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Query: UMLS:C0409974 (lupus)
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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.
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PMID:Severe pulmonary hypertension in a patient with systemic lupus erythematosus and minimal lupus activity. 1186 96

The etiologic evaluation of pericardial effusion is frequently unsuccessful when noninvasive methods are used. To determine the cause of the current episode, all patients with echographically identified pericardial effusion from May 1998 to December 2002 underwent noninvasive diagnostic testing of blood, throat, and stool samples. Patients with postpericardiotomy syndrome were excluded. To analyze the value of our tests, we tested randomly selected blood donors as negative controls. Among 204 included patients, 107 (52.4%) had a final etiologic diagnosis: the etiology of 52 was highly suspected at first examination and later confirmed (thyroid deficiency, 5 cases; systemic lupus erythematous, 7; rheumatoid arthritis, 7; scleroderma, 3; cancer, 25; and renal insufficiency, 5). A definite etiologic diagnosis was made in 11 patients from pericardial fluid analysis (cancer, 5 cases; tuberculosis, 3; Streptococcus pneumoniae, Citrobacter freundii, and Actinomyces, 1 case each). Among 141 patients considered to have idiopathic pericarditis, 44 (32.1%) gained an etiologic diagnosis by our systematic testing strategy. This included serologic evaluation of serum (Coxiella burnetii, 10 cases; Bartonella quintana, 1; Legionella pneumophila, 1; Mycoplasma pneumoniae, 4; influenza virus, 1), viral culture of throat swabs (enterovirus, 8 cases; and adenovirus, 1), high-level antinuclear antibodies (>1/400, 3 cases), and thyroid-stimulating hormone (15 abnormal results). Antibodies to Toxoplasma and cytomegalovirus, enterovirus recovered from rectal swabs, and low-level antinuclear antibodies were seen with equal frequency in patients and controls. Using our evaluation strategy, the number of pericardial effusions classified as idiopathic was less than in other series. Systematic testing for Q fever, Mycoplasma pneumoniae, thyroid abnormalities, and antinuclear antibodies, accompanied by viral throat cultures, frequently enabled us to diagnose diseases not initially suspected in patients with pericardial effusion.
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PMID:Etiologic diagnosis of 204 pericardial effusions. 1466 88

Most patients suffering from systemic lupus erythematosus develop secondary heart disease at some time during the course of the primary illness. The most common forms of this type of heart disease are acute fibrinous pericarditis and hypertension. By means of echocardiography, an increased incidence of pericardial effusion has been demonstrated. Although commonly noted at autopsy, myocarditis is often clinically silent. However, endomyocardial biopsy may confirm its presence during life. Libman-Sacks endocarditis, although encountered in 40 to 50% of hearts at autopsy, is rarely diagnosed during life. When significant valve dysfunction such as aortic insufficiency or mitral regurgitation develops during the course of systemic lupus erythematosus, then Libman-Sacks endocarditis should be strongly suspected. Cardiac arrhythmias, first degree AV block, and acquired complete heart block may develop either de novo or in association with lupus pericarditis, myocarditis, vasculitis, etc. Complete congenital heart block has been reported in newborns of mothers with systemic lupus erythematosus, particularly those who have an antibody to a soluble tissue ribonucleoprotein antigen called RO(SS-A). Coronary arteritis and premature coronary atherosclerosis manifesting in either angina pectoris or myocardial infarction in young adults, particularly women suffering from systemic lupus erythematosus, have received attention recently. The development of hypertension and hyperlipidemia while such patients are receiving prolonged corticosteroid therapy has been incriminated as the significant risk factor in premature coronary atherosclerosis. Longstanding hypertension and congestive heart failure have unfavorable prognoses. This report is based on a cumulative review of 50 patients with acute and chronic systemic lupus erythematosus seen at our institution and in private practice during the last 10 years.
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PMID:Heart disease in systemic lupus erythematosus: diagnosis and management. 1522 37

Reduction of pathological autoantibodies may be useful in the treatment of systemic lupus erythematosus (SLE). On the other hand clinically manifested myocarditis in SLE, though uncommon, may be life-threatening and its pathogenesis has been ascribed to autoimmunity. The aim of this study is to present a rare case of a patient with severe lupus myocarditis, where immunoadsorption (IA) was evaluated as rescue therapy. A case of SLE with initial manifestation of myocarditis is reported in a 29-year-old male who presented with arthritis, fever, lymphadenopathy, joint swelling and morning stiffness. Laboratory evaluation revealed increased antinuclear antibody (ANA), slightly decreased complement and positive anticoagulant panel. From the above clinical and laboratory features, criteria of SLE seemed applicable. During his hospitalization, the patient developed pericardial effusion and cardiogenic shock. Although pericardiotomy was performed and was treated with immunosuppressive agents, plasmapheresis and supported with current medications, his clinical condition remained critical with an ejection fraction of 20%. At this point it was decided to receive IA onto staphylococcal protein A. After 6 sessions with IA and concomitant immunosuppression, the patient responded well, his condition improved and was dismissed with an ejection fraction of 50%. Fulminant lupus myocarditis is a severe and rare situation lacking a satisfying specific therapy available today. In our presented case, IA in addition to immunosuppressive therapy was beneficial. Considering the benefits of our case and the current knowledge, it might be useful to clarify the open question in scale pilot studies.
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PMID:Immunoadsorption in lupus myocarditis. 1527 78

Regional or localized pericarditis has been infrequently reported. We report a patient with systemic lupus erythematosus (SLE), who presented with retrosternal pleuritic-type chest pain without audible friction rub, electrocardiographic changes or detectable pericardial effusion on echocardiography. Computed tomography, however, revealed a circumscribed area of pericardial inflammation, suggesting a diagnosis of localized lupus-associated pericarditis. This case demonstrates that localized pericarditis may occur in SLE and that chest CT may be required as part of the work-up in the diagnosis of lupus pericarditis.
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PMID:Localized pericardial inflammation in systemic lupus erythematosus. 1530 Dec 50

We report on a rare case of a late-onset drug-induced lupus erythematosus. A 35 year old male patient complained about dyspnea, chest pain and reduced physical activity for three months. His medical history consisted of epilepsy treated with carbamazepine for 20 years. After diagnosis of a large pericardial effusion and percardiocentesis (1200 ml) the diagnosis of viral perimyocarditis was suspected. Under antiphlogistic treatment the symptoms vanished initially. Four weeks later the pericardial effusion recurred and a livedo reticularis became evident. A structural or infectious heart disease, in particular viral myocarditis, was ruled out invasively. Serologic testing revealed antinuclear antibodies and antibodies against histones without presence of antibody against ds-DNA, thereby confirming the diagnosis of carbamazepine-induced lupus erythematodes. After discontinuation of carbamazepine and immunosuppressive medication the patient recovered completely.
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PMID:[Chest pain, reduced physical activity, and polyserositis in a 35-year old patient with anticonvulsive medication]. 1628 38

The objective of this study was to describe the prevalence and outcome of disease-related serositis in Chinese patients with systemic lupus erythematosus (SLE). The records of all SLE patients who attended the medical clinics of Tuen Mun Hospital, Hong Kong were retrospectively reviewed. Patients with disease-related serositis at any stage of their illness were identified and the outcome of these serositis episodes was reported. Three-hundred and ten patients (90% women) who fulfilled at least four of the ACR criteria for SLE were studied. The mean age of SLE onset was 32.6 +/- 13.1 years. sixty-nine episodes of SLE-related serositis occurred in 37 patients - 18 (26%) episodes were pericarditis/ pericardial effusion, 30 (44%) were pleuritis/pleural effusion and 21 (30%) were peritonitis/ascites. The prevalence of serositis was 12%. At the time of serositis, 34 (92%) patients had active SLE in other systems. Nonsteroidal anti-inflammatory drugs (NSAIDs) were initially used in 13 (35%) patients. Moderate to high doses of oral prednisolone was used in 28 (76%) patients for both serositis and concomitant disease activity in other organs. All episodes of serositis resolved completely within two months. Over a mean observation of 46 months, nine patients had 18 relapses of serositis, which were responsive to either NSAIDs or augmentation of prednisolone dosage. Pleural fibrosis developed in three patients. Serosal complications are not uncommon in patients with SLE and can be life-threatening. NSAIDs and corticosteroids are often effective but more aggressive immunosuppressive therapy is required for severe or refractory cases. The prognosis of lupus serositis is generally good. Relapse or progression to fibrotic disease is uncommon.
Lupus 2005
PMID:Serositis related to systemic lupus erythematosus: prevalence and outcome. 1630 77

Systemic lupus erythematosus is a chronic inflammatory autoimmune disorder that can affect any organ or system. Although pericarditis is the most frequent cardiac manifestation of this entity, usually is not a life threatening situation. Pericardial effusion causing cardiac tamponade is a very rare complication in lupus, with an incidence less than 2%. We report a case of pericardial tamponade due to SLE with severe hemodynamic involvement in a 21-year-old woman associated to rapidly progressive glomerulonephritis, acute pancreatitis, acute acalculous cholecystitis, pleural effusion, hematologic, cutaneous and neurologic lupus activity. Recognition of this rare manifestation of SLE may be life saving.
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PMID:[Hemorrhagic pericarditis and cardiac tamponade in systemic lupus erythematosus. A case report]. 1636 74

Pericarditis is the most frequent cardiac manifestation of systemic lupus erythematosus (SLE), but pericardial effusion rarely occurs as the only first clinical manifestation of SLE. We report the case of a male patient whose initial manifestation of lupus was a pericardial effusion. We comment on its clinical course and we reviewed in the medical literature different presenting features in SLE.
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PMID:[Pericardial effusion as the only first clinical manifestation of systemic lupus erythematosus]. 1656 57

Antiphospholipid syndrome (APS) is a clinical entity characterized by arterial and venous thrombosis, adverse obstetric outcome and the presence of antibodies against phospholipids in serum or plasma. The objective of the present study is to describe a rare case of APS that occurred in a neonate born from a patient previously diagnosed as primary APS. A male, preterm born twin infant, whose mother had been diagnosed as primary APS, developed thrombocytopenia, livedo reticularis, pericardial effusion and thrombosis of the left subclavian and external jugular veins concomitantly with severe respiratory tract infection soon after his delivery, that culminated with his death two months after the birth, in spite of the large spectrum antibiotic therapy and all supportive measures. Laboratory findings included high titers of IgM anticardiolipin antibodies and moderate titers of IgG isotype and negative antinuclear antibody, configuring a case of neonatal APS. Neonatal APS is a rare clinical condition, with only a few cases described in the literature. Its occurrence may depend on the passage of antibodies through the placenta or, as it seems to have occurred in the present case, by the production of de novo antibodies by the fetus. The present case illustrates the necessity of a higher surveillance of the neonates born from mothers with primary APS or systemic lupus erythematosus (SLE) for the eventual development of such complication.
Lupus 2006
PMID:Neonatal antiphospholipid syndrome. 1676 6


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