[Salmonella typhi murium spondylodiscitis in systemic lupus].
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Biomedical subjects
Publications and source records attributed to L Guillevin.
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The hypereosinophilic syndrome is an ill-defined nosological entity with predominant risks of cardiac and/or neurological lesions. In the light of new data on the effector cytotoxic effects of eosinophils, we have tried to establish new criteria of severity by purifying the circulating eosinophils of 14 patients with hypereosinophilic syndrome and testing their toxicity. This study has revealed the existence of low density ("hypodense") eosinophils with potential cytotoxicity in vitro. The worst clinical forms of the syndrome were observed in the group of patients with positive eosinophil toxicity tests. The significance of these cellular changes (hypodensity, eosinotoxicity) and their relationship with the clinical manifestations are discussed.
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Causes and precipitating factors for systemic necrotizing angiitis (NA) with asthma were sought in 43 patients, focusing on a history of vaccination and desensitization. Mean age of patients was 43.2 years. Diagnosis was based on histopathologic findings in 25 patients, arteriography in 2, and clinical criteria in 16. History of allergic manifestations (asthma, rhinitis, eczema, urticaria) was present in the family of 19 patients. Forty-two patients presented with asthma before development of NA and 23 of them were treated with steroids. Nineteen subjects gave a history of desensitization and 5 of vaccination in the 4 weeks preceding the disease. The main symptoms of NA were asthma in 43, fever in 25, weight loss in 31, peripheral neuropathy in 29, cutaneous signs in 25, digestive signs in 16 (abdominal pain, digestive bleeding, bowel perforation), noninfectious pneumopathy with pulmonary infiltrates in 33. Eosinophilia was 8,212 +/- 6,214/mm3. Antigen HBs was found in 2 of 30 patients. Prognosis of NA with asthma was good in 15 patients who recovered completely from the disease. Seven patients died and the other patients improved but remained under treatment. The survival curve showed that 75% of patients were alive after 60 months. Our findings suggest that different causes can be considered responsible for NA, and that, in cases of NA with asthma, there is reason to consider vaccination and desensitization as precipitating factors.
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Anatomical studies have demonstrated the high incidence of vasculitis in SLE, the appearances of which are variable and non-specific, ranging from necrotizing angiitis which is undistinguishable from periarteritis nodosa, to scarring lesions. Micro-angiitis is easily demonstrated in skin lesions and is also encountered to varying degrees in CNS, renal, cardiac, pulmonary and gastrointestinal localisations. Disease of large vessels is more rare and sometimes causes gangrene of the limbs. In SLE, vasculitis should be distinguished from thrombosis related to lupus anticoagulant and from atherosclerosis favoured by chronic steroid therapy but perhaps initiated by vascular deposits of immune complexes during the acute inflammatory stage. The treatment of lupic angiitis is mainly based on steroid therapy. The results are variable, probably due to the fibrous nature of some of the vascular lesions.
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Three cases of primary amyloidosis and 2 cases of familial amyloidosis complicated by asympathicotonic orthostatic hypotension are reported. Blood pressure measurements on a tilting table, plasma renin activity and plasma aldosterone or catecholamine concentrations enabled localisation of the lesion of the baroreceptor reflex in some cases. When the pre- and post-synaptic efferent sympathetic pathway was intact, treatment associating Tyramine and Tranylcypromine may provide these bedridden patients some autonomy of movement. When this pathway is affected by the disease the association of Indomethacin, Dihydroergotamine and 9-alpha-fluorohydrocortisone may be tried.
Endothelial-associated IgG were determined in 20 patients with systemic lupus erythematosis (SLE)-8 of whom had a lupus anticoagulant (LA) and 6 a history of thrombosis. The binding of IgG present in patient plasma to cultured human endothelial cells was detected using radiolabeled staphylococcal protein A. Thirteen samples gave positive results and a significant association between endothelial-associated IgG and lupus anticoagulant was found (p less than 0.05). No statistically significant relationship with a previous history of thrombosis was found. These results suggest that the lupus anticoagulant may be directly involved in immune vascular injury induced by either antibodies or immune complexes in SLE.
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A retrospective study of the diagnosis and causes of malaise and loss of consciousness has been conducted under the aegis of the French National Society of Internal Medicine. This multicentric and retrospective enquiry, which involved 512 patients, revealed that up to 44% of the malaise had no diagnostic label and that those of known aetiology were frequently due to cardiovascular disorders, such as postural hypotension (11.7%) or arrhythmia (9.5%). The study also demonstrated that the clinical enquiry was more important than complementary examinations which yielded few useful data.
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The mechanisms of blood pressure regulation in periarteritis nodosa and systemic sclerodermia are discussed in the light of results of personal studies. Hypertension during PAN may be divided into two groups: hypertension related to renal microangiopathy responsible for stimulation of the renin-angiotensin-aldosterone system which often runs a severe course, sometimes accompanied by renal insufficiency and requiring treatment with beta-blocking agents or angiotensin conversion enzyme inhibitors; secondly, hypertension may not be linked to the renin-angiotensin-aldosterone system and the prognosis is then better. In systemic sclerodermia, a similar pattern may be found but it would also seem that the renin-angiotensin-aldosterone system may be stimulated in the absence of any hypertension. This rise in plasma renin activity and aldosterone preceding the onset of an acute sclerodermic renal episode has a very poor prognosis. Corticosteroid therapy may favour such a process. Other regulation mechanisms are also discussed: prostaglandins, bradykinin system.