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)

Pleural disease is a common pulmonary manifestation of systemic lupus erythematosus (SLE) that usually responds to corticosteroids and other immunosuppressive agents. In the present report, a new approach, pleural decortication, was used in a patient with medically refractory chronic pleuritis secondary to severe SLE. A 26-year-old woman with known SLE developed progressive dyspnea and pleuritic chest pain over several months. The other systemic manifestations of her lupus were controlled with cyclophosphamide and prednisone. A computed tomography scan revealed a persistent, small, loculated right pleural effusion; pleural thickening; and atelectasis of the right middle and lower lobes. Pulmonary function tests showed a severe restrictive defect. The patient was disabled by her severe dyspnea despite maximal medical therapy, and, therefore, surgery was considered. A right thoracotomy revealed entrapment of the right lung by dense visceral pleura. Decortication was performed. On pathology, pleuritis with vascular pleural adhesions was found. No lupus pneumonitis was noted. Postoperatively, a significant clinical improvement in dyspnea was evident within several weeks. On a 6 min walk test, the patient achieved 384 m with a Borg dyspnea scale rating of 2 compared with 220 m and a Borg dyspnea scale rating of 4 preoperatively. Her forced vital capacity improved from 24% predicted to 47% predicted, and her total lung capacity improved from 35% predicted to 54% predicted. Medical therapy of systemic lupus erythematosus has been proven to be effective in controlling pleuritis in most cases. However, in the event of refractory pleuritis or pleural thickening, decortication may be a viable alternative.
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PMID:Fibrothorax and severe lung restriction secondary to lupus pleuritis and its successful treatment by pleurectomy. 1241 Mar 25

This study is to describe our clinical experience of diffuse alveolar hemorrhage (DAH) in patients with systemic lupus erythematosus (SLE) in Taiwan. From July 1994 to June 2001, eight patients of DAH among 1541 different SLE patients (0.52%) admitted to the Chang Gung Memorial Hospital were included for chart review. Dyspnea (100%) and fever (87.5%) were the most common symptoms instead of hemoptysis (62.5%). The most common extrapulmonary presentation was renal involvement (100%), which included clinical nephritis, nephrotic syndrome or acute renal failure. The overall mortality rate was 50%. Two pregnant patients were successfully treated with combined plasmapheresis and continuous venovenous hemofiltration in addition to high dose corticosteroid. Analysis of the prognostic factors showed that the higher APACHE II (Acute physiology, Age and Chronic Health Evaluation) and organ system failure (OSF) scores, but not the SLE activity index (SLEDAI), were associated with the greater mortality. The higher serum creatinine level or the need of hemodialysis did not adversely affect the survival. In conclusion, DAH in SLE patients are often accompanied with multiple organ failure, aggressive immunosuppressive therapy and multiple modalities of extracorporeal organ support should be started early for a favorable outcome.
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PMID:Diffuse alveolar hemorrhage in systemic lupus erythematosus: a single center retrospective study in Taiwan. 1247 1

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

Antiphospholipid syndrome is characterized by venous and arterial thrombosis, recurrent pregnancy loss and the presence of the lupus anticoagulant, anticardiolipin antibodies or both. Antiphospholipid syndrome may occur as a primary disease or in patients with systemic lupus erythematosus or other autoimmune, infectious or neoplastic disorders. In this paper we report a 29-year-old Saudi female, a known case of antiphospholipid syndrome, presented with complaints of fever, breathlessness and generalized fatigue. Further investigations confirmed her as a case of myeloblastic leukemia (M1, French-American-British classification). Acute myeloblastic leukemia is not described to be associated with primary antiphospholipid syndrome in the literature to date. This is the first case report of such association.
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PMID:Acute myeloblastic leukemia in a patient with primary antiphospholipid syndrome. 1297 90

Work in Department of Energy (DOE) facilities has exposed workers to multiple toxic agents leading to acute and chronic diseases. Many exposures were common to numerous work sites. Exposure to crystalline silica was primarily restricted to a few facilities. I present the case of a 63-year-old male who worked in DOE facilities for 30 years as a weapons testing technician. In addition to silica, other workplace exposures included beryllium, various solvents and heavy metals, depleted uranium, and ionizing radiation. In 1989 a painful macular skin lesion was biopsied and diagnosed as leukocytoclastic vasculitis. By 1992 he developed gross hematuria and dyspnea. Blood laboratory results revealed a serum creatinine concentration of 2.1 mg/dL, ethrythrocyte sedimentation rate of 61 mm/hr, negative cANCA (antineutrophil cytoplasmic antibody cytoplasmic pattern), positive pANCA (ANCA perinuclear pattern), and antiglomerular basement membrane negative. Renal biopsy showed proliferative (crescentric) and necrotizing glomerulonephritis. The patient's diagnoses included microscopic polyangiitis, systemic necrotizing vasculitis, leukocytoclastic vasculitis, and glomerulonephritis. Environmental triggers are thought to play a role in the development of an idiopathic expression of systemic autoimmune disease. Crystalline silica exposure has been linked to rheumatoid arthritis, scleroderma, systemic lupus erythematosus, rapidly progressive glomerulonephritis and some of the small vessel vasculitides. DOE workers are currently able to apply for compensation under the federal Energy Employees Occupational Illness Compensation Program (EEOICP). However, the only diseases covered by EEOICP are cancers related to radiation exposure, chronic beryllium disease, and chronic silicosis.
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PMID:Silica exposure and systemic vasculitis. 1464 69

The main purpose of the study was to evaluate the utility of technetium-99m sestamibi myocardial perfusion single-photon emission computed tomography (Tc-99m sestamibi SPECT) in detection of cardiac involvement in systemic lupus erythematosus (SLE) or systemic sclerosis (SS) patients. Fifty SLE or SS female patients with cardiac symptom/sign such as chest discomfort and/or dyspnea and/or occasionally palpitation and 50 SLE or SS female patients without any cardiac symptom/sign were investigated using Tc-99m sestamibi SPECT during rest and stress after dipyridamole infusion. Twenty-five age- and sex-matched healthy females were also included as controls in this study. The results of Tc-99m sestamibi SPECT were classified into four types including normal, persistent perfusion defect (PD), reversible perfusion defect (RD), and reverse perfusion defect (RR). The results of Tc-99m sestamibi SPECT in the 25 healthy females were normal. Perfusion abnormalities were detected in 44/50 (88%) symptomatic SLE or SS patients. However, myocardial perfusion abnormalities were only detected in 19/50 (38%) asymptomatic SLE or SS patients (P value<0.05 by a chi2 test). However, for risk factor of coronary artery disease and abnormal resting EKG, the incidences were not significant between symptomatic and asymptomatic patients (P values >0.05 by a chi2 test). Tc-99m sestamibi SPECT is a useful noninvasive imaging modality to detect cardiac involvement in symptomatic or asymptomatic SLE or SS patients.
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PMID:Usefulness of technetium-99m sestamibi myocardial perfusion SPECT in detection of cardiovascular involvement in patients with systemic lupus erythematosus or systemic sclerosis. 1465 47

Pulmonary fibrosis is characterized by the accumulation of excessive connective tissue in the lungs. Its causes include chronic administration of some drugs for example bleomycin, cyclophosphamide, amiodarone, procainamide, penicillamine, gold and nitrofurantoin; exposure to certain environmental factors such as gases, asbestos and silica and bacterial or fungal infections. Some systemic diseases also predispose to the disease for example rheumatoid arthritis and systemic lupus erythematosus. The disease is associated with release of oxygen radicals and some mediators such as tumor necrosis factor-alpha TNF-alpha, transforming growth factor-beta TGF-beta, PDGF, IGF-I, ET-I and interleukins 1, 4, 8 and 13. The symptoms of the disease include dyspnea, non-productive cough, fever and damage to the lung cells. It is diagnosed with the aid of chest radiography, high resolution computed tomographic scanning and the result of pulmonary function tests. Drug-induced pulmonary fibrosis may involve release of free oxygen radicals and various cytokines for example IL-Ibeta and TNF-alpha via activation of nuclear transcription factor NF-beta as in the case of bleomycin and mitomycin or via release of TGF-beta as in case of tamoxifen or via inhibition of macrophages' and lymphocytes' phospholipases as in the case of amiodarone with the resultant accumulation of phospholipids and reduction of the immune system.
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PMID:Drug-induced pulmonary fibrosis. 1519 96

Infliximab is a tumour necrosis factor (TNF)-alpha antagonist that has revolutionised the treatment of Crohn's disease and rheumatoid arthritis. However, infliximab therapy can be complicated by a variety of adverse reactions. Acute infusion reactions occur during or shortly after infusion and typically consist of fever, chills, nausea, dyspnoea and headaches. Delayed reactions, characterised by myalgias, arthralgias, fever, rash, pruritus, facial, hand or lip oedema, dysphagia, urticaria, sore throat and headache may occur 3-12 days after infusion. Although the mechanisms of these reactions are not yet clearly defined, emerging evidence indicates that these reactions may be associated with the immune response against infliximab and the development of antibodies to infliximab.A number of studies have identified protective factors that may minimise adverse reactions, presumably related to the immune response against infliximab. Factors that may be protective by helping to establish immune tolerance for the foreign infliximab protein include concomitant administration of immunomodulators or corticosteroids, starting infliximab therapy with a 0, 2, 6-week induction regimen, maintenance dose administration with infusions every 8 weeks or less, and avoiding long periods between infusions. Infliximab therapy also may have other immunological consequences. There is evidence that infliximab may impede the appropriate immune response to a number of pathogens, prohibiting its use in patients with active infections. In addition, patients should be screened and appropriately treated for tuberculosis before initiating infliximab therapy. The development of autoantibodies, such as antinuclear antibody or anti-ds-DNA, has also been described with infliximab therapy, although the development of clinical lupus-like syndrome is rare. While there is a theoretical risk of increased rate of malignancies due to antagonism of TNFalpha, to date there is no clear evidence of such an effect. In addition, cardiac and neurological adverse events associated with infliximab therapy have been described. The mechanism for these adverse events is unclear. In summary, infliximab therapy can be an effective treatment for Crohn's disease; however, a number of immunological consequences and adverse events may complicate the infusion of this agent. Appropriate prophylaxis and therapy of these adverse reactions will allow infliximab to be used safely in the vast majority of patients.
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PMID:Managing immunogenic responses to infliximab: treatment implications for patients with Crohn's disease. 1530 61

Bilateral diaphragmatic paralysis (BDP) can occur in the course of motor neuron disease, myopathy, or from mechanical damage or the use of "ice slush" during cardiac surgery. BDP has been observed during and after infections, associated with systemic lupus erythematosus and mediastinal tumors, or may have idiopathic etiology. It is a serious and life-threatening condition. A 62-yr-old man presented with slowly progressive dyspnoea that worsened in the supine position and on bending forward. Chest X-rays, fluoroscopy, lung-function parameters and blood-gas analysis revealed respiratory failure. BDP was confirmed from a phrenic nerve stimulation test and measurement of transdiaphragmatic pressure (Pdi). Since there was no evidence of an obvious etiology, BDP was considered idiopathic. Other muscles were not involved. The pathological basis was probably focal demyelination in segments of the phrenic nerve. Because of increasing diaphragmatic muscle fatigue, the patient was treated with a nasal mask providing bi-level positive airway pressure (BiPAP) ventilation during the night. Clinical suspicion of BDP should always be raised in patients suffering slowly progressive dyspnoea without any obvious cardiac, metabolic or traumatic predisposing factors, and orthopnoea and dyspnoea on bending forward. Electromyographic tests and measurement of Pdi can reveal the correct diagnosis.
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PMID:Spontaneous bilateral diaphragmatic paralysis: a rare cause of respiratory failure. 1547 Nov 85

Pulmonary hypertension (PH) is a rare but potentially life-threatening complication of systemic lupus erythematosus (SLE). We reviewed the literature on this complication, its pathogenesis and clinical presentation, and treatment options. PH is reported in 0.5% to 14% of patients with SLE. The literature describes the cases of 105 patients, 90% of whom were female. Average age was 33 years, and overall mortality was 25 to 50% two years after PH diagnosis. As in patients with primary pulmonary hypertension, dyspnea is the most common presenting symptom of PH in patients with SLE. Up to 58% of patients with both PH and SLE have Raynaud's phenomenon. Echocardiography can show right ventricular hypertrophy and dilation, even before symptom onset. Right-heart catheterization, with an assessment of vasoreactivity, is a necessary part of the work-up and is also needed for treatment decision-making. PH in patients with SLE is associated with intimal hyperplasia, smooth-muscle hypertrophy and medial thickening, similar to the changes seen in primary PH. Several pathological mechanisms have been proposed for PH associated with SLE. They include vasoconstriction, vasculitis, thrombosis, anticardiolipin antibody and lupus anticoagulant. Endothelial dysfunction may be an important factor in the onset of PH, possibly by contributing to vasospasm. Higher serum endothelin levels are found in patients with SLE and pulmonary hypertension than in other SLE patients. Several specific antibody patterns have been reported in patients with PH and SLE. Oral calcium channel blockers are indicated for patients who respond to acute NO challenge. Continuous intravenous prostacyclin represents a therapeutic advance, although it appears less effective than in primary PH. Some patients have been improved by new oral endothelin receptor antagonists, usually combined with intensive immunosuppressive therapy. Patients with SLE have an increased risk of PH. Vigilance is therefore required to detect early signs of PH. Early diagnosis allows treatment to start before irreversible vascular lesions occur.
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PMID:[Pulmonary hypertension associated with systemic lupus erythematosus]. 1565 27


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