[Systemic lupus erythematosus, antiphospholipid antibodies and severe arterial hypertension].
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Biomedical subjects
Publications and source records attributed to G Herreman.
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BCG has been disappointing as immunotherapy of numerous cancers, but it has been clinically successful in the intravesical treatment of bladder carcinomas sparing the muscle coat; it has indeed become the reference treatment for this type of cancer. However, complications are repeatedly reported, including generalized BCGitis. We report such a case with positive BCG culture. From the cases already published there emerges a homogeneous and often subacute clinical presentation suggestive of an ordinary pathogen. Bacteriology is not very helpful, even when recent techniques are used, and therefore the diagnosis rests on the context and, when samples are taken, on suggestive histological findings. To discuss the physiopathology of BCGitis--generalized immune reaction or multifocal BCG proliferation--is not useless since treatment depends on it. It is probable that these 2 mechanisms working together can be incriminated justifying the prescription of both antibiotics and corticosteroids. When this is done, the prognosis seems to be favourable in most patients. Yet a strict respect of contra-indications and a very careful subsequent radiotherapy should reduce the risks.
A false left ventricular aneurysm and coronary artery aneurysm were discovered in a 29 year old patient with Behçet's syndrome. The operation under cardiopulmonary bypass consisted of closing the neck of the false aneurysm by an endo-aneurysmal approach with a Gore-Tex patch. The coronary artery aneurysms were respected. There were no postoperative complications. Cardiac involvement is rare in Behçet's syndrome (6%). The originality of this case is the association of two aneurysmal pathologies: the coronary and ventricular aneurysms due to the angiitis and the myocardial fragility induced by ischaemia.
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During a mean therapeutic follow-up of 4 years, 25 out of 61 patients with Wegener's granulomatosis had one or several relapses. As in 7 of them the initial diagnosis had proved erroneous, this study concerns the remaining 18 patients who together totalled 31 relapses after the correct diagnosis was made. Twenty relapses occurred early on, within 4 months of a change in treatment, and they corresponded to recurrent activity of the disease. Ten relapses occurred later on, after treatment had been discontinued or kept at maintenance dose level. The relapses appeared as early as the first year in 41 percent of the cases. The ENT region was affected in 61 percent of the patients. Nine relapses occurred in the absence of treatment, including 6 which took place 1 to 8 years after treatment. Three relapses occurred less than 3 months after a prednisone and oral cyclophosphamide therapy was initiated. Compared with the group of patients without relapse, those in the relapse group were younger, more often treated with azathioprine and cyclophosphamide administered intravenously, and followed up for a longer period; their probability of survival was also higher. In case of early relapse after withdrawal of treatment the mean duration of that treatment had been shorter than in the absence of relapse. None of the patients whose treatment had been stopped more than 20 months after its onset had an early relapse. These data prompted us to make the following proposals: initially, cyclophosphamide should be administered as bolus injections (the time required to obtain results is then shorter than with the oral route), and maintenance therapy should last at least 20 months. Azathioprine should be used only when cyclophosphamide is contra-indicated. The relative indications for intravenous and oral cyclophosphamide remain to be determined. Discontinuing all treatments is a difficult decision to make, but even then the patients should be under close supervision in view of the possibility of late relapses.
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Congenital heart block is rare; it is acquired in utero, definitive and, more often than not, complete. It can be diagnosed by the appearance of fetal bradycardia around the 23rd week of gestation, during ultrasonographic monitoring of pregnancy. Heart block is usually associated with the presence of anti-Ro and/or anti-La antibodies in the mother's serum. These maternal immunological abnormalities can be isolated or associated with an autoimmune disease, usually systemic lupus erythematosus, but also Sjögren's syndrome, or more rarely still, an as yet unclassified connective tissue disease. Anti-Ro and anti-La antibodies cross the placental barrier and react with a fetal heart, leading to acute fetal myocarditis by the 17th week of gestation. When severe, it is lethal, otherwise it can result in degeneration and endocardial fibroelastosis, disrupting conduction and leading to congenital heart block. The ideal treatment would be prevention with corticosteroids. When the mother is Ro or La antibody-positive before pregnancy, elimination of these circulating antibodies can be attempted by treatment with 0.5 mg/kg body wt/d of prednisolone for 3 months. If the treatment is successful, corticotherapy can be prescribed early in the pregnancy to try to protect the fetus. However, there is not always a relationship between maternal anti-Ro antibodies and fetal heart block. If the Ro/La antibody-positive woman is already pregnant, but before her 17th week, it is possible to prescribe dexamethasone, which crosses the placenta and remains active, sometimes in association with plasmapheresis.
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To establish the clinical features and evolution of ureteral and testicular manifestations of polyarteritis nodosa, 8 cases among a group of 165 patients were reviewed. Urogenital manifestations are rare and were related to hepatitis B virus markers in 5 of the 8 patients. Malignant hypertension was present in 6 of 8 patients and renal insufficiency related to renal infarctions was noted in 5. Prognosis is good and regression generally is obtained with steroid therapy or with plasma exchange and vidarabine when polyarteritis is related to hepatitis B virus. In some cases ureteral stenosis may require an operation in association with steroids and/or immunosuppressive treatments.
Thirty cases of Wegener's Granulomatosis are reported, 16 men and 14 women aged 49.3 +/- 14 years at the onset of their illness. The presenting symptoms were oto-rhino-laryngeal in 22 cases and pulmonary in 16 cases. Renal disease was initial in only 1 case. The complete picture comprised oto-rhino-laryngeal (29 cases), pulmonary (26 cases), renal (27 cases), articular (16 cases), muco-cutaneous (19 cases), ocular (15 cases), neurological (13 cases), and muscular (10 cases) involvement. An increased sedimentation rate, leukocytosis, anaemia and thrombocytosis were common findings. The treatment comprised steroid therapy associated with immunosuppressor drugs (28 cases) and plasma exchanges (8 cases). External radiotherapy was used in 5 patients, including 3 with bronchopulmonary lesions, but was not effective. The probability of survival after the initial symptoms was 84 p. 100 at 1 year, 69 p. 100 at 3 years and 59 p. 100 at 5 and 10 years. Eight patients died; infection and renal failure were the principal causes. Late relapse occurred in 3 patients justifying long-term follow-up and the necessity of using the term "cure" with reserve. The clinical and therapeutic aspects are described in the light of the authors' experience and a review of the literature. The concept of localised form of the disease and bordeline forms with atrophic polychondritis, periarteritis nodosa, Churg and Strauss syndrome and lymphomatous granulomatosis are also discussed.
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A 26-year old woman gave birth, at term, to a child with isolated complete heart block. A second pregnancy was interrupted by foetal death. Among other immunological abnormalities, this young woman had an antibody resembling the anti-SS-B antibody. At pathological examination the foetus' heart was found to be free of malformation but presented with subacute myocarditis associated with microcalcifications of the conductive tissue. Such findings suggest that an incipient myocarditis may either result in foetal death or lead to fibrosis of conduction pathways with isolated complete heart block.
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.
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