Pericardial tamponade preceding cutaneous involvement in systemic sclerosis.
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Biomedical subjects
Publications and source records attributed to M Vilardell.
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In order to analyze the relationship between lipoprotein (a) [Lp (a)] and other lipoproteins during chronic renal failure and once renal function is restored after kidney transplantation, we determined the serum levels of total lipoprotein, high-density lipoprotein, low-density lipoprotein, and very-low-density lipoprotein cholesterols, total and very-low-density lipoprotein triglycerides, apolipoproteins A-I, B, C-II, C-III, and E, and E, and Lp (a) in 30 patients with chronic renal failure before and 12 months after renal transplantation. During the 1st year after transplantation, all patients were treated only with ciclosporin and prednisone and had serum creatinine levels < 1.6 mg/dl (140 mumol/l) and proteinuria < 500 mg/day. No patients had chronic hepatic disease. To determine reference values we studied a control group of 60 healthy volunteers. Before renal transplantation, the study group showed higher concentrations of triglycerides, very-low-density triglycerides, very-low density lipoprotein cholesterol, apolipoproteins, C-II and C-III, and Lp(a) than the control group. There was no correlation between Lp(a) and any of the studied variables. After renal transplantation, the serum levels of total lipoprotein, high-density lipoprotein, and low-density lipoprotein and apolipoproteins A-I and B increased significantly. Apolipoproteins C-II and C-III and Lp(a) decreased and normalized. After these changes had taken place, there was no relationship between Lp(a) and other parameters of lipoprotein metabolism. We conclude that the increase in Lp(a) during the chronic renal failure phase is basically related to the loss of renal function and does not depend on the resultant alterations which are produced in other lipoprotein variables.
Labial salivary gland tissues from 20 patients with primary Sjögren's syndrome (group 1), 26 with the secondary type of the disease (group 2), 16 patients with the incomplete form of the disease and 10 normal controls were examined for the presence of lymphocyte infiltrates, epithelial damages and plasma cell aggregates. Activated T lymphocytes of the helper phenotype predominated in groups 1 and 2. Aberrant epithelial expression of HLA class II molecules was found in the same group of patients, while IgG- and IgM- containing plasma cells were detected.
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A 45-year-old women presented with a deep ulcerative lesion of the left breast. Two months later, a new cutaneous necrotic lesion located in the right pretibial areas was observed. The only serum abnormality was the presence of circulating lupus anticoagulant. Clinical features suggested the diagnosis of pyoderma gangraenosum and histologic examination showed microthrombosis in the dermal capillary vessels adjacent to a dense, mixed inflammatory infiltrate with some degree of vascular damage. Although thrombosis may be the result of vasculitis, it is likely that in our patient the presence of a lupus anticoagulant activity played a role in the pathogenesis of the cutaneous ulcers.
We have studied the specificity of antiphospholipid antibodies in 148 patients with autoimmune diseases, 120 patients with systemic lupus erythematosus and 28 with the primary antiphospholipid syndrome. In addition, 20 patients suffering from syphilis were studied. As a control group, 64 healthy volunteers were investigated. Patient and control serum samples were tested for binding to seven different phospholipid antigens by ELISA. Interestingly, 90% of the sera from syphilis patients and 6% of the autoimmune patients exhibited a significant binding to platelet-activating factor (PAF), a molecule similar to the structure of phosphatidylcholine. In addition, the IgG fraction from one of the lupus patients, which showed a high binding activity to PAF, was further affinity-purified using both liposomes and an affinity chromatography column. Preincubation of these antibodies with PAF inhibited subsequent binding to immobilized PAF. These observations might suggest a putative interaction of antiphospholipid autoantibodies with PAF 'in vivo', which may have, in some patients, important pathophysiological consequences.
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OBJECTIVE: To evaluate the dependence on the serum cofactor of anticardiolipin antibodies (aCL) in infectious and autoimmune diseases. We also studied their correlation with some clinical manifestations, specially thrombosis. METHODS: aCL were determined with a standard ELISA method, and a modified ELISA in which we substituted bovine serum albumin (BSA), gelatin and skim milk powder for fetal calf serum (FCS). Categorized variables were analyzed by means of the chi 2 test and Fisher's test. Four groups of patients were studied. Group 1. Patients with aCL and autoimmune disease (systemic lupus erythematosus [SLE] and the primary antiphospholipid syndrome [PAPS]). Group 2. Patients with aCL, no symptoms and no underlying infection or autoimmune disease. Group 3. Patients with aCL and infectious diseases (syphilis, leprosy, HIV infection and Q fever). Group 4. Control group. RESULTS: (a) 19 of 20 samples from patients in Group 1 disclosed cofactor dependence in aCL activity. (b) 17 of 19 samples from patients in Group 3 had aCL activity, that was independent of the presence of the cofactor. (c) 3 of 4 patients in Group 2 had cofactor independent aCL and one had cofactor dependent aCL activity. (d) no control group patient had aCL. (e) association of cofactor dependent aCL with the development of clinical manifestations (thrombosis) was statistically significant (p < 0.0001). (g) cofactor dependent aCL and cofactor independent aCL were, respectively, associated with autoimmune and infectious diseases (p < 0.0001). CONCLUSIONS: (a) Dependence or independence of the cofactor helps to differentiate "infectious" aCL from "autoimmune" aCL. (2) aCL related clinical manifestations (thrombosis) depends on the presence of cofactor dependent aCL and not on cofactor independent aCL.
To determine the prevalence of Raynaud's phenomenon (RP) in Spain, we studied a working population of 988 men and 479 women. The overall prevalence of RP was 3.7%, with a 3.2% rate for men and 4.7% for women. There was a statistically significant association between Raynaud's phenomenon and a personal or familial history of perniosis, acrocyanosis or migraine among women. The age at onset was significantly lower in women. RP was classified as primary in 89% of the cases and as secondary in 11%. Examination by capillaroscopy revealed no significant differences between primary Raynaud cases and controls.
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OBJECTIVE: To determine the prevalence of cardiac valvular involvement in patients with the primary antiphospholipid syndrome. DESIGN: Cross-sectional study with evaluation of case patients and control patients by Doppler echocardiography. The mean follow-up for case patients was 22 months. SETTING: University-based tertiary medical center. PATIENTS: Twenty-eight consecutive patients who were diagnosed with the primary antiphospholipid syndrome during a 10-year period; 28 age- and sex-matched healthy controls. MEASUREMENTS AND MAIN RESULTS: Ten patients (36%; 95% Cl, 19% to 56%) with the primary antiphospholipid syndrome had cardiac valvular involvement: Four patients had mitral valve involvement; four patients, aortic valve involvement; and two patients, both mitral and aortic valvular involvement; no patients had tricuspid or pulmonary valve disease. Eight of 10 patients had a regurgitant murmur. None of the control patients had valvular disease. The mean mitral valve thickness in patients with mitral valve involvement was 7.0 +/- 1.6 mm, compared with 2.7 +/- 0.8 mm in patients with normal valves and 3.2 +/- 0.9 mm in the control group. The mean aortic valve thickness in patients with aortic valve involvement was 3.8 +/- 0.5 mm compared with 1.4 +/- 0.3 mm in patients with normal valves and 1.4 +/- 0.5 mm in the control group. Stenotic lesions were not found. Regurgitation was severe in two patients (one required surgery), moderate in three patients, and mild in three patients. CONCLUSIONS: Valvular involvement is frequently found in patients with the primary antiphospholipid syndrome. The lesions are left-sided, causing regurgitation that may be clinically important.
BACKGROUND: The idiopathic hypereosinophilic syndrome (IHS) is a rare entity of unknown etiology. Hematological and cardiac involvement is predominant. A series of 12 patients with this syndrome, initiated in 1982, is described. METHODS: Cardiological study by repeated echocardiograms and hematological study in peripheral blood and bone marrow upon initiation of the disease were performed. RESULTS: Median follow up was of 48 +/- 31 months. Males predominated (75%) with mean age being 55 +/- 15 years. The principal organs or systems involved were the heart (50%) and the nervous system (41%). Of the 6 cases with cardiac involvement only 2 had clinical manifestations. The remaining 4 patients were diagnosed from echocardiographic changes with the principal alterations observed being: atypical occupation of the ventricles, endocardial thickening and mitral and tricuspid subvalvular cumulus. Echocardiographic follow-up only showed changes in one case. Hematological involvement was characterized by moderate leukocytosis with hypereosinophilia formed by mature eosinophils, conservation of other hematopoietic series, absence of blasts in peripheral blood, finding suggestive of diseosinophilopoiesis and appearance of myelofibrosis and cytogenetic alterations. Survival at four years was 58%. CONCLUSIONS: In the series studied cardiac involvement is frequent, being principally diagnosed in a subclinical phase and with a very slow echocardiographic evolution. At a hematological level changes typical in myelodysplastic syndromes and myeloid leukemia were observed.