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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The diaphragm is an unusual localization for disseminated erythematous lupus and is generally not recognized. We present the case of a 34-year-old woman who developed bilateral pain in the base of the thorax 15 days before hospitalization without any bronchial signs and NYHA stage II dyspnea. The patient's general health status remained satisfactory and the physical examination was normal except for diffuse inflammatory joint pain and cutaneous photosensitivization. The chest x-ray disclosed ascension of both hemidiaphragms with retracted lungs associated with bilateral basal atalectasia. The diagnosis of lupus with diaphragmatic involvement was retained due to the clinical presentation with diffuse joint pain, photosensitization with facial erythema, pericardial effusion and elevated antinuclear antibody and lymphopenia (1 100/mm3). Lung function tests revealed a restrictive syndrome. Oral corticosteroids 1 mg/kg/d enabled clinical and functional improvement. In light of this observation we discuss the pathogenic mechanisms of this uncommon localization of lupus and the difficulty of establishing a sure diagnosis.
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PMID:[Disseminated erythematous lupus with bilateral diaphragmatic involvement]. 1684 Sep 98

The aim of this study was to determine the association between lupus autoantibodies and the clinical manifestations and outcome in a cohort of Puerto Ricans patients with systemic lupus erythematosus (SLE). All patients fulfilled the American College of Rheumatology classification criteria for SLE. Demographic parameters, clinical manifestations over time and damage accrual were obtained at the last study visit. Disease damage was assessed with the Systemic Lupus International Collaborating Clinics Damage Index (SDI). ANA, ANA pattern, and anti-dsDNA, anti-Smith, anti-Ro (SSA), anti-La (SSB) and anti-snRNP antibodies were measured at the time of SLE diagnosis. Chi-square test, Fisher exact test, ANOVA, logistic regression and general lineal model analyses were used to evaluate these associations. Ninety-six percent of patients were females. The cohort had a mean age of 40.2 +/- 12.0 years and mean disease duration of 9.6 +/- 7.0 years. Patients with elevated anti-dsDNA antibodies were more likely to have vasculitis, pericardial effusion, renal involvement, anaemia, leukopenia, lymphopenia and thrombocytopenia. Anti-Smith antibodies were positively associated with skin ulcerations, elevated liver enzymes, renal involvement and thrombocytopenia. Anti-Ro antibodies were related with the presence of discoid lupus, serositis, pneumonitis, elevated liver enzymes, hemolytic anaemia, leukopenia and lymphopenia. No positive associations were found for anti-snRNP or anti-La antibodies. The presence of anti-dsDNA, anti-Smith and anti-Ro antibodies was associated with higher SDI scores. In conclusion, anti-dsDNA, anti-Smith and anti-Ro antibodies are associated with several clinical manifestations and more damage accrual in Puerto Ricans with SLE. These findings provide valuable clinical and prognostic information for this ethnic population.
Lupus 2006
PMID:Clinical and prognostic value of autoantibodies in puerto Ricans with systemic lupus erythematosus. 1721 98

The authors report the case of a 48-years-old Caucasian women, with a previous diagnosis of systemic lupus erythematosus characterized by asthenia, fever, skin rash, alopecia, Raynaud's phenomenon, arthritis, pericardial effusion, interstitial pulmonary involvement, diffuse proliferative glomerulonephritis with crescents and anemia. The presence of severe anemia refractory to high doses of glucocorticoids (1 mg/ /Kg/day), iron therapy and blood transfusions, associated with a low reticulocyte count determined the execution of a bone marrow aspiration, biopsy and immunophenotyping, which were compatible with the diagnosis of Myelodysplastic Syndrome. The treatment with erythropoietin (5.000U 3x/week) and cyclophosphamide pulses (1 gr/m(2) month) induced complete regression of morphologic bone marrow changes and anemia. The main causes of anemia in lupus patients are discussed.
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PMID:[Systemic lupus erythematosus and anaemia]. 1745 Jul 68

Patients with cardiac dysfunction may be at increased risk of cardiac toxicity when undergoing hematopoietic stem cell transplantation (HSCT), which may preclude them from receiving this therapy. Cardiac dysfunction is, however, common in systemic lupus erythematosus (SLE) patients. While autologous HSCT (auto-HSCT) has been performed increasingly for SLE, its impact on cardiac function has not previously been evaluated. We, therefore, performed a retrospective analysis of SLE patients who had undergone auto-HSCT in our center to determine the prevalence of significant cardiac involvement, and the impact of transplantation on this. The records of 55 patients were reviewed, of which 13 were found to have abnormal cardiac findings on pre-transplant two-dimensional echocardiography or multi-gated acquisition scan: impaired left ventricular ejection fraction (LVEF) (n = 6), pulmonary hypertension (n = 5), mitral valve dysfunction (n = 3) and large pericardial effusion (n = 1). At a median follow-up of 24 months (8-105 months), there were no transplant-related or cardiac deaths. With transplant-induced disease remission, all patients with impaired LVEF remained stable or improved; while three with symptomatic mitral valve disease similarly improved. Elevated pulmonary pressures paralleled activity of underlying lupus. These data suggest that auto-HSCT is feasible in selected patients with lupus-related cardiac dysfunction, and with control of disease activity, may improve.
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PMID:Autologous hematopoietic stem cell transplantation in systemic lupus erythematosus patients with cardiac dysfunction: feasibility and reversibility of ventricular and valvular dysfunction with transplant-induced remission. 1748 45

Chronic granulomatous disease (CGD) is characterized by recurrent infections and granuloma formation. In addition, we have observed a number of diverse autoimmune conditions in our CGD population, suggesting that patients with CGD are at an elevated risk for development of autoimmune disorders. In this report, we describe antiphospholipid syndrome, recurrent pericardial effusion, juvenile idiopathic arthritis, IgA nephropathy, cutaneous lupus erythematosus, and autoimmune pulmonary disease in the setting of CGD. The presence and type of autoimmune disease have important treatment implications for patients with CGD.
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PMID:Chronic granulomatous disease as a risk factor for autoimmune disease. 1882 51

Systemic lupus erythematosus (SLE) is often complicated by pericarditis with effusion, which generally responds well to glucocorticoid. We report herein a Japanese patient with SLE who showed a sign of cardiac tamponade and severe chest and back pain because of massive intractable pericardial effusion. Pulse glucocorticoid and pulse cyclophosphamide gained marginal effects. Pericardial effusion accumulated again soon after ultrasound-guided pericardiocentesis and drainage. Pericardial fenestration performed surgically as a last resort, for draining pericardial fluid into the pleural space, was very effective, and only a much smaller amount of fluid was observed in the space thereafter in comparison with the volume before the surgery. Pathological examination of the retrieved pericardium unfolded intense hyperplasia of small vessels and capillaries. Levels of IL-6 and TNF-alpha in pericardial effusion were extremely higher than those in serum. Pericardial effusion with extensive capillary hyperplasia in SLE would be resistant to medical treatment and require surgical fenestration.
Lupus 2008 Nov
PMID:Massive intractable pericardial effusion in a patient with systemic lupus erythematosus treated successfully with pericardial fenestration alone. 1885 29

To determine the frequency and clinical correlates of asymptomatic pericardial effusion (PE) in patients with systemic lupus erythematosus (SLE), echocardiography and electrocardiography were performed in 50 consecutive patients with SLE. Among 50 patients with SLE, 12 patients (24%) had PE and 17 patients (34%) had hypoalbuminaemia. Patients with PE had a significantly lower serum albumin (P < 0.001), higher incidence of proteinuria (P = 0.003), higher C-reactive protein (P = 0.036) and pulmonary artery systolic pressure (P = 0.011) and tended to have a higher incidence of PR-segment depression (P = 0.082) compared with those without PE. When four variables (PR-segment depression, C-reactive protein, serum albumin and pulmonary artery systolic pressure) were used in the multivariate analysis, serum albumin (P = 0.005, odds ratio = 0.016) and pulmonary artery systolic pressure (P = 0.010, odds ratio = 1.106) emerged as significant variables related to the occurrence of asymptomatic PE. Thus, an increase in hydrostatic pressure of the right heart cavities and a decrease in colloid osmotic pressure were important factors associated with the presence of asymptomatic PE in patients with SLE.
Lupus 2009 Feb
PMID:Asymptomatic pericardial effusion in patients with systemic lupus erythematosus. 1915 Nov 13

Pericardial effusions causing pericardial tamponade are rare in patients with systemic lupus erythematosus (SLE). The goal of this study is to describe in detail the clinical and laboratory characteristics of a group of patients with pericardial effusions and pericardial tamponade secondary to SLE. We retrospectively reviewed the records of 71 patients with SLE, admitted to our Hospital between 1985 and 2006 with a diagnosis of pericarditis, pericardial effusion and tamponade. Clinical features in the patients with tamponade were compared with those with pericardial effusions without tamponade. Pericardial effusion and SLE was confirmed in 41 patients. Pericardial tamponade occurred in nine of these patients (21.9%) at the time of presentation. All tamponade patients were women. Patients with pericardial effusions who developed tamponade had a statistically significant (P = 0.05) lower C4 level as compared with patients who did not develop tamponade. A pericardial window was required in five patients even though the patients were receiving high-dose corticosteroids. In the present series, all patients with tamponade were treated with high-dose corticosteroids though five of nine patients required a pericardial window in contrast to previous studies. A low C4 level at presentation was predictive of the development of tamponade physiology.
Lupus 2009 Jun
PMID:The spectrum of clinical manifestations, outcome and treatment of pericardial tamponade in patients with systemic lupus erythematosus: a retrospective study and literature review. 1943 60

Cardiac disease is common among patients with Systemic Lupus Erythematosus (SLE). Pericardial, myocardial, valvular, and coronary artery involvement have been extensively reported. The three major coronary abnormalities associated with myocardial injury in SLE are premature atherosclerosis, coronary arteritis and, less frequently, coronary aneurysms. A 26-year-old black male patient with a 5-year history of anti-phospholipid syndrome sustained a lateral wall myocardial infarct associated to angiographic evidences of multiple, diffuse, saccular coronary aneurysms without evidence of atherosclerotic occlusive disease in all three coronary arteries. Serologic studies were consistent with active SLE. Lupus-associated nephritis was also present. Radiographic studies showed no evidence of brain or thoracic aneurysms. A transthoracic echocardiogram showed an estimated ejection fraction of 35% and a moderate pericardial effusion. High dose i.v. steroids were started along with systemic anticoagulation. The patient had an uneventful clinical course and was discharged by the fourteenth day on high doses of oral steroids (60 mg daily), statins, oral anticoagulation (warfarin 5 mg), antihypertensives and aspirin. The patient was lost to follow-up and developed steroid-induced hyperglycemia, 20 pound weight gain and warfarin intoxication. A three-month follow up coronary angiography showed complete resolution of the aneurysms and serologic studies showed no active autoimmune disorder at that time. Coronary artery aneurysms have previously been reported in 15 cases of patients with SLE. An extensive Medline search of the literature revealed no previous reports of diffuse saccular coronary aneurysms involving all three coronary arteries associated with active SLE. This case highlights the unusual presentation of acute SLE in a young patient complicated by multiple aneurysms and acute myocardial infarction. In this case, we believe that a direct causal association exists between acute SLE-associated aneurysms and myocardial ischemic injury. Early recognition and prompt treatment with large doses of corticosteroids may decrease the mortality and morbidity associated with this condition.
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PMID:An unusual presentation of chest pain. 1961 May 65

Jaccoud's arthropathy (JA) was initially described as a secondary complication to rheumatic fever (RF). However, most recently described cases are associated with systemic lupus erythematosus (SLE). At least in RF, this articular complication has been observed to occur in association with valvular heart disease. The aim of this work is to investigate the presence of valvulopathy in patients with SLE and JA, when compared to lupus patients without such complication. Patients with diagnosis of SLE based on the American College of Rheumatology criteria were enrolled in the study and divided into two groups: with or without JA and evaluated by transthoracic echocardiography. A total of 113 patients with SLE (25 with JA and 88 without JA) were assessed, of which 108 were females and five were males. Echocardiographic changes were found in 24 patients (21.2%) out of the entire population, including valvulopathy in 17 cases (15%), pulmonary hypertension in 7 cases (6.2%) and pericardial effusion in 2 cases (1.8%). In general, echocardiographic changes were more frequently seen in the JA group in comparison with the control group (p = 0.04). Additionally, in the JA group, valvulopathy was found in nine cases (36%) against eight cases (9%) in the control group (p = 0.001). This study reveals for the first time the association between the presence of valvular heart disease and JA in SLE patients, suggesting that the presence of JA may be a marker of such complication. Additional studies are required for clarification of the mechanisms involved in both complications.
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PMID:Valvular heart disease in systemic lupus erythematosus and Jaccoud's arthropathy. 1985 68


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