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.     

Postoperative constrictive pericarditis

Seven cases of postoperative constrictive pericarditis (PCP) were discovered in a retrospective study of patients given heart surgery in a hospital receiving patients from all over Italy in 1970-85. Five of those patients had received surgery for chronic rheumatic heart disease, 2 for congenital heart defects. Four had received a second heart operation before the pericardial condition was recognised. All were females and all presented systemic venous hypertension (one of them only after acute doses of physiological solution) with thickening of the pericardial layers revealed by sonography. In six cases the electrocardiographic ventricular complexes were normal or increased in amplitude and the heart/chest ratio was greater than 0.55. Pericardial knock was masked by natural or artificial atrioventricular valve opening noises in 6 cases. In one case only there were pericardial calcifications or signs of an earlier postpericardiotomy syndrome. The haemodynamic investigation revealed signs of ventricular diastolic constriction in 6 patients. Three patients died from complications of cardiac cirrhosis: 2 of them had previously received partial pericardiectomy. Another two, given the same operation, preserved a reasonable functional capacity 5 and 10 years after the pericardiectomy. One patient in NYHA functional class III has so far refused haemodynamic assessment (and surgical treatment) of the pericardial disease. Finally, the last patient complains only of attacks of heart palpitation caused by atrial flutter and controlled by antiarrhythmic treatment.(ABSTRACT TRUNCATED AT 250 WORDS)     

Carcinoid constrictive pericarditis

A 78 year old man presented with diarrhoea, anorexia, and progressive lower limb oedema. He was in atrial fibrillation and had a right pleural effusion and ascites. Ultrasound of the abdomen and 24 hour urinary hydroxyindoleacetic acid output indicated metastatic carcinoid syndrome. Cardiac catheterisation revealed pericardial constriction, and pericardial exploration showed a greatly thickened pericardium with no evidence of tumour invasion. The patient died within 24 hours of surgery. Necropsy findings were consistent with a diagnosis of constrictive pericarditis secondary to metastatic carcinoid syndrome.


Keywords: carcinoid syndrome; constrictive pericarditis; liver metastases     

Platelet antibodies in systemic lupus erythematosus

In systemic lupus erythematosus (SLE), the precise cause of the thrombocytopenia is unknown. Since platelet associated IgG is increased in many patients, it has been suggested that the destruction of platelets might be dependent on specific antibodies. In nine patients with SLE, platelet associated immunoglobulins were found together with free serum antibody which bound to platelets from all normal subjects. Using an immunoblotting technique with membrane proteins from normal platelets incubated with patient sera, target antigens were localized on a band of mol wt 108,000 in two cases (B. and N.) and on a band of mol wt 66,000 in a third (M.). When the same technique was applied to autologous platelets of patient N., autoantibody binding to the protein of mol wt 108,000 was demonstrated. The antigenic determinants were not removed from the platelets by enzyme treatment or by disulphide bond reduction, and were localized in the cytoplasmic fraction of the platelets.     

Anti-adenosine deaminase antibodies in lupus erythematosus

Adenosine deaminase (ADA) is an enzyme involved in purine metabolism and has a major role in the development and function of lymphoid cells. Congenital deficiency of ADA results in severe immunodeficiency. Patients with congenital ADA deficiency treated with polyethylene glycol-conjugated bovine ADA develop antibodies to ADA. This leads us to investigate the role of anti-ADA antibodies in patients with systemic rheumatic diseases. Commercially available ADA was used in ELISA and immunoblots for detection of anti-ADA antibodies. Four out of 100 patients examined were positive for anti-ADA antibodies. Two of them had peripheral blood lymphopenia but the antibody levels did not appear to correlate with the lymphocyte counts. Immunoblotting revealed that the antibodies recognized a 40 kDa peptide of ADA, corresponding to ADA1, the major component of ADA. Affinity-purified antibodies were used to locate the distribution of ADA on Hep-2 cells and lymphocytes by indirect immunofluorescence. Anti-ADA antibodies gave a distinct nuclear speckled pattern on acetone-fixed cells. With viable cell immunofluorescence, anti-ADA antibodies also stained the cell surface of HEp-2 cells and lymphocytes, indicating surface expression of ADA. The anti-ADA antibodies failed to gain access into the cytoplasm or nuclei when added to the cultures of HEp-2 cells. In summary, this is the first report of detection of anti-ADA1 autoantibody which is a new type of ANA with discrete, speckled nuclear staining, but which may not be associated with lymphopenia.     

Ventricular coupling in constrictive pericarditis

Because of the close anatomic association, the volume or pressure in one ventricle can directly influence the volume and pressure in the other ventricle. Disease states that reduce pericardial compliance should accentuate this coupling between the ventricles. We examined this hypothesis in six dogs. Constrictive pericarditis was induced by injecting an irritant mixture into the pericardial cavity. Three to 4 weeks after this injection, the hearts were removed and placed in cool cardioplegic solution. Balloons were inserted into each ventricle and the pressure and volume changes caused by increasing the contralateral ventricular volume were measured. Compared with that in a control group of four dogs, the coupling between the ventricles was significantly augmented in the group with constrictive pericarditis. All the measured changes in ventricular pressure or volume caused by increasing contralateral ventricular pressure or volume were significantly greater (p less than .05) in the group with constrictive pericarditis. The results of these experiments show increased coupling between the ventricles with constrictive pericarditis, which helps to explain some of the signs and symptoms of constrictive pericarditis.     

M-mode echocardiography in constrictive pericarditis

M-mode echocardiograms from 40 patients with proven constrictive pericarditis and 40 subjects without evidence of cardiac disease were reviewed for features previously described in constrictive pericarditis. In this large series, no single feature of the M-mode echocardiogram could be considered diagnostic, although a pattern of normal left ventricular size and systolic function, mild left atrial dilation, flattened diastolic left ventricular posterior wall motion and abnormal septal motion was found in most patients. It is concluded that the M-mode echocardiogram can provide findings suggestive of constrictive pericarditis but must be used in conjunction with hemodynamic and other studies to establish the diagnosis.     

系统性红斑狼疮血清抗核小体抗体水平及意义的探讨

目的 研究抗核小体抗体(AnuA)在系统性红斑狼疮(systemic lupus erythematosus，SLE)患者血清中的水平及其相关影响因素，探讨AnuA在SLE诊治中的作用和意义。方法 采用酶联免疫吸附法(EUSA)测定120例初诊SLE患者、55例其他风湿性疾病和30名健康对照血清中AnuA水平。同时记录各种临床表现，检测并分析其治疗前的其他自身抗体和实验室指标。结果 自身抗体在SLE及其他风湿病对照组的阳性率分别为AnuA56％和7％，抗dsDNA抗体35％和1％，抗Sm抗体24％和0。AnuA与SLE患者性别、年龄、病程无相关性，对sLE的诊断敏感性和特异性分别为55．8％、9513％，对狼疮肾炎(1upus nephritis，LN)的诊断敏感性和特异性分别为77．％、64．5%。AnuA与肝脏损害和疾病活动呈线性相关(t=3．152，2．171，P＜O．05)。AnuA分别与抗dsDNA、抗Sm联合检测对SLE诊断敏感性提高27％、40％（X^2值分别为38．930、18．161，P＜O．01)。结论AnuA在SLE血清中水平显著增高，AnuA测定是SLE诊断和治疗监测中有价值的新的实验室检测指标之一，与抗dsDNA抗体联合检测可提高诊断SLE、LN的敏感性和特异性。