[Clinical approach to antiphospholipid syndrome: from facts to questions].
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Biomedical subjects
Publications and source records attributed to B Wechsler.
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A review of the literature and our own experience have shown that the three types of antibodies to phospholipids (APL) usually looked for (i.e. dissociated treponema serology, antiprothrombinase and anticardiolipin) increase the risk of foetal loss, irrespective of whether the mothers have or do not have systemic lupus erythematosus. The prevalence of APL may exceed 40 percent in some series of women who suffered at least 3 foetal losses. Conversely, the incidence of foetal loss (often late in pregnancy) in women with APL has been estimated at 25 to 75 percent, depending on the studies. The presence of lupus seems to increase the risk. There is no consensus on the best factor predictive of foetal loss (antiprothrombinase or one of the anticardiolipin isotypes). Foetal loss seems to be caused by thrombosis of the placenta, the origin of which remains controverted. The therapeutic escalade consists of abstention (in the first pregnancy), aspirin (about 100 mg/day), aspirin-corticosteroid combination or subcutaneous heparin, high-dose intravenous immunoglobulins and plasmapheresis. With these various methods, the birth of a normal child can be expected in almost every case.
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Ten men aged 56 to 84 were hospitalized with a diagnosis of periarteritis nodosa, whereas they had multiple cholesterol embolism. The diagnosis was corrected post mortem in the first 3 patients and subsequently in live patients. The particularly misleading clinical manifestations were neurological (polyneuritis in 5 cases, mononeuritis in 1, central nervous system disorders in 3), pulmonary (alveolar haemorrhage in 2 cases, respiratory failure of unknown mechanism in 4) and pericardial (2 cases). Five patients had eosinophilia (more than 500 eosinophils/mm3). The elements that led to the correct diagnosis were the presence of vascular risk factors in all 10 patients (but hyperlipidaemia in only one), severe complications of the atheromatous disease in all cases, a precipitating or aggravating factor in 8 patients (anticoagulant therapy in 7, arteriography in 6) and the finding of purple or necrotic toes (6 cases). Histological (5 cases) and/or ophthalmological (2 cases) evidence was obtained in only 6 patients. Seven patients died 1 to 3 years after the onset of the disorders. Studies on low-density lipoprotein metabolism are in progress to determine the mechanism of clinical manifestations unexplainable by embolism.
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Among 250 patients with Behcet's disease, we describe 25 cases of angiographically proven cerebral venous thrombosis. Intracranial hypertension was the most frequent manifestation. Two initially untreated patients relapsed. Treatment of cerebral venous thrombosis consisted of combined heparin and steroids in 19 patients, steroids alone in three, and heparin alone in three others. Neurologic symptoms improved rapidly in all. Nineteen patients received long-term anticoagulation, and two received aspirin. Relapse of cerebral venous thrombosis or development of optic atrophy did not occur in treated patients. Partial or total recanalization of the occluded sinus was frequent. After more than 3 years of follow-up, the prognosis of dural sinus thrombosis is satisfactory.
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The ocular prognosis of Behçet's disease was assessed in 31 French native patients with ocular involvement. The mean follow-up time was 6 years. Ophthalmologic manifestations revealed the disease in 61% of patients, and it was the first symptom in 29%. Two groups of patients could be identified: the minor forms (23%) which remained stable with colchicine alone and the severe forms (77%) which required steroid (32%) and/or immunosuppressive (45%) therapy. In the severe forms, 32 eyes were unchanged, 4 eyes showed improvement, and 12 eyes showed deterioration in visual acuity. Positivity of HLA B5, sex and complete form of the disease were not predictive factors of ocular involvement, or of its seriousness. At 6 years, 16% of the eyes (versus 14% at the beginning) had a visual acuity less than 1/10, and 71% (versus 74% at the beginning) had a visual acuity greater than 8/10. A close collaboration between ophthalmologists and internists allows a good visual prognosis in Behçet's disease.
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Six young-adult patients (19- to 32-years-old) are described: 3 men with temporally localized systemic vasculitis (thromboangiitis obliterans 2, Churg-Strauss angiitis 1) and 3 patients (2 men, 1 woman) with isolated temporal arteritis. Temporal arteritis in subjects under 40 years of age consists of either a temporal localization of systemic vasculitis (thromboangiitis obliterans or Buerger's disease, Churg-Strauss angiitis or polyarteritis nodosa) or a distinct entity of which only 12 biopsy-proven cases have been reported to date. The latter is differentiated from temporal (giant cell) arteritis of the older patient by a higher incidence in men, and the absence or rarity of general symptoms, ocular complications and an accelerated erythrocyte sedimentation rate. Two types of temporal arteritides in young adults seem to be distinguishable: an asymptomatic form with an isolated temporal nodule and a more symptomatic one. In some cases, temporal arteritis in young adults corresponds to a unique entity "juvenile temporal arteritis", which seems to be different from Takayasu's arteritis, localized forms of polyarteritis nodosa and Kimura's disease. Although its treatment remains difficult to define, therapy of the symptomatic form could include steroids, whereas the asymptomatic one seems to require only simple monitoring.
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