Chorea, systemic lupus erythematosus, circulating lupus anticoagulant.
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Biomedical subjects
Publications and source records attributed to B Devulder.
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3 new cases of dermatomyositis associated with diffuse interstitial pulmonary fibrosis and, more exceptionally, with Sjögren's syndrome are reported. The pulmonary fibrosis observed in patients with dermatomyositis differs from that found in other connective tissue diseases in that it follows a more acute course and may respond to corticosteroids. Thus, in 2 of these patients treated with corticosteroids (combined in 1 case with cyclophosphamide) the high percentage of lymphocytes and polymorphonuclears in the broncho-alveolar lavage fluid, which reflects alveolitis activity, was reduced and the pulmonary fibrosis was cured in one patient and stabilized in the other.
The authors report the case of a woman with Löfgren's syndrome who developed renal failure associated with abnormalities of acidification and concentration of the urine. In the absence of a phosphate-calcium metabolic disturbance, these signs were attributed to a granulomatous interstitial nephropathy demonstrated on renal biopsy. These abnormalities regressed completely with steroid therapy. Secondary renal failure due to an isolated granulomatous interstitial nephropathy is exceptionally rare in sarcoidosis and has never been previously reported in Löfgren's syndrome.
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Circulating anticoagulant was detected during the course of choreic manifestations in three young women with systemic lupus erythematosus, associated in two cases with false syphilitic serology due to the presence of antiphospholipid antibodies. The high prevalence of the presence of these antibodies in lupic chorea was confirmed by reports of 41 cases in the literature. These findings could suggest a possible pathogenic role for these antibodies in choreic manifestations by either a thrombotic or auto-immune encephalitis mechanism.
The authors studied the role of Doppler ultrasonography in the diagnosis of Horton's disease, the assessment of the ophthalmic risk and the management of therapy. The study involved 24 patients and 20 control patients (mean age: 68 years) without inflammatory arteritis. A score (0 to 3) was established according to the degree of abnormality of blood flow. 12 patients were regularly followed up over a 2 year period. 83 p. 100 of the temporal arteries examined were pathological, 50 p. 100 of which had severe stenoses (scores 2 and 3). The facial, occipital and ophthalmic arteries were commonly involved (79 p. 100 of arteries examined), as was the central artery of the retina (92 p. 100, with 53 p. 100 of scores 2 and 3), although only 15 patients had minor (11 patients) or major (4 patients) ocular symptoms. However, a close correlation was observed between the presence of ocular symptoms and the severity of the stenoses observed on the central artery of the retina. The low incidence of signs of stenosis in the control group explains the excellent specificity of the Doppler examination (95 p. 100). Improvement with steroid therapy took at least 3 months and correlated well with the clinical and biological response. Blood flow changes reverted to normal in 4 patients after 1 year's treatment; 3 patients had relapses which were always associated with aggravation of the Doppler signs of stenosis. This study shows that Doppler ultrasonography is a valuable diagnostic tool, helps establish a prognosis and manage therapy in Horton's disease.
We report 10 cases of systemic manifestations of angiitis with asthma and hypereosinophilia, some of which had the pathologic features of an allergic granulomatous angiitis of Churg and Strauss. We compare these 10 cases with 109 observations previously reported in the literature from which they differ by a high incidence of digestive tract involvement and by a near constant increase of total plasma immunoglobulin E concentration during the evolutive phases of vasculitis. Broncho-alveolar lavage had been performed in 3 patients, showing pulmonary hypereosinophilia. We discuss the clinical, biological and pathogenic features which differentiate this syndrome, particularly from periarteritis nodosa.
Serum Total Thyroxine (T4), Triiodothyronine (T3), Free Thyroxine (FT4), Free Triiodothyronine (FT3) Reverse Triiodothyronine and T3 Resin Uptake (T3RU) as well as basal and post stimulating TSH were measured in twenty clinically euthyroid patients with the nephrotic syndrome. In comparison with control values, our patients showed (1) significantly lowered mean serum TBG levels (p less than 0,05) (2) unmodified mean serum T3, FT3 and rT3 values (3) significantly lowered mean serum T4 and FT4 levels (p less than 0,001 and p = 0,027 respectively) (4) significantly higher mean basal TSH, and increased TSH response to TRH. We found a significant correlation between proteinemia and T4 or FT4 (p less than 0,001; p less than 0,01 respectively). In summary, our study: Shows that the low Total T4 observed in the Nephrotic Syndrome is not only due to a decreased protein bound T4, but also to a decreased FT4; And brings evidence for mild hypothyroidism in nephrotic patients.
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Levamisole, an anthelmintic drug with immunopotentiating activity, is shown to have variable effects on cell-mediated immunity. We have studied the effect of a single oral dose (2.5 mg/kg or 5 mg/kg) on early and spontaneous E rosettes percentages in healthy and cancer patients. Pharmacokinetic study of this compound was conducted in parallel. The results indicated that single administration of levamisole (2.5 mg/kg) in healthy men can promote an increase of early E rosettes with mean peak plasma level of 0.8 micrograms. ml-1. On the other hand, there was no change in the proportion of early E rosettes in cancer patients and in the proportion of spontaneous E rosettes in healthy and cancer subjects.
Aorto-coronary by pass, widely accepted in the treatment of patients with coronary artery disease is still controversial in the treatment of Prinzmetal variant angina. Recurrence of attacks, occlusion of grafts and post-operative infarction were frequent by described and seem to be related with the persistence of coronary spasm. Authors propose the association of myocardial revascularization and coronary denervation by resection of periaortic plexi. Results of 50 surgical plexectomies associated with coronary by pass appear far better than by pass alone (mortality 4%, myocardial necrosis 4%, persistence of attacks 4%).
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