Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary hemorrhage due to small vessel vasculitis is a well recognized complication of many autoimmune and collagen vascular disorders. The antiphospholipid antibody syndrome (APS) is a disorder associated with hypercoagulability, thrombocytopenia, and thromboembolic phenomena. Pulmonary manifestations include pulmonary embolism, pulmonary hypertension, and respiratory failure associated with diffuse pulmonary infiltrates. We describe a patient with the APS syndrome, without systemic lupus erythematosus, who developed pulmonary hemorrhage due to pulmonary capillaritis with evidence of perivascular immune complex deposition.
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PMID:Pulmonary capillaritis and alveolar hemorrhage associated with the antiphospholipid antibody syndrome. 778 81

Chronic thromboembolic pulmonary hypertension (CT-E PH) is a rare and aberrant outcome of acute pulmonary embolism. Because it has become a potentially curable form of pulmonary hypertension, the frequency of recognized cases has increased. We report a case series of 72 patients with CT-E PH evaluated in our institution between 1984 and 1993, and discuss diagnostic clues and therapeutic approaches. All patients complained of dyspnea on exertion, a history of acute thromboembolic event, and lung murmurs were found in 60% and 17% of patients, respectively. The presence of a disorder of coagulation was found in 30% of the patients tested, the most common abnormality being lupus anticoagulant. The key noninvasive study for diagnosis was the lung perfusion scan which showed at least one segmental or larger perfusion defect in all patients. Pulmonary angiography confirmed the diagnosis in all cases, and sometimes associated to intravascular ultrasound imaging, established the feasibility of thromboendarterectomy. Medical therapy included the use of long-term oral anticoagulant, and in the case of lower limb venous thrombosis, inferior vena cava filtration. Finally two surgical procedures were discussed in selected patients: thromboendarterectomy and lung transplantation. Since 1988, eight patients have benefited from lung transplantation (six patients are still alive), and 11 patients underwent thromboendarterectomy which was successful in 9 patients with a dramatic functional and hemodynamic improvement.
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PMID:Surgical management of unresolved pulmonary embolism. A personal series of 72 patients. 781 30

Non-resolved chronic pulmonary thromboembolism is a frequent cause of pulmonary hypertension. In long-standing disease hypertension is progressive due to intimal and medial changes in the perfused vessels. Non-resolution of thromboemboli is often associated with underlying coagulopathies; the presence of a lupus anticoagulant may pose a significant problem in the peri-operative management of these patients. Pulmonary thrombendarterectomy presents an efficient option of treatment which is feasible in the majority of patients. By means of pulmonary angiography and computed tomography operability is verified by the often difficult recognition of thromboembolic changes in the central pulmonary arteries. Patients with solely peripheral thromboembolic changes or primary pulmonary hypertension must be excluded. In presence of significant exertional dyspnea and/or pulmonary pressure elevation surgery is indicated. Mortality is high and mainly related to unrelieved pulmonary hypertension or pulmonary complications; pulmonary reperfusion edema, respiratory failure or pneumonia and sepsis. In all survivors the reduction of pulmonary hypertension is highly significant and persistent. Thromboembolic pulmonary hypertension may be treated curatively in most patients by thrombendarterectomy. Correct selection of surgical candidates is mandatory, and the patients should preferably be diagnosed and undergo surgery in an early stage of their disease.
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PMID:[Surgical treatment of thromboembolism-induced pulmonary hypertension]. 786 94

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)
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PMID:Five-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus. 787 81

Active rheumatic disease during pregnancy may require drug treatment to ensure the mother's health is maintained and that there is a good outcome for the fetus. However, knowledge on the use of antirheumatic drugs during pregnancy is limited, rendering decision making difficult both for the patient and the physician. The effect of nonsteroidal anti-inflammatory drugs (NSAIDs) in the treatment of rheumatoid arthritis has been investigated in depth for aspirin (acetylsalicylic acid) and indomethacin only. Information about the use of ibuprofen, sulindac, ketoprofen and diclofenac during pregnancy is scanty and there is no such information for newer agents such as the fenemates and oxicams. There is no evidence for teratogenicity of any NSAID in humans. However, due to the shared property of inhibition of prostaglandin synthesis, adverse effects such as constriction of the ductus arteriosus in utero, persistent pulmonary hypertension in the neonate and prolongation of pregnancy and labour are possible. When administered to pregnant patients, NSAIDs should be given in the lowest effective dose, and should be withdrawn within the 8 weeks prior to expected delivery. Transplacental passage varies for different corticosteroids. Because of the inability of the fetal liver to convert prednisone to its active metabolite and the ability of the placenta to convert prednisolone to the inactive prednisone, both prednisolone and prednisone are drugs of choice in pregnant patients requiring corticosteroid treatment. Corticosteroids do not increase the risk of congenital malformations. Possible adverse effects are perinatal infection and adrenal insufficiency in the newborn. Both events are only rarely reported in the literature, which comprises information on more than 1000 pregnancies. The clinical experience on the effect of slow-acting antirheumatic drugs (SAARDs) on pregnancy is insufficient to draw substantial conclusions. Available data from the literature give no clear evidence of an increased risk of teratogenicity for any of these drugs. Rheumatologists differ in their view on the advisability of using SAARDs during pregnancy. Hydroxychloroquine, which is regarded as less toxic than chloroquine, is recommended by some rheumatologists for the treatment of pregnant patients with active systemic lupus erythematosus (SLE) or rheumatoid arthritis. Sulfasalazine can be continued during pregnancy. Data on gold compounds and penicillamine are sparse and inconclusive. A reasonable approach is to stop these agents as soon as pregnancy is confirmed. The limited experience with cyclosporin has been obtained when the drug was used to prevent allograft rejection. Further data regarding the use of this drug in pregnant patients with rheumatic diseases are needed.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Optimisation of antirheumatic drug treatment in pregnancy. 788 37

Mixed connective tissue disease (MCTD) was proposed by Sharp and others in 1972. MCTD is a unique disease in which the presence of nuclear RNP antibody is characteristic and the patient shows partial symptoms of SLE, PSS and or PM/DM. Among them, Raynaud's phenomenon and sausage like finger or swollen hand are the most common symptoms. Although patients with MCTD generally respond to small amount of corticosteroid and the prognosis is not so bad, some patients with MCTD especially those with pulmonary hypertension show high mortality.
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PMID:[MCTD (mixed connective tissue disease)]. 793 96

An autopsied case of systemic lupus erythematosus with pulmonary hypertension is reported. A 29-year-old woman with a seven-year history of polyarthralgia, butterfly rash, nephrotic syndrome and Raynaud's phenomenon was admitted because of progressive dyspnea on exertion. Tests for antinuclear antibody, anti-cardiolipin antibody and lupus anticoagulant were positive. Echocardiographic examination revealed right ventricular hypertrophy and a moderate pericardial effusion. Estimated systolic pulmonary arterial pressure was 53 mmHg. Despite treatment with corticosteroids including pulse methylprednisolone therapy, lipo-PGE1 and warfarin, she died of progressive congestive heart failure. Postmortem examination of the pulmonary vasculature revealed findings consistent with plexogenic pulmonary arteriopathy, without evidence of vasculitis, fibrinoid necrosis, or thromboemboli.
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PMID:Pulmonary hypertension in systemic lupus erythematosus: a report of an autopsied case. 800 Jan 4

To clarify the histopathological characteristics of pulmonary hypertension (PH) in Japan, and to clarify the role of serotonin and endothelin in monocrotaline induced PH, human histopathological studies and experimental studies were carried out. An epidemiological study based on the Annual of the Pathological Autopsy Cases in Japan, and a morphological study on autopsy cases of congenital heart disease and idiopathic PH were performed. Plasma levels of serotonin and endothelin, vascular responsiveness to serotonin, and the effects of a selective serotonin antagonist, DV-7028, were investigated after monocrotaline injection. Plexogenic pulmonary arteriopathy was prevalent, and recurrent pulmonary thromboembolism and pulmonary veno-occlusive disease extremely rare among primary pulmonary hypertension in Japan. In secondary PH, systemic lupus erythematosus and mixed connective tissue disease were frequent and showed particularly severe intimal and medial thickening. After an injection of monocrotaline, plasma serotonin and endothelin levels were raised, and pulmonary arteries showed hyperreactivity to serotonin. DV-7028 (5-HT2 receptor antagonist) attenuated the rise in pulmonary artery pressure and the various effects of monocrotaline. There may be some genetic difference between PH in Japan and other countries. Roles for serotonin and endothelin in the initiation and progression of monocrotaline induced PH are suggested.
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PMID:Pathology of pulmonary hypertension: a human and experimental study. 804 94

Recurrent pulmonary emboli or microthromboses are hypothesized as possible causes of pulmonary hypertension in the antiphospholipid syndrome (APS), but thrombosis of the pulmonary vessels has been rarely documented. We describe the case of a 45-year-old Caucasian man affected by thrombocytopenia, recurrent deep venous thrombosis, recurrent pulmonary embolism and fatal chronic pulmonary hypertension (systolic pressure: 85 mm Hg). Anticardiolipin antibodies were highly positive, and the lupus anticoagulant was present. At autopsy, recent thromboses of small vessels were observed in the lung, with organized clots and recanalized channels. Furthermore, friable and firm vegetations and nodules were observed on the cusps of the mitral and tricuspid valves, intermingled with recent surface fibrinous thrombi. In the adrenals we found vascular thrombotic lesions similar to those in the lungs. The pathological lesions suggest pulmonary hypertension secondary to pulmonary arterial microthromboses. Moreover, this is the first documentation of tricuspid valve pathology in a patient with APS.
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PMID:Pulmonary hypertension secondary to thrombosis of the pulmonary vessels in a patient with the primary antiphospholipid syndrome. 806 40

A case of non-cirrhotic portal hypertension in a patient with systemic lupus erythematosus, the first of our knowledge, is described. Severe pulmonary hypertension was associated with the portal hypertension and with markers of active auto-immunity. Pulmonary hypertension has not previously been associated with non-cirrhotic portal hypertension. The coexistence of vasculopathy of the portal and pulmonary vascular beds in this patient with active autoimmunity supports the postulate that portal-pulmonary hypertension may be immunologically mediated.
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PMID:Pulmonary hypertension associated with non-cirrhotic portal hypertension in systemic lupus erythematosus. 814 19


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