The spectrum of pericardial tamponade in systemic lupus erythematosus. Report of ten patients.

OBJECTIVE. To describe the range of clinical manifestations and the outcome of pericardial tamponade in patients with systemic lupus erythematosus (SLE). METHODS. Patients with pericarditis and with pericardial tamponade were identified from our computerized database of 395 SLE patients. Medical records were reviewed to establish activity of SLE at the time of tamponade, as well as clinical and laboratory features, treatment, and outcome of the tamponade. RESULTS. Pericarditis occurred in 75 patients (19%), with 11 episodes of tamponade in 10 of them (13%; 2.5% of entire series). Tamponade was the initial manifestation of SLE in 4 patients. Seven episodes occurred during active lupus, with nephritis present in 6. Signs of venous congestion, including ascites and facial or peripheral edema, were the most common manifestation of tamponade. Pericardial fluid was exudative, and pericardial tissue demonstrated a range of findings including fibrinous and fibrotic changes, acute and chronic inflammatory infiltrates, and vascular proliferation. Tamponade was fatal in 1 patient, and 2 patients each had recurrent effusions and pericardial thickening. CONCLUSION. Pericardial tamponade may occur at any point in the course of SLE, and should be considered in patients with unexplained signs of venous congestion. The differential diagnosis includes active SLE, uremia, and infection. Treatment with high-dose steroids and either pericardiocentesis or placement of a pericardial window is indicated, but recurrent effusions or pericardial thickening may develop.     

Pericardial effusion and mitral valve involvement in systemic lupus erythematosus. Echocardiographic study.

Echocardiography was used in 30 women and 2 men with systemic lupus erythematosus (SLE) in order to determine the incidence and severity of pericardial effusion and mitral valve involvement. 31 patients showed normal thickness of the mitral valve leaflets, only one patient showed irregular thickening of the leaflets suggesting the presence of vegetations. Mitral valve motions were normal in all patients. These results indicate that myocardial and valvular involvement in SLE is usually not severe enough to result in haemodynamic abnormalities. Pericardial effusion was found in 2 patients who were symptom free, whereas 4 of the patients with a past history suggestive of pericarditis showed no echocardiographic evidence of pericardial effusion. These suggest the transient nature of pericarditis in SLE, and the value of echocardiography as a diagnostic tool in detecting clinically inapparent lupus pericarditis.     

The spectrum of pericardial tamponade in systemic lupus erythematosus: Report of ten patients

Objective. To describe the range of clinical manifestations and the outcome of pericardial tamponade in patients with systemic lupus erythematosus (SLE). Methods. Patients with pericarditis and with pericardial tamponade were identified from our computerized database of 395 SLE patients. Medical records were reviewed to establish activity of SLE at the time of tamponade, as well as clinical and laboratory features, treatment, and outcome of the tamponade. Results. Pericarditis occurred in 75 patients (19%), with 11 episodes of tamponade in 10 of them (13%; 2.5% of entire series). Tamponade was the initial manifestation of SLE in 4 patients. Seven episodes occurred during active lupus, with nephritis present in 6. Signs of venous congestion, including ascites and facial or peripheral edema, were the most common manifestation of tamponade. Pericardial fluid was exudative, and pericardial tissue demonstrated a range of findings including fibrinous and fibrotic changes, acute and chronic inflammatory infiltrates, and vascular proliferation. Tamponade was fatal in 1 patient, and 2 patients each had recurrent effusions and pericardial thickening. Conclusion. Pericardial tamponade may occur at any point in the course of SLE, and should be considered in patients with unexplained signs of venous congestion. The differential diagnosis includes active SLE, uremia, and infection. Treatment with high-dose steroids and either pericardiocentesis or placement of a pericardial window is indicated, but recurrent effusions or pericardial thickening may develop.     

Pericardial tamponade complicating systemic lupus erythematosus

We describe a case of pericardial tamponade as an initial manifestation of late onset systemic lupus erythematosus (SLE). Tamponade is uncommon in SLE, but when it occurs, it is usually during the initial presentation of the patient. The occurrence of tamponade in patients with SLE with pericarditis is unpredictable, but usually associated with radiographic evidence of cardiomegaly. Treatment consists of pericardiocentesis, administration of high dose glucocorticoids and drainage via pericardial catheter. Recognition of this rare manifestation of SLE may be life saving.     

Massive intractable pericardial effusion in a patient with systemic lupus erythematosus treated successfully with pericardial fenestration alone

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.     

SALMONELLA PERICARDITIS WITH TAMPONADE IN SYSTEMIC LUPUS ERYTHEMATOSUS

Despite the high frequency of both pericardial involvement andof infectious complications in SLE, septic pericarditis is uncommon.We report here a patient with SLE who developed tamponade dueto Salmonella infection. Most of the other eight recorded casesof septic pericarditis in SLE were due to Staphylococcus aureusand none has been previously attributed to Gram-negative bacteria. KEY WORDS: Septic pericarditis, Systemic lupus erythematosus, Gram-negative organisms, Pericardiocentesis     

Rapidly progressive fatal interstitial lung disease in a patient with an overlap syndrome of systemic lupus erythematosus and systemic sclerosis

A 31-year-old woman with a prior history of an overlap syndrome of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) developed fever, pericarditis with pericardial effusion and a rapidly progressive fatal interstitial lung disease. Diagnostic test and procedures, differential diagnosis and therapeutic approach are discussed.     

Antimyosin antibodies and constrictive pericarditis in lupus erythematosus

We report the first case of constrictive pericarditis in a woman with systemic lupus erythematosus (SLE). Immunopathologic studies demonstrated IgG, IgA, IgM and C3 distributed throughout her pericardium including vessel walls. Cardiospecific antimyosin, antisarcolemmal and antipericardial antibodies were detected in serum obtained prior to surgery or steroid therapy. Antibodies disappeared and creatine phosphokinase decreased to normal concentrations during prednisone therapy. Antimyosin antibodies were not detected in 11 sera or in one pericardial fluid obtained from patients with lupus with active pericarditis unaccompanied by constriction. Evaluation of antimyosin antibodies in other patients with this rare manifestation of SLE is warranted to further assess their pathologic significance.     

Systemic lupus erythematosus: an unusual cause of cardiac tamponade in a young man.

Although pericarditis and pericardial effusion are common cardiac complications of systemic lupus erythematosus (SLE), cardiac tamponade is a very rare initial manifestation of this disease. We describe a case of a young male patient in whom cardiac tamponade secondary to a loculated pericardial effusion was the presenting symptom of SLE.     

Systemic lupus erythematosus presenting as cardiac tamponade-a case report

Although pericarditis and pericardial effusion (PE) are some of the common manifestations of systemic lupus erythematosus (SLE), the occurrence of cardiac tamponade is quite rare. We present herewith a young girl with cardiac tamponade presenting as initial manifestation of SLE.     

Procainamide-induced lupus erythematosus pericarditis encountered during coronary bypass sugery

Procainamide is probably the most common offending drug responsible for the drug-induced lupus erythematosus syndrome today. Pericarditis has been reported to occur in from 14 to 18 per cent of the cases of procainamide-induced lupus erythematosus, and occasional reports of massive pericardial effusion, pericardial tamponade and constrictive pericarditis have appeared in the literature. We describe a patient who presented with features of procainamide-induced lupus erythematosus without any clinical evidence of pericarditis. He underwent coronary bypass surgery 12 days after administration of the drug was stopped and was found to have a significant pericardial effusion at the time of surgery; histologic examination of pericardial tissue and pericardial fluid confirmed that the pericardial effusion was related to the procainamide-induced lupus syndrome. The incidence of pericarditis in procainamide-induced lupus erythematosus may be higher than presently accepted figures would indicate. Symptoms and signs related to procainamide-induced lupus pericarditis may cause diagnostic confusion with common postoperative bypass complications; the full implications of this disease entity to the patient undergoing coronary bypass are unknown.     

Endoscopic pericardial fenestration for a patient with sustained lupus pericarditis.

A 57-year-old woman was diagnosed in January 1982 with SLE based on ANA 1:640, positive LE cell preparation, proteinuria (3+), and pericarditis. In 1984, 1994, and 1997, the pericardial effusion was noted to have increased without signs of disease exacerbation or cardiac tamponade, and pericardial drainage was repeated to control the effusion. A massive pericardial effusion developed in August 1997. After tuberculosis, hypothyroidism, neoplasm, and progression of SLE were ruled out, we decided to perform pericardial fenestration. A safe and minimally invasive pericardial fenestration was successfully completed endoscopically. Pathologic study of the specimen revealed chronic pericarditis. We consider endoscopic pericardial fenestration to be useful for at risk patients with pericarditis to control the effusion and establish a differential diagnosis.     

Cardiac constriction syndromes

This article focuses on syndromes associated with cardiac constriction (i.e., constrictive pericarditis). These include classic chronic constrictive pericarditis, subacute constriction including effusive-constrictive pericarditis, transient cardiac constriction, and occult constrictive pericarditis, all of which have their own clinical and developmental peculiarities. Establishing clinical suspicion is the basic first step in making a diagnosis, which can subsequently be confirmed by careful interpretation of imaging studies. With pericardial calcification, a simple chest radiograph may be sufficient; in other cases, Doppler echocardiography or chest computed tomography are necessary. The diagnosis of effusive-constrictive pericarditis requires cardiac catheterization combined with pericardiocentesis and the recording of intracavitary and intrapericardial pressures both before and after pericardiocentesis. It should be remembered that spontaneous regression is possible in some forms of constrictive pericarditis, particularly those that appear during the resolution of acute idiopathic pericarditis with effusion or that develop after cardiac surgery. Finally, there are only a few reports in the literature about occult constrictive pericarditis and its diagnosis is problematic.     

Large pericardial effusions due to systemic lupus erythematosus: a report of eight cases

The aim of this study was to describe the clinical, echocardiographic and laboratory characteristics of large pericardial effusions and cardiac tamponade secondary to systemic lupus erythematosus (SLE). An ongoing prospective study was conducted at Tygerberg Academic Hospital, South Africa between 1996 and 2002. All patients older than 13 years presenting with large pericardial effusions (> 10 mm) requiring pericardiocentesis were included. Eight cases (out of 258) were diagnosed with SLE. The mean (SD) age was 29.5 (10.7) years. Common clinical features were Raynaud's phenomenon, arthralgia and lupus nephritis class III/IV. Echocardiography showed Libman-Sacks endocarditis (LSE) in all the mitral valves. Two patients developed transient left ventricular dysfunction; both these patients had pancarditis. Typical serological findings included antinuclear antibodies, anti-double stranded DNA antibodies, low complement C4 levels and low C3 levels. CRP was elevated in six cases. Treatment consisted of oral steroids and complete drainage of the pericardial effusions. No repeat pericardial effusions or constrictive pericarditis developed amongst the survivors (3.1 years follow up). This study concludes that large pericardial effusions due to SLE are rare, and associated with nephritis, LSE and myocardial dysfunction. Treatment with steroids and complete drainage is associated with a good cardiac outcome.     

Transesophageal echocardiography in pericardial disease and tamponade

While most pericardial disorders can be imaged by transthoracic echocardiography, transesophageal echocardiography may be required in those cases where pericardial pathology is clinically suspected, but cannot be imaged adequately with transthoracic echocardiography. Transesophageal echocardiography is especially helpful in patients after heart or chest surgery, with cardiac compression by a loculated pericardial hematoma, in patients with dissection, endocarditis, or interatrial shunting associated with pericardial effusion, in patients with pericardial tumors, and in the differential diagnosis between constrictive pericarditis and restrictive cardiomyopathy.     

Cardiovascular manifestations of systemic lupus erythematosus: Current perspective

Cardiovascular manifestations develop in the majority of SLE patients at some time during the course of their illness, the most common being acute fibrinous pericarditis and pericardial effusion. Echocardiography has demonstrated an increased incidence of pericardial effusion, even in those who have minimal symptoms. Chronic adhesive pericarditis, pericardial tamponade, and constrictive pericarditis occur rarely. While myocarditis is commonly noted at autopsy, it is often silent clinically. Diagnosis during life can be confirmed only by endomyocardial biopsy. Electrocardiographic changes are often nonspecific. Endocarditis with superimposed nonbacterial verrucous vegetations (Libman-Sacks) is noted in more than 40% of hearts at autopsy, but is rarely diagnosed during life. Valve dysfunctions, such as aortic stenosis, aortic insufficiency, mitral stenosis, and mitral insufficiency, occasionally manifest during life and rarely may necessitate surgery.

Atrial and ventricular arrhythmias, first degree AV block, and acquired CHB occur in association with pericarditis, myocarditis, vasculitis, and myocardial fibrosis, respectively. CCHB developing in newborns of mothers with SLE, particularly those who have an antibody to soluble tissue ribonuclear protein RO(SS-A), is increasingly being appreciated by both pediatric cardiologists and rheumatologists.

Recently, severe coronary atherosclerosis resulting in angina pectoris and/or myocardial infarction in young adults has been noted, particularly in those who had developed risk factors such as hypertension and hyperlipidemia while receiving prolonged corticosteroid therapy. Rarely, coronary arteritis may produce similar symptoms. Congestive heart failure of either single or multiple etiologies carries an ominous prognosis. It remains a cause of high morbidity and mortality unless recognized early and treated properly. Extracardiac vascular manifestations of SLE include telangiectasia, vasculitis, livedo reticularis, Raynaud's phenomena, and thrombophlebitis, all of which may occur either alone or in different combinations.

Evidence is now slowly accumulating that substantiates that immune complex deposition, complement activation and subsequent inflammatory reaction is responsible for the majority of the cardiovascular manifestations of SLE, for example, pericarditis, myocarditis, endocarditis, coronary arteritis, coronary atherosclerosis, and systemic and pulmonary vasculitis. For the pathogenesis of CCHB, a maternal antibody to the soluble tissue ribonuclear protein RO(SS-A), which crosses the placenta during the early stage of gestation and either interferes with the development or afflicts a degenerating process in the developing conduction system, is being incriminated.

Corticosteroid therapy remains the cornerstone for the management of pericarditis, myocarditis, vasculitis, and coronary arteritis. However, nonsteroidal inflammatory agents, antiarrhythmic drugs, antihypertensive agents, vasodilator drugs, intensive endocarditis prophylaxis, and rarely, intracardiac pacing when indicated also play an important role in the overall management of cardiovascular manifestations of SLE.

Renal failure, lupus cerebritis, and infections are still the three most common causes of death in patients with SLE. However, recently increased cardiac mortality as a result of hypertension, congestive heart failure, coronary atherosclerosis, and thrombosis, the latter particularly in young women, has been recognized. Mortality of infants with CCHB born to mothers with SLE remains significant despite intracardiac pacing.     

Constrictive pericarditis in procainamide-induced lupus erythematosus syndrome

A rare case of constrictive pericarditis in procainamide-induced lupus erythematosus syndrome is reported. After 6 months of procainamide therapy fever, pleuritic chest pain, arthralgia and muscle soreness developed in a 47 year old man. These symptoms were soon followed by the onset of acute pericarditis and rapidly accumulating massive pericardial effusion. After withdrawal of procainamide therapy and administration of corticosteroids in large doses, there was marked subjective improvement and rapid reduction in pericardial effusion. However, constrictive pericarditis with massive leg edema and ascites developed 6 weeks after admission as corticosteroid therapy was gradually discontinued. These manifestations subsided after pericardiectomy was performed.     

原发性心包间皮瘤七例临床病理分析

原发性心包间皮瘤临床罕见，本组分析７例原发性心包间皮瘤，其症状、体征无特异性．胸片、心电图、超声心动等检查不能确定诊断，常被误诊为结核性心包炎、缩窄性心包炎等病，明确诊断需借助心包积液及心包的活组织的病理学检查及Ｇａ￣（６７）血池扫描Ｂｅｖ－ＥＰ＿４，免疫组织化学标记等方法。     

Pneumococcal pericarditis: A persisting problem in contemporary diagnosis

We reviewed the clinical and laboratory features of six patients with pericarditis caused by Streptococcus pneumoniae who were admitted to Boston City Hospital. The diagnosis of pneumococcal pericarditis was delayed or missed entirely during life in all patients. The frequent absence of pericardial friction rubs and cardimegaly on chest roentgenograms contributed to the difficulty in recognizing this illness. Electrocardiograms and physical examinations of the heart almost always disclosed abnormalities, but they were not sufficiently specific to suggest pericarditis, and patients were often thought to have had an acute complication of arteriosclerotic heart disease. Review of the English literature since 1945 supports the recent experience in our hospital that the diagnosis of pneumococcal pericarditis may be elusive.