1/61. Pulmonary hypertension secondary to systemic lupus erythematosus: prolonged survival following treatment with intermittent low dose iloprost.Pulmonary hypertension (PHT) associated with systemic lupus erythematosus (SLE) has a dismal prognosis. Vasodilators and immunosuppressive therapy have been tried over the years with discouraging results. Prostacyclin (PGI2) which has potent vasodilatatory and anti-platelet effects has been demonstrated to significantly decrease pulmonary arterial pressure and pulmonary vascular resistance during acute infusion. Satisfactory response has been reported in SLE patients with PHT treated with short-term intravenous continuous PGI2 infusion. We report here a 48-month experience of the use of monthly low dose infusion of a PGI2 analogue, iloprost, in a SLE patient with pulmonary hypertension in new york heart association functional Class III. There was an initial haemodynamic response to an acute infusion of iloprost. Repeated infusions were followed by marked improvement in her functional status and her mean pulmonary arterial pressure dropped from 80 mmHg in the first few months and remained static at around 55 mmHg for the subsequent years.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
2/61. Successful treatment of systemic lupus erythematosus and pulmonary hypertension with intravenous prostaglandin I2 followed by its oral analogue.Systemic lupus erythematosus (SLE) is sometimes reported to complicate fatal pulmonary hypertension. A 46-year-old woman, with a ten-year history of SLE and pulmonary hypertension, was admitted to our hospital complaining of dyspnea and chest pain. She suffered pulmonary hemorrhage and after steroid pulse therapy, she underwent continuous intravenous infusion of epoprostenol (prostaglandin I2) with corticosteroid for four weeks, which reduced the pulmonary artery pressure and resistance. Following the successful treatment, beraprost sodium, an oral PGI2 analogue, was given and it maintained pulmonary hypertension remittance for four years.- - - - - - - - - - ranking = 0.9593132487138keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/61. Case report. Two cases of pulmonary hypertension with Raynaud's phenomenon. Primary pulmonary hypertension and systemic lupus erythematosus.Two cases of hypertensive pulmonary vascular disease with Raynaud's phenomenon are reported. One is a 32-year-old housewife of primary pulmonary hypertension with some immunological disorders and the other is a 28-year-old female of systemic lupus erythematosus. It may be considered that some immunological process could play a role in the etiology of some cases of primary pulmonary hypertension and the relationship of pathogenesis between these 2 cases was discussed.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/61. Familial lupus anticoagulant.The antiphospholipid antibody syndrome (APS) is defined by widespread arterial and venous thromboses associated with elevated plasma levels of antiphospholipid antibodies (APLA). The primary antiphospholipid antibody syndrome (PAPS) appear to be a fairly homogeneous disease, and HLA, family and other studies provide new insights into this cause of thrombosis and vascular disease. We describe two patients with PAPS (lupus anticoagulant positive), whose family members were analyzed for clinical and laboratory abnormalities associated with APS. Familial screening seems to be important, in order to prevent the thrombotic events. Low dose aspirin is the first line treatment in asymptomatic subjects with APLA, previous or present thrombosis requiring long-term, possibly life-long anticoagulation.- - - - - - - - - - ranking = 0.041991492353585keywords = lupus (Clic here for more details about this article) |
5/61. Severe pulmonary hypertension in a patient with systemic lupus erythematosus and minimal lupus activity.Pulmonary hypertension (PH) sometimes occurs in patients with systemic lupus erythematosus (SLE). We report a case of 51-year-old-woman with PH associated with SLE. She had been diagnosed as SLE on the basis of pericardial effusion, hematological disorder, positive antinuclear antibody, and hypocomplementemia. Despite minimal lupus activity, she had marked elevation of pulmonary arterial pressure (101/53 mmHg) and decreased cardiac index (1.5 l/min/m2). Symptoms related to PH were progressive under treatment with oral corticosteroids, oxygen, calcium antagonists, and warfarin. After 17 months of epoprostenol treatment, she died of pulmonary infarction. SLE-associated PH is often severe and progressive even in association with minimal activity.- - - - - - - - - - ranking = 1.0419914923536keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
6/61. Cardiac valvular disease in chronic inflammatory disorders of connective tissue. Factors influencing survival after surgery.Three cases of cardiac valvular disease complicating chronic inflammatory disorders of connective tissue are described. Each underwent surgical valve replacement. The outcome in the first case, a patient with aortitis complicating Reiter's syndrome and in good health otherwise, has been excellent. The other two patients, one with rheumatoid arthritis and vasculitis, the other with systemic lupus erythematosus and nephritis, died after operation as the result of their systemic disease. The importance of systemic features in assessing this type of patient is emphasized.- - - - - - - - - - ranking = 0.2keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
7/61. Management of pregnancy in women with pulmonary hypertension secondary to SLE and anti-phospholipid syndrome.Pulmonary hypertension is found in about 10% of patients with systemic lupus erythematosis (SLE). Pulmonary hypertension may be present at the time of diagnosis or may develop after the diagnosis of SLE or anti-phospholipid syndrome (APS). It often presents in the reproductive years and has a significant impact on pregnancy outcome, being a significant cause of indirect maternal deaths. In our observational case series of three patients there were two deaths (66%). In cases 1 and 2 the pulmonary hypertension developed during pregnancy and deteriorated rapidly with markedly abnormal mean pulmonary artery pressures of 80 and 70 mmHg respectively prior to death. Both patients died within 48 hours of delivery. In case 3 the pulmonary hypertension was milder and was diagnosed very early in pregnancy. The patient received multidisciplinary care from the first trimester and the management of the pregnancy, delivery and the early puerperium was planned. Careful epidural anaesthesia was used and the patient had invasive monitoring on the intensive therapy unit (ITU) for 72 hours. women with pulmonary hypertension need to be aware of the high risk of maternal mortality associated with pregnancy but we believe that an improvement in outcome can be achieved by careful assessment and the use of a multidisciplinary approach from early in pregnancy.- - - - - - - - - - ranking = 0.016578185009905keywords = lupus, systemic lupus (Clic here for more details about this article) |
8/61. Lupus cor pulmonale with electron microscope and immunofluroescent antibody studies.A case of systemic lupus erythemaosus with an unusual complication of pulmonary hypertension leading to cor pulmonale is reported. lung biopsy showed an interstitial pneumonitis with pulmonary vascular narrowing causing pulmonary hypertension. These changes appeared to be immunologically mediated by the utilization of IgG and C3. Microtubular virus-like particles were present in the endothelial cell cytoplasm of both lung and kidney.- - - - - - - - - - ranking = 0.016578185009905keywords = lupus, systemic lupus (Clic here for more details about this article) |
9/61. Intermittent epoprostenol infusions in systemic lupus erythematosus associated pulmonary hypertension--a series of three cases.INTRODUCTION: Pulmonary hypertension (PHT) associated with systemic lupus erythematosus (SLE) has a grave prognosis. Continuous prostacyclin infusion significantly reduces pulmonary arterial pressure (PAP) and may improve survival in patients with primary and secondary PHT. We report our experience with intermittent epoprostenol (EPO) infusion in SLE patients with PHT. CLINICAL CHARACTERISTICS: We reviewed patients with SLE associated PHT who were treated with intermittent EPO infusions in 1998. All 3 patients had severe PHT, with maximum systolic PAP (PASP) of 58, 96 and 67 mm Hg, respectively, when measured using Doppler echocardiography, and were in new york heart association functional class III. TREATMENT: All patients were given 6 infusions of EPO monthly via peripheral venous access without significant side effects. OUTCOME: The PASP of all patients remained stable during the therapy. The therapeutic response as measured by patient symptoms and PASP was variable. No patient had significant reduction of PASP during the 6-month treatment period. Two patients developed rebound elevation of PASP after cessation of treatment, while the PASP of the other patient remained stable for 10 months. CONCLUSION: Although intermittent EPO infusion did not substantially reduce PASP, it prevented further rise in PAP during the treatment period, suggesting that this regimen may benefit a subset of SLE patients with PHT.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
10/61. Systemic lupus erythematosus-associated pulmonary hypertension: good outcome following sildenafil therapy.A 46-year-old woman with systemic lupus erythematosus (SLE) and concomitant severe pulmonary hypertension (PH) is described. Other secondary causes of PH including thromboembolism, phospholipid syndrome, valvular disease and interstitial pulmonary involvement were ruled out. Owing to her lack of clinical response to conventional therapy, sildenafil was begun at increasing doses up to 400 mg daily. Both clinical and hemodynamic improvement ensued. This appears to be the first clinical report of the use of sildenafil in SLE followed by resolution of severe PH.- - - - - - - - - - ranking = 0.83725299485521keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
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