Clinical findings and serologic tests confirmed the diagnosis of systemic lupus erythematosus. The patient was successfully treated with prednisolone and hydroxychloroquine, and follow-up echocardiography showed the disappearance of the vegetations.
 ...
PMID:Isolated tricuspid valve Libman-Sacks endocarditis and valvular stenosis: unusual manifestations of systemic lupus erythematosus. 1983 4

We report a 43-year-old female who developed pulmonary arterial hypertension (PAH) during intensive immunosuppressive therapy for systematic lupus erythematosus (SLE). She was diagnosed as SLE at the age of 32 years based on serological and hematological abnormalities, oral ulcers, and facial erythema. She experienced frequent flare-ups of disseminated discoid lupus between the ages of 33 and 36 years and developed immune thrombocytopenia at the age of 39 years. In 2007 when she was 43 years old, she developed lupus nephritis (LN) with elevated serum anti-double stranded DNA antibodies and urine protein of less than 1 g/day. Combination therapy for the LN with 35 mg/day prednisolone and intravenous cyclophosphamide (IVCY) led to renal remission. After the seventh monthly session of IVCY, she developed dyspnea on exertion. PAH was diagnosed based on enlarged main pulmonary arteries on the chest x-ray, right ventricular outflow and a peak tricuspid regurgitant pressure gradient exceeding 45 mmHg on echocardiography, an elevated plasma brain natriuretic peptide (BNP) level of 260 pg/ml, the exclusion of pulmonary thromboembolism, and no lung fibrosis. The PAH was treated successfully with bosentan. At present the tricuspid regurgitation has disappeared, and the plasma BNP level has normalized.
 ...
PMID:[Successful bosentan therapy in a case of pulmonary arterial hypertention developed during immunosuppressive therapy for lupus nephritis]. 2162 52


1 2 Next >>