[Wegener's granulomatosis, 1992].
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Biomedical subjects
Publications and source records attributed to M Vilardell.
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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.
The case is reported of a patient who developed a vertebral osteomyelitis caused by Enterobacter cloacae. The organism was isolated in cultures of blood and vertebral puncture biopsy samples. The patient was satisfactorily treated with trimethroprim and sulphamethoxazole. Enterobacter cloacae, a Gram negative organism, has been confirmed as the cause of bacteremia in patients with burns, urinary infections, in adults with pneumonia, and in children with joint infections. Spondylodiscitis caused by Enterobacter cloacae has not previously been described.
In order to ascertain the incidence of monoclonal gammopathy (MG) in systemic lupus erythematosus (SLE), 120 SLE patients were evaluated. Four patients (3.3%), two of whom were under 50 years of age, were found to have a monoclonal immunoglobulin in their serum. Our data agree with the previous literature in demonstrating a higher incidence of MG in SLE patients in comparison with the general population. Neither the clinical importance nor the pathogenetic significance of this association is known.
Pathogenesis of neuropsychiatric manifestations of systemic lupus erythematosus (SLE) has not been clearly defined, and the search for pathogenic mechanisms has focused on the importance of several autoantibodies. There is increasing evidence that antibodies against gangliosides may have a pathogenic role in some neurological disorders. The aim of the present study was to examine the association between antibodies against gangliosides and neuropsychiatric SLE. We found anti-type II ganglioside antibodies in two out of 32 patients with multiple sclerosis (6.25%) and in 10 out of 60 patients with SLE (16.6%); five of 17 patients with neurological abnormalities also had high levels of these antibodies (29.4%). Five of the 10 patients with SLE and positive antiganglioside antibody had only IgM antibodies, three had IgG antibodies and two had both isotypes. By chi-square analysis, the incidence of anti-type II ganglioside antibodies was not significantly higher in patients with symptoms related to the nervous system than in SLE patients without neurological involvement (P > 0.2). No clear correlation was found between antibodies against gangliosides and cardiolipin.
BACKGROUND: The changes of nailfold capillaries detected by capillary microscopy is a characteristic frequently observed in systemic sclerosis. The relationship between the different capillary microscopic changes and clinical manifestation, organic disease and disease progression were studied. METHODS: Sixty-three patients were studied in whom capillary microscopy was performed and organic disease was determined by analytical and morphological examination. The presence of association between the capillary microscopic signs and clinical manifestation and disease progression were investigated by the chi-square and Fisher tests. RESULTS: Capillary microscopic alterations were found in 95% of the patients. The extense capillary loss was associated as statistically significant to the diffuse form of scleroderma. Statistically significant associations were not found between the different capillary microscopic signs and disease progression and organic disease (global and individual per each organ). CONCLUSIONS: Although capillary microscopic changes are present in most scleroderma patients they are not useful in the evaluation of organ involvement of the disease. The presence of extense capillary loss is linked to worse prognosis in its association to the diffuse form of scleroderma.
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A series of 31 patients suffering idiopathic inflammatory myopathy (IIM); we describe the extramuscular manifestations, specially pulmonary, the association to neoplasia, the histopathological characteristics, and their response to treatment. Fourty three percent of IIM patients presented a pulmonary involvement, 9% presented an associated neoplasia. The histopathological study allowed us to clearly differentiate dermatomyositis and polymyositis within IIM. 65% of patients initially responded to glucocorticoids and the most usefull therapeutic alternatives were azatioprine and cyclosporin-A.
We describe 2 patients with systemic lupus erythematosus (SLE) and high grade atrioventricular (AV) block. In one patient anti-Ro antibodies were positive. This antibody has been associated with congenital complete heart block in children of mothers with SLE; however, its role in adult high grade AV block is not clear. We reviewed the 8 published cases of adults with SLE and high grade AV block. The possible mechanisms that could be implicated in the pathology of high grade AV block are discussed.
The presence of anticardiolipin antibodies (aCL), von Willebrand factor activity and platelet function was studied in 35 patients with systemic sclerosis (SSc) scleroderma and 22 healthy controls. aCL positivity was observed in no patient with SSc or controls, whereas beta-thromboglobulin and platelet factor 4 levels were significantly higher in patients with SSc (p less than 0.001 and p less than 0.002, respectively). Furthermore, plasma from patients with SSc had a greater degree of aggregation to adenosine diphosphate 1 microM (p less than 0.05) but not to collagen or arachidonic acid. The plasma of patients with scleroderma also had increased von Willebrand factor activity compared with controls (p less than 0.001). We conclude that aCL appears not to play a central role in the pathogenesis of vascular and hemostatic alterations in SSc.
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Capillary microscopy is an easy, noninvasive procedure to examine in vivo the dermis capillaries of the nailfold. It has demonstrated clinical use in the etiologic study of Raynaud's phenomenon. We consider a "vascular activity pattern" to be present at capillary microscopy when one of the following associations is observed: tortuous capillary vessels plus ramifications with or without loss of a moderate amount of capillaries. Capillary tortuousity alone is not considered pathologic. It is well known that bleomycin (BLEO) can occasionally induce vascular-associated diseases. To examine the vascular damage produced by BLEO, we performed capillary microscopic studies on 40 patients with neoplasia, 21 of whom had received BLEO during the previous year. The maximum accumulated doses ranged from 15 to 379 U. The other 19 patients had advanced neoplasia, and 10 of them had received antitumoral combinations that did not contain BLEO. No one had clinical signs attributable to vascular toxicity. Twenty-six patients had no pathologic patterns (11 received BLEO and 15 did not). In the group of 14 patients with activity patterns, 10 had received BLEO and 4 had not (P = 0.035). Ten of 11 patients treated with BLEO who had normal capillary microscopic studies received total doses of less than 100 U. We conclude that capillary microscopy may demonstrate the vascular damage induced by BLEO even in asymptomatic patients.
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