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Query: UMLS:C0024141 (systemic lupus erythematosus)
44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this paper, the implementation of an Internet-based telematic service for medical support is presented, which was developed and operated in pilot form within the INTRANET HEALTH CLINIC project--a 2-year project supported by the European Commission under the Health Telematics Programme. The aim of the application is to offer high quality care to users of health services over inexpensive communication pathways, using Internet-based, interactive communication tools, like remote access to medical records and transmission of multimedia information. The XML technology was employed to achieve customised views on patient data, according to the access rights of different user profiles. Strict security and access control policy were implemented to ensure secure transmission of medical data through the Internet. The system was designed to collaborate with existing clinical patient record systems and to be adjustable to different medical applications. Current implementations include the fields of Oncology, Lupus Erythrematosis, Obstetrics and Chronic Obstructive Pulmonary disease. The results of the pilot operation with oncological patients in Greece were encouraging, so that the refining of the system and its expansion to a large number of patients is already in progress.
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PMID:Medical support system for continuation of care based on XML web technology. 1173

We present a case with diagnosis of pulmonary alveolar microlithiasis that illustrates the appearance of this rare chronic lung disease on conventional chest X-ray, high-resolution CT, and transbronchial lung biopsy. This is the first case reported which developed pulmonary alveolar microlithiasis after Varicella zoster infection in a patient with antiphospholipid antibodies and discoid lupus.
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PMID:Pulmonary alveolar microlithiasis after Varicella zoster infection in a patient presenting with antiphospholipid syndrome and discoid lupus. 1532 12

Urticarial vasculitis, a form of leukocytoclastic vasculitis involving the postcapillary venules, is classified as a type III hypersensitivity reaction and has been associated with connective tissue disease. The lesions resemble urticaria and typically persist for more than 24 hours. Urticarial vasculitis usually affects young women, and the diagnosis is confirmed at histologic examination. Patients with urticarial vasculitis can be divided into 2 types--those with normal complement levels and those with hypocomplementemic urticarial vasculitis (HUV). Patients with normocomplementemic urticarial vasculitis have a milder course than do patients with HUV, a condition that has a strong association with systemic lupus erythematosus. Angioedema, ocular inflammation, obstructive lung disease, and glomerulonephritis are commonly associated with HUV. We describe the case of a girl with systemic lupus erythematosus and HUV who also had pancreatitis, hypothyroidism, and elevated levels of antiphospholipid antibodies.
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PMID:Hypocomplementemic urticarial vasculitis: report of a 12-year-old girl with systemic lupus erythematosus. 1239 49

TNFalpha inhibition has a clearly beneficial effect in a number of arthritides and in Crohn's disease. The exact mechanism of action is uncertain with studies showing inhibition of chemokines, inhibition of adhesion molecule expression, and improved T-cell function. Unlike most therapeutic interventions for autoimmune disease, TNFalpha inhibition appears to act on specific pathologic processes. It is not known how wide-spread these TNFalpha-mediated pathologic processes are. Efforts to expand the use of TNFalpha inhibition have had notable successes but have been disappointing in other disorders. We hypothesized that TNFalpha-mediated pathologic processes might play a significant role in the end-organ effects seen in SLE. We modeled SLE by using MRL/lpr mice and treated with two types of TNFalpha inhibitor. Pulmonary disease was significantly improved in the treated groups compared to controls. In contrast, renal disease was unaffected suggesting that in lupus, where multiple organs are affected, different pathologic processes may be mediating the end-organ damage. This has important implications for designing therapeutics for SLE.
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PMID:TNFalpha inhibition in MRL/lpr mice ameliorates pulmonary but not renal disease. 1247 42

Scleroderma (systemic sclerosis) is associated with several autoantibodies, each of which is useful in the diagnosis of affected patients and in determining their prognosis. Anti-centromere antibodies (ACA) and anti-Scl-70 antibodies are very useful in distinguishing patients with systemic sclerosis (SSc) from healthy controls, from patients with other connective tissue disease, and from unaffected family members. Whereas ACA often predict a limited skin involvement and the absence of pulmonary involvement, the presence of anti-Scl-70 antibodies increases the risk for diffuse skin involvement and scleroderma lung disease. Anti-fibrillarin autoantibodies (which share significant serologic overlap with anti-U3-ribonucleoprotein antibodies) and anti-RNA-polymerase autoantibodies occur less frequently and are also predictive of diffuse skin involvement and systemic disease. Anti-Th/To and PM-Scl, in contrast, are associated with limited skin disease, but anti-Th/To might be a marker for the development of pulmonary hypertension. Other autoantibodies against extractable nuclear antigens have less specificity for SSc, including anti-Ro, which is a risk factor for sicca symptoms in patients with SSc, and anti-U1-ribonucleoprotein, which in high titer is seen in patients with SSc/systemic lupus erythematosus/polymyositis overlap syndromes. Limited reports of other autoantibodies (anti-Ku, antiphospholipid) have not established them as being clinically useful in following patients with SSc.
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PMID:The clinical relevance of autoantibodies in scleroderma. 1271 48

Cardiac abnormalities has been receiving increased attention in patients with systemic lupus erythematosus (SLE). Cardiovascular system involvement has been found to have a substantial effect on mortality and morbidity in patients with SLE [1]. Recent diagnostic methods using echocardiography examination have allowed the delineation of cardiac manifestations such as myocarditis and myocardial dysfunction, valvular disease, pericardial disease or pulmonary hypertension. A report of two cases is presented: 23-year-old man with acute myocarditis with left ventricular failure and pulmonary oedema as a initial presentation of active SLE, and 51-year-old woman with SLE, antiphospholipid antibodies, with history of cerebral embolic infarction, TIA and venous thrombosis and with mitral valvular dysfunction in course of nonbacterial thrombotic endocarditis. Pulmonary hypertension has been recognised in both patients probably as a result of vasculaopathy and intimal proliferation, vasculitis, thromboembolic disease or parenchymal lung disease in SLE. Recent advances in diagnosis and treatment have substantially improved the prognosis of patients with systemic lupus erythematosus and cardiovascular system involvement [2].
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PMID:[Cardiovascular involvement in systemic lupus erythematosus: report of two cases]. 1287 81

Primary Pulmonary Artery Sarcoma is a rare entity, which shares some clinical features with Thromboembolic Pulmonary Disease (TEPD), complicating differential diagnosis. The authors report a Clinical Case of a Primary Pulmonary Artery Sarcoma in a 59 years old man, admitted with a history of dyspnoea on exertion, chest pain and general symptoms. Chest X-ray, Computed Tomography Scan, Angiographies and Magnetic Resonance Imaging suggested TEPD. Blood Analysis performed before anticoagulation therapy: Lupus Anticoagulant-and Ig M Anticardiolipin +. Our presumptive initial diagnosis was TEPD in a patient with a hypercoagulable state. Intravenous heparin was started, with some clinical improvement but 2 months later he was readmitted, due to clinical and radiological deterioration. Pulmonary Thromboendarterectomy was considered but a right pneumonectomy was necessary because of bleeding. He died of ARDS in a single lung in the 7th day after surgery. Pathology revealed pulmonary artery sarcoma with pulmonary and pleural metastases.
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PMID:[Pulmonary Artery Sarcoma - diagnostic and treatment difficulties]. 1295 67

In some children with cystic fibrosis (CF), percutaneous long lines occlude sooner than expected (due to thrombophlebitis or thrombosis), and many have a totally implantable venous access device (TIVAD), a recognized complication of which is thrombosis. This complication is more likely if the child has an underlying thrombotic tendency, which may be enhanced in the presence of inflammatory lung disease. There are no reports of an identified association of heritable thrombophilia with CF, although individual cases have been recognized. Our aim was to determine the incidence of thrombophilia in children with CF. In a tertiary pediatric CF center, blood was screened for thrombophilia at annual review, and retested if abnormal. A thrombotic abnormality was found in 41/204 (20%) patients. These included activated protein C resistance (10/204, 5%) with a prevalence similar to that expected, but the following abnormalities had an increased prevalence: antithrombin deficiency (2/204, 1%), protein S deficiency (11/204, 5%), protein C deficiency (8/204, 4%), and lupus anticoagulant (18/204, 9%). There were no differences found in those with thrombophilia for the following parameters: age, gender, genotype, lung function, presence of Pseudomonas aeruginosa, prothrombin time, serum IgE, aspergillus-specific IgE, liver function, and blood inflammatory markers. Fifteen children had TIVADs, 4 of whom had evidence of thrombophilia. In conclusion, a significant proportion of patients had a thrombophilic abnormality. We recommend that thrombophilia screening be performed prior to insertion of a TIVAD, and also in those with a history of venous thrombosis, blocked TIVADs, or recurring problems with long lines.
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PMID:Thrombophilia in children with cystic fibrosis. 1567 7

The objective of this study was to assess the prevalence, clinical, histological and immunological characteristics, and the long-term outcome of polymyositis- (PM) and dermatomyositis- (DM) associated lung disease, and to define subgroups of lung-associated inflammatory myopathies. This retrospective study included 81 consecutive patients diagnosed with PM/DM. Pulmonary involvement was systematically investigated in relation to clinical symptoms by chest radiography, high resolution computed tomography and pulmonary function testing. Anti-synthetase autoantibodies (ASA) were analysed by ELISA and confirmed by protein and RNA immunoprecipitation methods. Statistical analyses were done with the Student t-test and Fisher exact test. Cumulative survival probabilities were estimated by the Kaplan-Meier method and Cox regression analysis. Fifty patients (61%) presented pulmonary involvement. Thirty-two (39%) had interstitial lung disease and five of them had devastating acute interstitial pneumonia with pneumomediastinum and an unfavorable prognosis. Histology showed diffuse alveolar damage in this subgroup and ASA were negative. Eighteen patients (22%) presented restrictive myopathic lung disease; in three of them respiratory muscles could not maintain ventilation. ASA were positive in 17 of the 50 patients (34%) and were significantly associated with interstitial lung disease (OR: 4.5 [95% CI: 1.3-15.3]), arthritis (OR: 6.0 [95% CI: 1.3-29.2]) and 'mechanic hands' (OR: 8.5 [95% CI: 1.7-41.4]); the presence of these autoantibodies did not imply worse survival prognosis. We concluded that clinical and immunological characteristics allowed the grouping of patients into different types of PM/DM lung-associated disease. Presence of ASA did not affect survival. ASA-negative patients with acute interstitial pneumonitis and pneumomediastinum had an unfavorable prognosis.
Lupus 2005
PMID:Polymyositis/dermatomyositis-associated lung disease: analysis of a series of 81 patients. 1721

Scleroderma heart involvement (SHI) is often manifest, and virtually always present when accurately searched and holds a significant prognostic value. Myocardial involvement by patchy fibrosis (secondary to both repeated ischaemia and immunoinflammatory damage) leads to ventricular diastolic dysfunction, whereas right ventricle overload and failure may complicate pulmonary hypertension. Left ventricular systolic dysfunction is present in a minority of patients, namely those presenting atherosclerotic coronary artery disease and/or arterial hypertension, sometimes triggered by sclerodermic renal involvement. Dysrhythmias and conduction disturbances are considered an hallmark of SHI, facilitated by autonomic dysfunction. SHI is frequently linked to parenchimal and/or vascular lung disease; they determine symptom occurrence, particularly dyspnoea, fatigue, palpitations and chest pain when pericardium is affected. Accurate cardiologic baseline screening and subsequent follow-up are mandatory in all patients, initially consisting in some noninvasive diagnostic procedures: visit, electrocardiogram (EKG), chest X-ray, Doppler-echocardiography. When needed, these examinations should be integrated by EKG Holter-monitoring, cardiopulmonary stress tests, cardiac magnetic resonance imaging, nuclear studies of myocardial function and perfusion, cardiac catheterization to better estimate pulmonary hypertension, and cardiac natriuretic hormone evaluation. Several vasodilator approaches (prostacycline or NO/endothelin) may counteract the microvascular dysfunction at peripheral and cardiopulmonary level, and fight the sequelae of pulmonary hypertension.
Lupus 2005
PMID:Heart involvement and systemic sclerosis. 1621 71


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