[Sarcoidosis disclosed by involvement of the small intestine].
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Biomedical subjects
Publications and source records attributed to J Cabane.
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The relationship between abortive disease and systemic lupus erythematosus is complex as shown by data from the literature and by our 9 patients selected for presenting with abortive disease, circulating anticoagulant and biological signs of autoimmunity. The risk of transformation into a systemic disease is real, although difficult to evaluate in the absence of prospective studies, but the major problem with these patients is the severity of the obstetrical pathology. The sombre foetal prognosis, already reported in the literature, requires close supervision and sustained treatment during pregnancy. For these reasons we suggest calling autoimmune abortive disease the disease which affects women with signs of autoimmunity who have had several, often late foetal deaths associated with the presence of circulatory anticoagulant thought to interfere with placental blood flow.
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Among the various autoantibodies commonly found in women with systemic lupus erythematosus, the so-called lupus anticoagulant has been described in association with fetal losses. Recently, women with repeated spontaneous abortions and lupus anticoagulant, but no apparent systemic lupus erythematosus have been described. We have studied prospectively the possible connections between fetal losses and autoimmunity in a large control study of 130 idiopathic habitual aborters. These non-pregnant patients without any antecedent autoimmune disease were explored for the presence for autoantibodies especially lupus anticoagulant. Ten percent of the patients exhibit an antithromboplastin antibody (lupus anticoagulant) and half of this group possesses a striking association of biological manifestations of autoimmunity. In conclusion, two points appear: firstly, statistical correlation is demonstrated between antithromboplastin antibody and habitual abortion; secondly, doubt remains about the significance of the association between autoimmunity, fetal losses and antithromboplastin: beginning of systemic lupus erythematosus or new autoimmune entity with gynaeco-obstetrical expression.
The aspects of abdominal ultrasonography and computed tomography (CT) were studied in 4 patients (3 African and 1 Haitian) with abdominal tuberculosis. All were markedly debilitated and three patients had protracted fever. Tuberculosis was documented in all cases by demonstrating Mycobacterium tuberculosis in cultures of lymph nodes taken during laparotomy and/or cultures of products of gastric aspiration. Ultrasonography showed enlarged lymph nodes in the pancreatic and peripancreatic areas and also in the mesenteric, perivascular and hepatic pedicle areas. CT showed hypertrophied lymph nodes with low tissue density ranging from 20 to 35 Hounsfield units. Although non pathognomonic, these aspects were suggestive of tuberculosis. Intravenous contrast medium administration failed to increase the density in the center of lymph nodes but disclosed the existence of a thick hyperdense rim surrounding the hypodense center of the caseous lymph nodes. Repeated ultrasound and CT examination allowed to control the efficacy of antituberculous chemotherapy.
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In the prospective study reported, 35 patients with systemic lupus erythematosus underwent thorough psychiatric examination. They were divided into three groups according to whether their psychiatric symptoms were acute (10 patients), subacute (9 patients) or non-existent (16 patients). Particular attention was paid to such factors as obstetrical events (pregnancy, spontaneous or induced abortion), history of psychiatric disorders, emotional traumas and treatment with corticosteroids. The overall incidence of psychiatric symptoms was high (54%), mainly due to isolated depressive states (37%). The only significant contributing factors were a personal history of psychiatric disorders (p = 0.001) and emotional traumas (p = 0.0004). There was no difference between the three groups as regards the distribution, dosage and duration of corticosteroid therapy.
Severe forms of Raynaud's phenomenon are very disabling. In a randomized, single-blind trial, we have evaluated the effects of PGI2, a natural compound with strong vasodilator and anti-platelet activities, in 14 patients presenting with Raynaud's phenomenon. The patients received a 24-hour infusion of either PGI2 in doses of 10 mg/kg.min, or only the solvent (glycine buffer). All patients recorded the frequency and severity of the attacks before and after treatment, and 7 of the 8 patients who received the solvent benefited from a PGI2 infusion 30 to 60 days later. Among the 14 patients (6 men, 8 women), 10 had underlying collagen disease. The number of attacks per week was initially 15.9 +/- 5.3 (mean +/- s.e.). The resulting impairment was pronounced (+ + on a + to + + + scale). Radioimmunoassays of prostaglandins showed a strong increase in 6-keto PGF1 alpha levels during the infusion, without changes in thromboxane levels. A significant (p less than 0.05) reduction was observed in the number of attacks (2.6 +/- 2.5 per week) and in impairment (+ on average after PGI2 but not after the buffer). Improvement after PGI2 lasted from 0.5 to 12 months, and all but one patient regarded the treatment as effective in long-term, despite undesirable side-effects (flush, hypotension) which occurred regularly during PGI2 infusion. In all patients with ulcerations of the finger tips, these healed more rapidly after PGI2. It is concluded that in spite of immediate discomfort, PGI2 in 24-hour infusions seems to be of value in the treatment of severe Raynaud's phenomenon.
Superior vena cava obstruction (SVCO) is an infrequent syndrome. Clinical diagnosis is often late. 20 cases of SVCO collected in 7 years are being reported. In 17 cases, SVCO revealed the underlying disease. It was mostly neoplastic (12 times), especially pulmonary cancers (5 times) and lymphomas (twice). Among benign causes (5 times), mediastinal fibrosis was the most often recognized (twice). Prognosis depended on etiology: when it was malignant, mortality rate was 50 p. cent (actuarial survival rate after one year: 40 p. 100); when it was benign, there was no mortality (follow-up: 21 months). Thrombo-embolic complications (25 p. 100) were seen during the end-stage of neoplasms and were responsible in 4 out of 7 deaths. Surgery was interesting for diagnosis (histology) and treatment (bypass surgery, the result of which was good only in the 3 operated benign causes). A simple model is proposed for treatment.
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Syphilis is a well-established cause of uveal disease and is thought to be responsible for several distinct types of intraocular inflammation. Four cases of acquired syphilitic uveitis are reported. The diagnosis was essentially based on the clinical picture, the elimination of other possible causes, the results of reliable blood immunologic tests eg, the TPHA, FTA and Nelson tests, and the rapid response to penicillin therapy. Evaluation of anti-Treponema pallidum antibodies in the aqueous humor does not contribute to the diagnosis of syphilitic uveitis. Two cases needed large amounts of penicillin (total dosage, 440 million units). Oral steroids were used to control the associated inflammatory reaction and to prevent a Herxheimer reaction. All the patients responded well to therapy.
The microcirculation (terminal vessels with a calibre of less than 30 mu) was examined by means of capillaroscopy which was correlated with histological studies. According to the classification of vasculitis, microvasculitis is a pathological process involved in a number of collagen diseases. The study of the cutaneous lesions of leukocytoclastic angiitis is one of the best method for approaching the study of these microangiopathies, especially in the context of hypersensitivity vasculitis. The study of the microcirculation may also be valuable in other connective tissue diseases (various forms of necrotic vasculitis, lupus, scleroderma, polydermatomyositis) and in Behçet's disease. However, this study is only in its preliminary stages and the data of capillaroscopy need to be interpreted critically.
Sixty-five cases of necrotizing angiitis, including 20 with asthma (group A) and 45 without asthma (group B) were studied retrospectively. All patients were investigated for markers of hepatitis B, at least the HBs antigen. Skin lesions and arthralgias were more common in group B patients. Eosinophilia during angiitis was almost constant in group A and was observed in only 6% of group B patients. Renal involvement, hepatic lesions and arterial hypertension seemed to be more frequent in group B but the difference was not significant. The HBs antigen was present in 1/20 patients of group A and in 17/45 patients of group B (p less than 0.01). The anti-HBs antibody was found with equal frequency in both groups. The clinical and biological differences observed between these two groups of patients strongly suggest that necrotizing vasculitis with and without asthma are two separate nosological entities and in particular, that they have different causes.
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