[Clinical approach to antiphospholipid syndrome: from facts to questions].
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Biomedical subjects
Publications and source records attributed to J C Piette.
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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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We looked for evidence of a Borrelia infection in 15 patients with morphoea. We were not able to detect antibodies to Borrelia burgdorferi in any of these 15 patients. None of the 14 skin biopsies examined by immunohistochemistry showed evidence of spirochaetes. Skin biopsies were cultured in 10 patients. All were negative. These results do not support a spirochaetal origin of localized scleroderma.
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.
OBJECTIVES: During the acute phase response, interleukin-1 induces production of inter-alpha-trypsin inhibitor. The measurement of urinary trypsin inhibitory activity which results from the effects of inter-alpha-trypsin inhibitor degradation products is easy, quick and inexpensive. We conducted a prospective study to investigate its value as a diagnostic tool in comparison with C-reactive protein. METHODS: Comparisons were made in 690 consecutive patients at admission to a department of internal medicine. RESULTS: The level of urinary trypsin inhibitory activity was significantly higher in patients with bacterial infection (mean = 123 IU/g creatinine) than in patients with either viral infection (34 IU), cancer (50 IU), elevated erythrocyte sedimentation rate without infection (45 IU), miscellaneous non-inflammatory diseases (27 IU) or in non-organic controls (19 IU) (Dunnet's test, p << 0.01). The receiver operating characteristic curve showed that sensitivity and specificity of urinary trypsin inhibitory activity were higher than those of C-Reactive protein for the diagnosis of bacterial infection. For levels > or = 60 IU, sensitivity was 75% and specificity 89%. Urinary trypsin inhibitory activity levels fell within 2 days in patients treated for acute bacterial infection. CONCLUSION: Urinary trypsin inhibitory activity could be a useful marker of bacterial infection particularly in patients with fever of unknown origin and/or elevated erythrocyte sedimentation rate.
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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.